Materiały źródłowe

• #1 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary. […] Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin. […] Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease.

• #2 Ebstein anomaly: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/ebstein-anomaly-management-and-prognosis

  Ebstein anomaly: Management and prognosis […] The approach to the management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including the presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention. […] Indications for intervention or surgical repair of children and adults with Ebstein anomaly include worsening heart failure (HF), progressive cyanosis or uncontrolled arrhythmias. Surgical repair may also be considered if there is decline in functional capacity with evidence of right ventricular (RV) dysfunction and/or hypoxia. Occasionally, asymptomatic adult patients with severe tricuspid valve regurgitation and repairable tricuspid valves are considered for early repair to prevent progressive right heart enlargement.

• #3 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #4 Ebstein Anomaly Diagnosis & Treatments | Mount Sinai – New York

  https://www.mountsinai.org/locations/childrens-heart/conditions/ebsteins-anomaly

  Our Mount Sinai Kravis Children’s Heart Center team treats your child and offers you peace of mind, whether your child has a mild form of Ebstein’s anomaly or a more serious one. Our pediatric cardiology specialists are prepared to make an accurate diagnosis. And we offer the best possible treatment for your child. […] In mild cases of Ebstein’s anomaly, no treatment may be necessary. In serious cases, we may use medication to relieve some symptoms. The only way to fix Ebstein’s anomaly is through one of the following surgical procedures: […] Following treatment, we work with you to create a long-term care plan for your child’s lifetime. Lifelong care and follow-up will be necessary to monitor your child’s health. We will check how your child’s valve and heart function. […] The Children’s Heart Center has doctors and nurses who are dedicated to providing the most personalized and expert care for your child with congenital heart disease, including Ebstein’s anomaly, at any age. We involve you, parents and family, as we use our expertise for diagnosis and treatment with the most effective minimally invasive techniques.

• #5 Ebstein anomaly – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132

  Treatment of Ebstein anomaly depends on: […] The goals of treatment are to: […] Treatment may include regular health checkups, medicines, or a heart procedure or surgery. […] If Ebstein anomaly isn’t causing irregular heartbeats or other symptoms, regular health checkups may be all that’s needed. […] If you have Ebstein anomaly, you may get medicine to: […] Surgery is typically done if Ebstein anomaly causes severe tricuspid regurgitation and there is heart failure or increasing difficulty with exercise. […] Surgery also may be recommended if other symptoms, such as some irregular heartbeats, are very bad or affecting quality of life. […] If surgery is needed, it’s important to choose a surgeon who’s familiar with Ebstein anomaly. […] Surgery to treat Ebstein anomaly and related heart conditions may include:

• #6 Ebstein anomaly // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/ebstein-anomaly

  Treatment of Ebstein anomaly depends on the severity of the heart problem and the symptoms. Treatment may include regular health checkups, medicines, or a procedure or surgery. […] The goals of treatment are to reduce symptoms and prevent complications, such as irregular heartbeats and heart failure. […] If Ebstein anomaly isn’t causing irregular heartbeats or other symptoms, a doctor may recommend regular checkups. […] If you have Ebstein anomaly, you may get medicine to help: Control irregular heartbeats or other changes in the heart’s rhythm. Prevent fluid buildup in the body. Too much fluid in the body can be a sign of heart failure. Prevent blood clots, which may occur if Ebstein anomaly occurs with a hole in the heart. […] Surgery is typically recommended if Ebstein anomaly causes severe tricuspid regurgitation and there is heart failure or increasing difficulty with exercise.

• #7 Ebstein’s Anomaly: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16946-ebsteins-anomaly-for-adults

  Treatment depends on how severe your symptoms are. If you have mild or no symptoms, your provider may monitor your heart and watch for changes. […] If you have signs of heart failure or arrhythmia, you may receive noninvasive treatments, such as: […] For more severe symptoms, tricuspid valve surgery may be the best option to provide lasting improvement in valve function. If there is enough tissue present, surgeons can repair the valve. This is the preferred treatment because it uses your own tissue. […] If repair isn’t an option, it’s possible to insert a replacement mechanical valve or one made of biological tissue. If you receive a mechanical valve, you will need to take blood-thinning medication for the rest of your life. […] Your provider may combine tricuspid valve surgery with other heart procedures. This may include repair of the hole between the heart’s upper chambers or arrhythmia treatment. There are many types of treatment for arrhythmia, including catheter ablation or a pacemaker. Rarely, you may need a heart transplant if other treatments have failed. […] If this occurs, you may need surgery to repair or replace your tricuspid valve and fix other related heart problems.

• #8 Ebstein anomaly – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132

  Treatment of Ebstein anomaly depends on: […] The goals of treatment are to: […] Treatment may include regular health checkups, medicines, or a heart procedure or surgery. […] If Ebstein anomaly isn’t causing irregular heartbeats or other symptoms, regular health checkups may be all that’s needed. […] If you have Ebstein anomaly, you may get medicine to: […] Surgery is typically done if Ebstein anomaly causes severe tricuspid regurgitation and there is heart failure or increasing difficulty with exercise. […] Surgery also may be recommended if other symptoms, such as some irregular heartbeats, are very bad or affecting quality of life. […] If surgery is needed, it’s important to choose a surgeon who’s familiar with Ebstein anomaly. […] Surgery to treat Ebstein anomaly and related heart conditions may include:

• #9 Ebstein Anomaly treatment by the Irish Congenital Heart Centre

  https://congenitalheartcentre.ie/congenital-heart-conditions/ebstein-anomaly/

  If you have no signs or symptoms associated with Ebstein anomaly, careful monitoring of your heart may be all that’s necessary. […] Treatment for Ebstein anomaly may be necessary. Treatment options include medications and surgery. […] Treatment of Ebstein anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias. Treatments may include: […] If you have heart rhythm disturbances, medications may help control your heart rate and maintain normal heart rhythm. […] Your doctor may recommend surgery when your symptoms are affecting your quality of life. […] Several different types of procedures can be used to surgically treat Ebstein anomaly and associated defects, including:

• #10 Ebstein’s Anomaly – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/ebstein-s-anomaly/

  When is treatment needed? […] What types of treatment are indicated? Medications will not stop problems from developing in Ebsteins anomaly. But if you are having symptoms, certain medications might be helpful. These include diuretics to treat fluid retention and/or medications that treat abnormal heart rhythms. An ablation might also be done. Your doctor might also prescribe a blood thinner if you have atrial flutter or atrial fibrillation. Repair or replacement of the tricuspid valve may be indicated. This might be necessary if you develop symptoms and/or if your symptoms are worsening. […] It is important to find a surgeon who has expertise in performing surgery on patients with Ebsteins anomaly. The goal of surgery is to enable the leaflets to open and close correctly. The preferred surgical method is repairing the valve because it uses the patients own tissue. This can be done if there is enough tissue present. It is very complex surgery. If the existing valve cannot be repaired, it can be replaced with a tissue valve. Mechanical valves are rarely used for replacement in Ebsteins anomaly. […] In the most severe cases, if the valve is seriously deformed, heart function is poor and/or other treatments are not effective, a heart transplant may be an option.

• #11 Ebstein Anomaly | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/ebstein-anomaly

  Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. […] If the deformity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. […] If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. […] If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. […] Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.

• #12 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Although survival has significantly improved in the last four decades, the diagnosis of Ebsteins anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. […] A wait-and-see approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebsteins anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome. […] Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention. […] The effect of medical therapy on patients with EA has not yet been solidly investigated.

• #13 Ebstein’s Anomaly: Symptoms and Treatment | Doctor

  https://patient.info/doctor/ebsteins-anomaly-pro

  Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. […] Tricuspid valve repair is the goal of operative intervention. Repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation. […] Non-surgical management of Ebstein’s anomaly includes: prevention of infective endocarditis, management of heart failure, and treatment of arrhythmias. […] Increasingly the trend is for operative intervention early in the development of heart failure. Complete repair of Ebstein’s anomaly in symptomatic neonates has been shown to be possible, with good survival and excellent functional status.

• #14 Ebstein Anomaly | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/ebstein-anomaly

  Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. […] If the deformity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. […] If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. […] If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. […] Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.

• #15 Ebstein anomaly – UF Health

  https://ufhealth.org/conditions-and-treatments/ebstein-anomaly

  Treatment depends on the severity of the defect and the specific symptoms. Medical care may include: […] Surgery to correct the valve. […] Replacement of the tricuspid valve. This may be needed for children who continue to worsen or who have more serious complications.

• #16 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary. […] Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin. […] Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease.

• #17 Ebstein Anomaly Medication: Diuretics, Cardiac glycosides, Angiotensin-converting enzyme inhibitors

  https://emedicine.medscape.com/article/154447-medication

  Ebstein anomaly requires drug treatment for cardiovascular consequences resulting from tricuspid atrialization of the right ventricle, valvular regurgitation, and septal defects. Patients may require antibiotic prophylaxis for bacterial endocarditis. Treatment of SVT is typically by radiofrequency ablation rather than drug therapy. CHF is treated with ACE inhibitors, diuretics, and digoxin. […] ACE inhibitors are beneficial in all stages of chronic heart failure. Dyspnea and exercise tolerance are improved. Unlike diuretics, studies demonstrate improvement of survival and reduced progression of mild or moderate heart failure to more severe stages. They have benefit in asymptomatic left ventricular dysfunction.

• #18 Ebstein Anomaly

  https://www.rwjbh.org/treatment-care/heart-and-vascular-care/diseases-conditions/congenital-heart-disease/ebstein-anomaly/

  Treatment for Ebstein anomaly depends on the severity of the condition, whether signs or symptoms are present, and if the condition is getting worse. If symptoms are mild, your doctor will continue to monitor via regular follow-up appointments. Other treatment options include lifestyle changes, medications, and medical and surgical procedures. […] Antiarrhythmic medications will help control your hearts rhythm. Anticoagulants blood-thinners will help treat, prevent, and reduce blood clots. Beta blockers will help reduce your blood pressure. Diuretics water pills will help reduce the amount of fluid retention in your body. […] Tricuspid valve repair or replacement, Atrial septal defect surgery (includes transcatheter option), Maze procedure, Ablation therapy.

• #19 Ebsteins anomaly of the tricuspid valve medical therapy – wikidoc

  https://www.wikidoc.org/index.php/Ebsteins_anomaly_of_the_tricuspid_valve_medical_therapy

  Medical management of patients with Ebsteins anomaly consists of supportive care such as control of the heart rhythm with antiarrhythmic drugs, inotropic agents and diuretics for heart failure, anticoagulation in patients with atrial fibrillation and paradoxical embolization. […] Some Ebstein’s anomaly patients present with an (antidromic) AV nodal reentrant tachycardia with associated pre-excitation. Among these patients, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers and digoxin are contraindicated. If there is atrial fibrillation with pre-excitation, treatment options include procainamide, flecainide, propafenone, dofetilide and ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response.

• #20 Ebstein’s Anomaly: Comprehensive Guide to Symptoms and Treatment – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/ebsteins-anomaly-comprehensive-guide-to-symptoms-and-treatment/

  Digoxin helps the heart beat more strongly and with a more regular rhythm by increasing the force of heart contractions and controlling heart rate. Digoxin is often prescribed for patients with Ebstein’s anomaly who have heart failure or certain arrhythmias. It is typically used when other medications are insufficient. Patients can expect improved heart function and a more regular heartbeat within a few weeks of starting digoxin. […] […] Antiarrhythmic medications help control abnormal heart rhythms by stabilizing the heart’s electrical signals. They are used to prevent or treat arrhythmias. These medications are often prescribed for patients with Ebstein’s anomaly who experience frequent or severe arrhythmias. They may be used in combination with other treatments like beta-blockers or anticoagulants. Patients can expect fewer arrhythmia episodes and improved heart function within weeks of starting antiarrhythmics. […]

• #21 Ebstein’s anomaly – Wikipedia

  https://en.wikipedia.org/wiki/Ebstein%27s_anomaly

  Ebstein’s cardio physiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred medication treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated. […] If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response. […] The Canadian Cardiovascular Society (CCS) recommends surgical intervention for these indications: Limited exercise capacity (NYHA III-IV), Increasing heart size (cardiothoracic ratio greater than 65%), Important cyanosis (resting oxygen saturation less than 90% – level B), Severe tricuspid regurgitation with symptoms, Transient ischemic attack or stroke. […] The CCS further recommends patients who require operation for Ebstein’s anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.

• #22 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Class IC and III anti-arrhythmic drugs may help to control the arrhythmic burden, however, there is general agreement to avoid sotalol and amiodarone as long-term therapy due to their known proarrhythmic risk and side effects. […] Cardiac resynchronisation therapy can be applied in case of bundle branch block and either LV or RV dysfunction. […] Monocusp reconstruction for surgical treatment of EA had been used for many years. […] Nonetheless, there is nowadays a general consensus on adopting a more anatomical form of repair, described by da Silva et al in 2007. […] The concept of da Silvas cone repair (CR) is to perform a surgical delamination of all the rescuable leaflets in order to obtain a 360 TV annulus in anatomical position. […] Current indication to surgical intervention is severe TR with symptoms or, in the absence of symptoms, with progression in either right-sided heart dilation or fRV systolic dysfunction. […] Nonetheless, the surgical mortality risk for younger patients is not negligible even in experienced centres and, in the absence of randomized control studies, whether an early operation may effectively prevent complications of EA natural history is still debated.

• #23 Ebsteins anomaly of the tricuspid valve medical therapy – wikidoc

  https://www.wikidoc.org/index.php/Ebsteins_anomaly_of_the_tricuspid_valve_medical_therapy

  Medical management of patients with Ebsteins anomaly consists of supportive care such as control of the heart rhythm with antiarrhythmic drugs, inotropic agents and diuretics for heart failure, anticoagulation in patients with atrial fibrillation and paradoxical embolization. […] Some Ebstein’s anomaly patients present with an (antidromic) AV nodal reentrant tachycardia with associated pre-excitation. Among these patients, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers and digoxin are contraindicated. If there is atrial fibrillation with pre-excitation, treatment options include procainamide, flecainide, propafenone, dofetilide and ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response.

• #24 Ebstein’s anomaly – Wikipedia

  https://en.wikipedia.org/wiki/Ebstein%27s_anomaly

  Ebstein’s cardio physiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred medication treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated. […] If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response. […] The Canadian Cardiovascular Society (CCS) recommends surgical intervention for these indications: Limited exercise capacity (NYHA III-IV), Increasing heart size (cardiothoracic ratio greater than 65%), Important cyanosis (resting oxygen saturation less than 90% – level B), Severe tricuspid regurgitation with symptoms, Transient ischemic attack or stroke. […] The CCS further recommends patients who require operation for Ebstein’s anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.

• #25 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary. […] Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin. […] Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease.

• #26 Ebstein Anomaly Medication: Diuretics, Cardiac glycosides, Angiotensin-converting enzyme inhibitors

  https://emedicine.medscape.com/article/154447-medication

  Ebstein anomaly requires drug treatment for cardiovascular consequences resulting from tricuspid atrialization of the right ventricle, valvular regurgitation, and septal defects. Patients may require antibiotic prophylaxis for bacterial endocarditis. Treatment of SVT is typically by radiofrequency ablation rather than drug therapy. CHF is treated with ACE inhibitors, diuretics, and digoxin. […] ACE inhibitors are beneficial in all stages of chronic heart failure. Dyspnea and exercise tolerance are improved. Unlike diuretics, studies demonstrate improvement of survival and reduced progression of mild or moderate heart failure to more severe stages. They have benefit in asymptomatic left ventricular dysfunction.

• #27 Ebstein’s Anomaly: Comprehensive Guide to Symptoms and Treatment – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/ebsteins-anomaly-comprehensive-guide-to-symptoms-and-treatment/

  ACE inhibitors relax blood vessels and lower blood pressure by inhibiting a specific enzyme, reducing strain on the heart and slowing the progression of heart failure. ACE inhibitors are often used in patients with Ebstein’s anomaly who have heart failure or high blood pressure. They are typically prescribed if other medications, like diuretics or beta-blockers, are insufficient. Patients may notice improved heart function and reduced heart failure symptoms within a few weeks of starting ACE inhibitors. […] […] Anticoagulants, or blood thinners, help prevent blood clots by reducing the blood’s ability to clot, which is crucial for patients with abnormal heart rhythms that increase the risk of stroke. These medications are prescribed for patients with Ebstein’s anomaly who have atrial fibrillation or other arrhythmias. They are typically used long-term to prevent complications like stroke. Patients taking anticoagulants can expect a reduced risk of stroke or other clot-related complications, but regular monitoring is necessary to ensure safe use. […]

• #28 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Medical Management In infants with Ebstein anomaly, the mainstay of treatment is supportive to reduce pulmonary vascular resistance and hypoxemia. In symptomatic infants with either heart failure or cyanosis, inhaled nitric oxide can reduce pulmonary vascular resistance. In cyanotic infants, prostaglandin E1 (PGE1) infusion can maintain ductus arteriosus patency and lower pulmonary vascular resistance by increasing pulmonary vasodilation. Newborns with heart failure and cardiogenic shock may require inotropic therapy; milrinone is the drug of choice since it contributes to pulmonary vasodilation. Symptoms of heart failure are treated with loop diuretics and guideline-directed medical therapy. Patients with supraventricular tachyarrhythmias can receive rate-control medications such as beta blockers or calcium channel blockers; if ineffective, the class I antiarrhythmic procainamide or class III antiarrhythmic amiodarone or sotalol can be used to treat paroxysmal atrial fibrillation. Patients with intractable arrhythmias frequently require catheter ablation.

• #29 Ebstein’s Anomaly: Comprehensive Guide to Symptoms and Treatment – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/ebsteins-anomaly-comprehensive-guide-to-symptoms-and-treatment/

  Diuretics, commonly known as “water pills,” help the body eliminate excess fluid by increasing urine production. This reduces the heart’s workload by lowering blood volume and decreasing swelling. Diuretics are typically prescribed for patients with Ebstein’s anomaly who experience fluid retention or symptoms of heart failure. They are often used alongside other medications to manage symptoms, rather than as a first-line treatment. Patients may notice reduced swelling and improved breathing within days to weeks of starting diuretics. However, these medications do not address the underlying congenital heart defect. […] […] Beta-blockers work by reducing heart rate and lowering blood pressure, helping the heart pump more efficiently and reducing the risk of arrhythmias. These medications are often prescribed to patients with Ebstein’s anomaly who have arrhythmias or high blood pressure. They are usually introduced after lifestyle changes or other medications have not sufficiently controlled symptoms. Patients can expect a more regular heart rhythm and improved heart function within weeks of starting beta-blockers. […]

• #30 Ebstein Anomaly

  https://www.rwjbh.org/treatment-care/heart-and-vascular-care/diseases-conditions/congenital-heart-disease/ebstein-anomaly/

  Treatment for Ebstein anomaly depends on the severity of the condition, whether signs or symptoms are present, and if the condition is getting worse. If symptoms are mild, your doctor will continue to monitor via regular follow-up appointments. Other treatment options include lifestyle changes, medications, and medical and surgical procedures. […] Antiarrhythmic medications will help control your hearts rhythm. Anticoagulants blood-thinners will help treat, prevent, and reduce blood clots. Beta blockers will help reduce your blood pressure. Diuretics water pills will help reduce the amount of fluid retention in your body. […] Tricuspid valve repair or replacement, Atrial septal defect surgery (includes transcatheter option), Maze procedure, Ablation therapy.

• #31 Ebsteins anomaly of the tricuspid valve medical therapy – wikidoc

  https://www.wikidoc.org/index.php/Ebsteins_anomaly_of_the_tricuspid_valve_medical_therapy

  Warfarin is recommended for patients with Ebsteins anomaly with a history of paradoxical embolus or atrial fibrillation. […] Diuretics may aid in reducing volume overload. Cardiac glycosides like digoxin can be used to maintain proper heart rhythm and increase contractility. […] Anticoagulation with warfarin is recommended for patients with Ebsteins anomaly with a history of paradoxical embolus or atrial fibrillation.

• #32 Ebstein’s Anomaly: Comprehensive Guide to Symptoms and Treatment – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/ebsteins-anomaly-comprehensive-guide-to-symptoms-and-treatment/

  ACE inhibitors relax blood vessels and lower blood pressure by inhibiting a specific enzyme, reducing strain on the heart and slowing the progression of heart failure. ACE inhibitors are often used in patients with Ebstein’s anomaly who have heart failure or high blood pressure. They are typically prescribed if other medications, like diuretics or beta-blockers, are insufficient. Patients may notice improved heart function and reduced heart failure symptoms within a few weeks of starting ACE inhibitors. […] […] Anticoagulants, or blood thinners, help prevent blood clots by reducing the blood’s ability to clot, which is crucial for patients with abnormal heart rhythms that increase the risk of stroke. These medications are prescribed for patients with Ebstein’s anomaly who have atrial fibrillation or other arrhythmias. They are typically used long-term to prevent complications like stroke. Patients taking anticoagulants can expect a reduced risk of stroke or other clot-related complications, but regular monitoring is necessary to ensure safe use. […]

• #33 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Medical Management In infants with Ebstein anomaly, the mainstay of treatment is supportive to reduce pulmonary vascular resistance and hypoxemia. In symptomatic infants with either heart failure or cyanosis, inhaled nitric oxide can reduce pulmonary vascular resistance. In cyanotic infants, prostaglandin E1 (PGE1) infusion can maintain ductus arteriosus patency and lower pulmonary vascular resistance by increasing pulmonary vasodilation. Newborns with heart failure and cardiogenic shock may require inotropic therapy; milrinone is the drug of choice since it contributes to pulmonary vasodilation. Symptoms of heart failure are treated with loop diuretics and guideline-directed medical therapy. Patients with supraventricular tachyarrhythmias can receive rate-control medications such as beta blockers or calcium channel blockers; if ineffective, the class I antiarrhythmic procainamide or class III antiarrhythmic amiodarone or sotalol can be used to treat paroxysmal atrial fibrillation. Patients with intractable arrhythmias frequently require catheter ablation.

• #34 Ebstein Anomaly – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/ebstein-anomaly

  Treatment is with medications and surgery, and sometimes with interventional catheterization. […] Medications play a role in managing neonates who are severely cyanotic and have inadequate pulmonary blood flow. Maintaining ductal patency with prostaglandins allows time for the pulmonary vascular resistance to drop. The decrease in pulmonary artery pressure allows the compromised right ventricle to provide adequate pulmonary blood flow. Some neonates with very severe Ebstein anomaly and functional compromise of the right ventricle require more prolonged palliation with a Blalock-Thomas-Taussig (BTT) shunt or other complex surgical interventions. […] After the first several months of age, surgical repair of the tricuspid valve should be considered when any symptoms are present or there is evidence of progressive tricuspid valve dysfunction or enlargement of the right heart chambers. Surgical treatment of this anomaly has evolved over the years with developments such as the cone procedure. This approach to repair of the tricuspid valve involves delamination and mobilization of the tricuspid valve leaflets, plicating the atrialized portion of the right ventricle and the annulus and reattaching newly created „leaflet cone” to the true tricuspid annulus. These advances have greatly enhanced the surgeon’s ability to achieve an excellent functional repair of even the most deformed tricuspid valves. Any atrial defect is closed at the same time. […] Interventional catheterization techniques may be used to close an atrial communication when cyanosis is present, but the tricuspid valve dysfunction is only modest.

• #35 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #36 Ebstein’s Anomaly Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/ebsteins-anomaly

  After delivery, your baby will need prostaglandin (PGE), an intravenous medication that keeps the patent ductus arteriosus (a normal connection present in babies in the womb) open. The patent ductus arteriosus (PDA) will allow blood flow to the lungs. […] For mild cases, medication may be all your child needs. For more severe cases, surgery in the newborn period may be necessary. […] There are a few possible surgeries, depending on the nature of the heart defect and the age of your child at the time of surgery: […] Surgical valve repair: If possible, the surgeon repairs the structure of the tricuspid valve. […] Tricuspid valve replacement: If the tricuspid valve cannot be surgically repaired, surgeons can replace the existing valve. […] Three-staged surgical procedures: In severe cases of Ebsteins Anomaly, the right ventricle may be too small to do the work of the right heart. In this case three surgeries will be performed.

• #37 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary. […] Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin. […] Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease.

• #38 Ebstein’s Anomaly | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/ebsteins-anomaly

  Treatment depends on the severity of the child’s anomaly. Children with a mild anomaly may be able to use medication alone to control symptoms, including: […] Children who have arrhythmias and heartbeat irregularities that cannot be controlled by medication may need a procedure, called radiofrequency catheter ablation, to treat these heart arrhythmias. […] There are several surgical options for repairing your child’s tricuspid valve and repairing other defects that are associated with Ebstein’s anomaly. These techniques include: […] In children who can’t be treated with a catheter, arrhythmias are addressed at the time of surgery with surgical ablation. […] Treatments for Ebstein’s anomaly are continually being refined, and the long-term outlook is continually improving. Most children who’ve had surgery recover and grow normally.

• #39 Ebstein Anomaly (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/ebstein-anomaly.html

  Doctors may recommend an electrophysiology study (EP study) when there are serious abnormal heart rhythms. During this study, ablation (freezing or burning) can get rid of electrical pathway fibers that make the heart beat too fast. […] Babies with Ebstein anomaly need care from a pediatric cardiologist (a doctor who diagnoses and treats children’s heart problems) because the heart’s pumping ability may fall short as the child grows. The doctor will recommend treatment that is tailored to a child’s needs. […] Many children whose Ebstein anomaly was corrected by surgery can be as active as other kids.

• #40 Ebstein Anomaly | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/ebstein-anomaly

  Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. […] If the deformity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. […] If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. […] If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. […] Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.

• #41 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #42 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #43 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #44 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #45 Ebstein’s Anomaly – Leeds Teaching Hospitals NHS Trust

  https://www.leedsth.nhs.uk/patients/resources/ebsteins-anomaly/

  We may consider medications to thin the blood to reduce the risk of blood clots that may go across the small hole in your heart. If you have had a stroke or go particularly blue, due to low oxygen in the blood, we may suggest a keyhole procedure to close the small hole. If your tricuspid valve is very severely affected and leaky, sometimes we operate to repair or replace the abnormal heart valve.

• #46 Ebstein’s Anomaly | Schneeweiss Adult Congenital Heart Center

  https://www.congenitalheart.cuimc.columbia.edu/conditions-we-treat/ebsteins-anomaly

  Such patients should be evaluated at an experienced Adult Congenital Heart Disease center to determine if they should undergo cardiac surgery and to assess arrhythmia treatment options. […] Although most of these arrhythmias can be successfully treated with catheter ablation, management is more complicated because of the anatomy and should be approached in conjunction with an assessment of the tricuspid valve and right ventricle. […] Some of these patients may be candidates for device closure of the atrial septal defect to eliminate cyanosis from the right to left shunt. However, before embarking on this approach it should be certain that the degree of tricuspid regurgitation is only mild. […] Ebstein’s anomaly is a complicated lesion, and management of adolescents and adults at an experienced center is advisable.

• #47 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Consult an electrophysiologist and cardiac surgeon for the management of patients with Ebstein anomaly. […] Surgical care includes correction of the underlying tricuspid valve and right ventricular abnormalities, correction of any associated intracardiac defects, palliative procedures in early days of life as a bridge to more definitive surgical treatment later, and surgical treatment of associated arrhythmias. […] The trend is to perform surgery earlier rather later in the course of heart failure. Indications for surgery are generally as follows: New York Heart Association (NYHA) class I-II heart failure with worsening symptoms or with a cardiothoracic ratio of 0.65 or greater, NYHA class III-IV heart failure, history of paradoxical embolism, significant cyanosis with arterial O2 saturation of 80% or less and/or polycythemia with hemoglobin of 16 g/dL or more, arrhythmias refractory to medical and radiofrequency ablation.

• #48 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Surgical Management Newborns with Ebstein anomaly are initially observed as the pulmonary vascular resistance decreases. If surgery is required, a systemic-to-pulmonary artery shunt is typically employed to relieve cyanosis and secure adequate pulmonary and systemic circulations. Due to high periprocedural mortality, more definitive surgery is usually delayed unless the neonate meets specific indications. Data show that delaying surgery until heart failure or RV systolic dysfunction occurs is associated with worse outcomes. This is thought to be because Ebstein anomaly is a myopathic disorder in addition to abnormal valves, and the myopathic RV may be unable to tolerate increased volumes. Indications for surgery in neonates with Ebstein anomaly include: Right heart failure due to severe tricuspid regurgitation, A cardiothoracic ratio 80%, Severe cyanosis with dependency on PGE1, Mechanical ventilation dependency. In children and adults with Ebstein anomaly, indications for surgery include: Heart failure symptoms (New York Heart Association functional class III or IV), Progressive exercise intolerance, Evidence of RV dysfunction or progressive RV dilatation, Evidence of paradoxical emboli, Arrhythmias refractory to medical and catheter ablation therapies, Cyanosis with oxygen saturations 90%, Severe tricuspid regurgitation, Significant concomitant lesions such as pulmonic stenosis or atrial or ventricular septal defects, Progressive cardiomegaly, Cardiothoracic ratio 65%, Reduced left ventricular function. Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. For atrial arrhythmias in these patients, catheter ablation carries risks of paradoxical emboli, given the high incidence of interatrial shunts. A surgical biatrial Maze procedure during valvular repair is recommended. Multiple surgical approaches have been described for the treatment of Ebstein anomaly. Cone reconstruction is the currently preferred surgical approach in young children and adults. This approach involves mobilizing the anterior and posterior leaflets from their anomalous attachments, rotating the detached edges of these leaflets clockwise, and suturing them to the septal edge of the anterior leaflet at the level of the tricuspid valve annulus. The Danielson repair is the plication of the atrialized portion of the RV to narrow the size of the tricuspid valve and create a monoleaflet tricuspid valve that is competent. The Carpentier monocusp repair plicates the atrialized portion of the RV, implants a ring within the tricuspid annulus to provide support, mobilizes the anterior tricuspid valve leaflet, and attaches it to the annulus anteriorly. When the native tricuspid valve cannot be repaired, especially in older patients, it can be replaced with a bioprosthetic or mechanical valve. A bioprosthetic valve might require temporary anticoagulation; a mechanical valve will require lifelong anticoagulation. Mechanical valves are avoided when RV dysfunction is severe to mitigate disc motion abnormalities and the increased risk of valve thrombosis.

• #49 Ebstein’s anomaly – Wikipedia

  https://en.wikipedia.org/wiki/Ebstein%27s_anomaly

  Ebstein’s cardio physiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred medication treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated. […] If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response. […] The Canadian Cardiovascular Society (CCS) recommends surgical intervention for these indications: Limited exercise capacity (NYHA III-IV), Increasing heart size (cardiothoracic ratio greater than 65%), Important cyanosis (resting oxygen saturation less than 90% – level B), Severe tricuspid regurgitation with symptoms, Transient ischemic attack or stroke. […] The CCS further recommends patients who require operation for Ebstein’s anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.

• #50 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Surgical Management Newborns with Ebstein anomaly are initially observed as the pulmonary vascular resistance decreases. If surgery is required, a systemic-to-pulmonary artery shunt is typically employed to relieve cyanosis and secure adequate pulmonary and systemic circulations. Due to high periprocedural mortality, more definitive surgery is usually delayed unless the neonate meets specific indications. Data show that delaying surgery until heart failure or RV systolic dysfunction occurs is associated with worse outcomes. This is thought to be because Ebstein anomaly is a myopathic disorder in addition to abnormal valves, and the myopathic RV may be unable to tolerate increased volumes. Indications for surgery in neonates with Ebstein anomaly include: Right heart failure due to severe tricuspid regurgitation, A cardiothoracic ratio 80%, Severe cyanosis with dependency on PGE1, Mechanical ventilation dependency. In children and adults with Ebstein anomaly, indications for surgery include: Heart failure symptoms (New York Heart Association functional class III or IV), Progressive exercise intolerance, Evidence of RV dysfunction or progressive RV dilatation, Evidence of paradoxical emboli, Arrhythmias refractory to medical and catheter ablation therapies, Cyanosis with oxygen saturations 90%, Severe tricuspid regurgitation, Significant concomitant lesions such as pulmonic stenosis or atrial or ventricular septal defects, Progressive cardiomegaly, Cardiothoracic ratio 65%, Reduced left ventricular function. Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. For atrial arrhythmias in these patients, catheter ablation carries risks of paradoxical emboli, given the high incidence of interatrial shunts. A surgical biatrial Maze procedure during valvular repair is recommended. Multiple surgical approaches have been described for the treatment of Ebstein anomaly. Cone reconstruction is the currently preferred surgical approach in young children and adults. This approach involves mobilizing the anterior and posterior leaflets from their anomalous attachments, rotating the detached edges of these leaflets clockwise, and suturing them to the septal edge of the anterior leaflet at the level of the tricuspid valve annulus. The Danielson repair is the plication of the atrialized portion of the RV to narrow the size of the tricuspid valve and create a monoleaflet tricuspid valve that is competent. The Carpentier monocusp repair plicates the atrialized portion of the RV, implants a ring within the tricuspid annulus to provide support, mobilizes the anterior tricuspid valve leaflet, and attaches it to the annulus anteriorly. When the native tricuspid valve cannot be repaired, especially in older patients, it can be replaced with a bioprosthetic or mechanical valve. A bioprosthetic valve might require temporary anticoagulation; a mechanical valve will require lifelong anticoagulation. Mechanical valves are avoided when RV dysfunction is severe to mitigate disc motion abnormalities and the increased risk of valve thrombosis.

• #51 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Surgical Management Newborns with Ebstein anomaly are initially observed as the pulmonary vascular resistance decreases. If surgery is required, a systemic-to-pulmonary artery shunt is typically employed to relieve cyanosis and secure adequate pulmonary and systemic circulations. Due to high periprocedural mortality, more definitive surgery is usually delayed unless the neonate meets specific indications. Data show that delaying surgery until heart failure or RV systolic dysfunction occurs is associated with worse outcomes. This is thought to be because Ebstein anomaly is a myopathic disorder in addition to abnormal valves, and the myopathic RV may be unable to tolerate increased volumes. Indications for surgery in neonates with Ebstein anomaly include: Right heart failure due to severe tricuspid regurgitation, A cardiothoracic ratio 80%, Severe cyanosis with dependency on PGE1, Mechanical ventilation dependency. In children and adults with Ebstein anomaly, indications for surgery include: Heart failure symptoms (New York Heart Association functional class III or IV), Progressive exercise intolerance, Evidence of RV dysfunction or progressive RV dilatation, Evidence of paradoxical emboli, Arrhythmias refractory to medical and catheter ablation therapies, Cyanosis with oxygen saturations 90%, Severe tricuspid regurgitation, Significant concomitant lesions such as pulmonic stenosis or atrial or ventricular septal defects, Progressive cardiomegaly, Cardiothoracic ratio 65%, Reduced left ventricular function. Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. For atrial arrhythmias in these patients, catheter ablation carries risks of paradoxical emboli, given the high incidence of interatrial shunts. A surgical biatrial Maze procedure during valvular repair is recommended. Multiple surgical approaches have been described for the treatment of Ebstein anomaly. Cone reconstruction is the currently preferred surgical approach in young children and adults. This approach involves mobilizing the anterior and posterior leaflets from their anomalous attachments, rotating the detached edges of these leaflets clockwise, and suturing them to the septal edge of the anterior leaflet at the level of the tricuspid valve annulus. The Danielson repair is the plication of the atrialized portion of the RV to narrow the size of the tricuspid valve and create a monoleaflet tricuspid valve that is competent. The Carpentier monocusp repair plicates the atrialized portion of the RV, implants a ring within the tricuspid annulus to provide support, mobilizes the anterior tricuspid valve leaflet, and attaches it to the annulus anteriorly. When the native tricuspid valve cannot be repaired, especially in older patients, it can be replaced with a bioprosthetic or mechanical valve. A bioprosthetic valve might require temporary anticoagulation; a mechanical valve will require lifelong anticoagulation. Mechanical valves are avoided when RV dysfunction is severe to mitigate disc motion abnormalities and the increased risk of valve thrombosis.

• #52 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Surgical options include a biventricular repair pathway with a valvuloplasty. […] Patients with EA with anatomic pulmonary atresia, a circular shunt, dependency on prostaglandins or mechanical ventilation, worsening cyanosis or heart failure with inability to tolerate feeds due to mesenteric congestion, and generally severe forms such as Carpentier type C/D or GOSE score 3 will require a surgical intervention during the neonatal period. […] The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, reduction right atrioplasty, fenestrated or partial closure of the atrial septum, establishment of RV-PA continuity in case of pulmonary valve atresia, and closure of the VSD if present. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #53 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Surgical options include a biventricular repair pathway with a valvuloplasty. […] Patients with EA with anatomic pulmonary atresia, a circular shunt, dependency on prostaglandins or mechanical ventilation, worsening cyanosis or heart failure with inability to tolerate feeds due to mesenteric congestion, and generally severe forms such as Carpentier type C/D or GOSE score 3 will require a surgical intervention during the neonatal period. […] The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, reduction right atrioplasty, fenestrated or partial closure of the atrial septum, establishment of RV-PA continuity in case of pulmonary valve atresia, and closure of the VSD if present. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #54 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Various approaches are available to treat structural abnormalities. Tricuspid valve repair is preferred over valve replacement, and bioprosthetic valves are preferred over mechanical prosthetic valves. […] In a study of 27 consecutive cone reconstructions to treat severe tricuspid valve (TV) regurgitation associated with Ebstein anomaly, Ibrahim and colleagues retrospectively compared the clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data between preoperative baseline and follow-up. […] Da Silva’s cone repair is a technique for the surgical reconstruction of the tricuspid valve and the right ventricle in Ebstein anomaly. […] Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt.

• #55 Ebstein’s Anomaly: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16946-ebsteins-anomaly-for-adults

  Treatment depends on how severe your symptoms are. If you have mild or no symptoms, your provider may monitor your heart and watch for changes. […] If you have signs of heart failure or arrhythmia, you may receive noninvasive treatments, such as: […] For more severe symptoms, tricuspid valve surgery may be the best option to provide lasting improvement in valve function. If there is enough tissue present, surgeons can repair the valve. This is the preferred treatment because it uses your own tissue. […] If repair isn’t an option, it’s possible to insert a replacement mechanical valve or one made of biological tissue. If you receive a mechanical valve, you will need to take blood-thinning medication for the rest of your life. […] Your provider may combine tricuspid valve surgery with other heart procedures. This may include repair of the hole between the heart’s upper chambers or arrhythmia treatment. There are many types of treatment for arrhythmia, including catheter ablation or a pacemaker. Rarely, you may need a heart transplant if other treatments have failed. […] If this occurs, you may need surgery to repair or replace your tricuspid valve and fix other related heart problems.

• #56 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #57 Ebstein’s Anomaly | Da Silva Center for Ebstein’s Anomaly | UPMC

  https://www.chp.edu/our-services/heart/cardiothoracic-surgery/da-silva-center/ebsteins-anomaly

  Ebstein’s anomaly treatments depend on the extent of the defect. Some potential treatments include: […] A variety of medications may help with treating Ebstein’s anomaly or to manage side effects from the condition. For example: […] A cone procedure, pioneered by Jose da Silva, MD, is the standard treatment for children with Ebsteins anomaly. […] It uses tissue from a child’s own malfunctioning tricuspid valve to create a new, cone-shaped valve. […] Other, less common surgeries for Ebsteins anomaly include: […] The Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital of Pittsburgh provides world-class care for people with congenital heart defects. Our experts are among the most experienced in the world at treating children with Ebsteins anomaly and other heart valve problems.

• #58 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Class IC and III anti-arrhythmic drugs may help to control the arrhythmic burden, however, there is general agreement to avoid sotalol and amiodarone as long-term therapy due to their known proarrhythmic risk and side effects. […] Cardiac resynchronisation therapy can be applied in case of bundle branch block and either LV or RV dysfunction. […] Monocusp reconstruction for surgical treatment of EA had been used for many years. […] Nonetheless, there is nowadays a general consensus on adopting a more anatomical form of repair, described by da Silva et al in 2007. […] The concept of da Silvas cone repair (CR) is to perform a surgical delamination of all the rescuable leaflets in order to obtain a 360 TV annulus in anatomical position. […] Current indication to surgical intervention is severe TR with symptoms or, in the absence of symptoms, with progression in either right-sided heart dilation or fRV systolic dysfunction. […] Nonetheless, the surgical mortality risk for younger patients is not negligible even in experienced centres and, in the absence of randomized control studies, whether an early operation may effectively prevent complications of EA natural history is still debated.

• #59 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Various approaches are available to treat structural abnormalities. Tricuspid valve repair is preferred over valve replacement, and bioprosthetic valves are preferred over mechanical prosthetic valves. […] In a study of 27 consecutive cone reconstructions to treat severe tricuspid valve (TV) regurgitation associated with Ebstein anomaly, Ibrahim and colleagues retrospectively compared the clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data between preoperative baseline and follow-up. […] Da Silva’s cone repair is a technique for the surgical reconstruction of the tricuspid valve and the right ventricle in Ebstein anomaly. […] Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt.

• #60 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Surgical Management Newborns with Ebstein anomaly are initially observed as the pulmonary vascular resistance decreases. If surgery is required, a systemic-to-pulmonary artery shunt is typically employed to relieve cyanosis and secure adequate pulmonary and systemic circulations. Due to high periprocedural mortality, more definitive surgery is usually delayed unless the neonate meets specific indications. Data show that delaying surgery until heart failure or RV systolic dysfunction occurs is associated with worse outcomes. This is thought to be because Ebstein anomaly is a myopathic disorder in addition to abnormal valves, and the myopathic RV may be unable to tolerate increased volumes. Indications for surgery in neonates with Ebstein anomaly include: Right heart failure due to severe tricuspid regurgitation, A cardiothoracic ratio 80%, Severe cyanosis with dependency on PGE1, Mechanical ventilation dependency. In children and adults with Ebstein anomaly, indications for surgery include: Heart failure symptoms (New York Heart Association functional class III or IV), Progressive exercise intolerance, Evidence of RV dysfunction or progressive RV dilatation, Evidence of paradoxical emboli, Arrhythmias refractory to medical and catheter ablation therapies, Cyanosis with oxygen saturations 90%, Severe tricuspid regurgitation, Significant concomitant lesions such as pulmonic stenosis or atrial or ventricular septal defects, Progressive cardiomegaly, Cardiothoracic ratio 65%, Reduced left ventricular function. Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. For atrial arrhythmias in these patients, catheter ablation carries risks of paradoxical emboli, given the high incidence of interatrial shunts. A surgical biatrial Maze procedure during valvular repair is recommended. Multiple surgical approaches have been described for the treatment of Ebstein anomaly. Cone reconstruction is the currently preferred surgical approach in young children and adults. This approach involves mobilizing the anterior and posterior leaflets from their anomalous attachments, rotating the detached edges of these leaflets clockwise, and suturing them to the septal edge of the anterior leaflet at the level of the tricuspid valve annulus. The Danielson repair is the plication of the atrialized portion of the RV to narrow the size of the tricuspid valve and create a monoleaflet tricuspid valve that is competent. The Carpentier monocusp repair plicates the atrialized portion of the RV, implants a ring within the tricuspid annulus to provide support, mobilizes the anterior tricuspid valve leaflet, and attaches it to the annulus anteriorly. When the native tricuspid valve cannot be repaired, especially in older patients, it can be replaced with a bioprosthetic or mechanical valve. A bioprosthetic valve might require temporary anticoagulation; a mechanical valve will require lifelong anticoagulation. Mechanical valves are avoided when RV dysfunction is severe to mitigate disc motion abnormalities and the increased risk of valve thrombosis.

• #61 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, and establishment of RV-PA continuity in case of pulmonary valve atresia. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #62 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Class IC and III anti-arrhythmic drugs may help to control the arrhythmic burden, however, there is general agreement to avoid sotalol and amiodarone as long-term therapy due to their known proarrhythmic risk and side effects. […] Cardiac resynchronisation therapy can be applied in case of bundle branch block and either LV or RV dysfunction. […] Monocusp reconstruction for surgical treatment of EA had been used for many years. […] Nonetheless, there is nowadays a general consensus on adopting a more anatomical form of repair, described by da Silva et al in 2007. […] The concept of da Silvas cone repair (CR) is to perform a surgical delamination of all the rescuable leaflets in order to obtain a 360 TV annulus in anatomical position. […] Current indication to surgical intervention is severe TR with symptoms or, in the absence of symptoms, with progression in either right-sided heart dilation or fRV systolic dysfunction. […] Nonetheless, the surgical mortality risk for younger patients is not negligible even in experienced centres and, in the absence of randomized control studies, whether an early operation may effectively prevent complications of EA natural history is still debated.

• #63 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, and establishment of RV-PA continuity in case of pulmonary valve atresia. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #64 Cone Procedure for Ebstein’s Anomaly | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/heart-surgery/programs-services/cone-procedure-ebsteins-anomaly

  A retrospective study published in May 2020 in the Journal of Thoracic and Cardiovascular Surgery compared outcomes from 151 patients with Ebsteins Anomaly. Results indicated the cone reconstruction had a higher success rate and lower incidence of tricuspid regurgitation when compared to valve replacement or other surgical interventions. According to Dr. Velez, Weve reached the point where we can say with confidence that the cone reconstruction procedure is a safe and highly effective treatment for children with Ebsteins Anomaly. Evidence has confirmed we can expect consistent results and long-term durability across all variations in tricuspid valve migration and dilation.

• #65 Ebstein Centre at the German Heart Centre Munich – Herz- und Gefäßchirugie – DHM

  https://www.deutsches-herzzentrum-muenchen.de/en/department-of-cardiovascular-surgery/ebstein-centre-at-the-german-heart-centre-munich/

  At the German Heart Centre Munich, we have been offering the most modern surgical method for the reconstruction of the tricuspid valve, the Cone operation, since 2010. Prof. Rdiger Lange learned this surgical method in person from the first person to describe it, Prof. Da Silva, and introduced it in Munich. It is a very complicated operation that is only performed in a few centres worldwide. Here, the valve is reconstructed with its own tissue, thus avoiding an artificial valve replacement. We have already treated many patients with very good results.

• #66 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

• #67 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

• #68 Congenital Defects Tutorial – Congenital Heart Defects | Atlas of Human Cardiac Anatomy

  https://www.vhlab.umn.edu/atlas/congenital-defects-tutorial/right-heart-lesions/ebsteins-anomaly.shtml

  Procedures include: median sternotomy, cannulation, cardiopulmonary bypass, cardioplegia, and hypothermia. The right atrium will be opened after inspection of the coronary vasculature. For the Carpentier technique the anterior leaflet is mobilized first. Afterwards vertical plication sutures will be applied under consideration of a satisfactory anterior leaflet reconstruction. The leaflet will then be advanced across the plicated area to reduce the size of the valve orifice. Finally, an annular ring can be sutured in to support the tricuspid valve.

• #69 Ebstein anomaly – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132

  If the valve can’t be repaired, surgery to replace the valve may be needed. […] This surgery is done to fix a hole between the upper chambers of the heart. […] If Ebstein anomaly causes irregular heartbeats, this treatment may be done during valve repair or replacement surgery. […] This treatment is done to correct fast or irregular heartbeats. […] If severe Ebstein anomaly is causing the heart to fail, a heart transplant might be needed.

• #70 Ebstein’s Anomaly: Symptoms and Treatment | Doctor

  https://patient.info/doctor/ebsteins-anomaly-pro

  In patients over 50 years of age with Ebstein’s anomaly, surgery is associated with good long-term survival and improved functional status, although long-term survival might be improved by performing surgery earlier. […] Tricuspid valve repair is preferred over valve replacement. Bioprosthetic valves are preferred over mechanical prosthetic valves. […] Both the atrialised portion of the right ventricle and the very dilated, thin-walled right atrium can be resected. […] Palliative procedures are usually reserved for severely ill infants with an otherwise poor prognosis. These include: creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through an aorto-pulmonary shunt. […] Heart transplantation is appropriate in selected patients.

• #71 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Various approaches are available to treat structural abnormalities. Tricuspid valve repair is preferred over valve replacement, and bioprosthetic valves are preferred over mechanical prosthetic valves. […] In a study of 27 consecutive cone reconstructions to treat severe tricuspid valve (TV) regurgitation associated with Ebstein anomaly, Ibrahim and colleagues retrospectively compared the clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data between preoperative baseline and follow-up. […] Da Silva’s cone repair is a technique for the surgical reconstruction of the tricuspid valve and the right ventricle in Ebstein anomaly. […] Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt.

• #72 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #73 Ebstein’s Anomaly: Symptoms and Treatment | Doctor

  https://patient.info/doctor/ebsteins-anomaly-pro

  In patients over 50 years of age with Ebstein’s anomaly, surgery is associated with good long-term survival and improved functional status, although long-term survival might be improved by performing surgery earlier. […] Tricuspid valve repair is preferred over valve replacement. Bioprosthetic valves are preferred over mechanical prosthetic valves. […] Both the atrialised portion of the right ventricle and the very dilated, thin-walled right atrium can be resected. […] Palliative procedures are usually reserved for severely ill infants with an otherwise poor prognosis. These include: creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through an aorto-pulmonary shunt. […] Heart transplantation is appropriate in selected patients.

• #74 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. […] Heart transplantation should be considered in patients with associated left ventricular dysfunction. […] Surgical options include a biventricular repair pathway with a valvuloplasty. […] Anatomic pulmonary atresia is not a contraindication to a biventricular approach as an RV-PA conduit or a transannular patch with a monocusp can be used to establish continuity depending upon how satisfactory the valvuloplasty is. […] Some neonates who are hemodynamically unstable with some degree of end-organ damage yet have a favorable morphology for a biventricular repair and thus are unlikely to tolerate a prolonged procedure with a valvuloplasty or concerns exist for a possibility of a tenuous repair given the friability of neonatal valvular tissues can be channeled down the single ventricle pathway initially with a modified Starnes procedure.

• #75 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

• #76 EBSTEIN ANOMALY: A COMPLEX HEART DEFECT | Mya Care

  https://myacare.com/blog/ebstein-anomaly-a-complex-heart-defect

  Catheter ablation is a minimally invasive surgical method that uses a catheter to target and destroy abnormal heart tissue responsible for causing abnormal heart rhythms. […] Transcatheter procedures are minimally invasive techniques involving using a catheter to treat and repair heart valve abnormalities without needing open-heart surgery. […] Heart transplantation can treat severe cases where repair is not possible. […] The prognosis for most cases of surgery is generally positive. […] Open heart surgery is preferable in complicated situations where heart surgeons require a better view of the heart tissue. […] Individuals with Ebstein anomaly need to work closely with their doctors to monitor their heart health and manage any symptoms that may arise, even after surgery.

• #77 Ebstein anomaly – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132

  If the valve can’t be repaired, surgery to replace the valve may be needed. […] This surgery is done to fix a hole between the upper chambers of the heart. […] If Ebstein anomaly causes irregular heartbeats, this treatment may be done during valve repair or replacement surgery. […] This treatment is done to correct fast or irregular heartbeats. […] If severe Ebstein anomaly is causing the heart to fail, a heart transplant might be needed.

• #78 Ebstein Anomaly treatment by the Irish Congenital Heart Centre

  https://congenitalheartcentre.ie/congenital-heart-conditions/ebstein-anomaly/

  Tricuspid valve repair: In this procedure, surgeons reduce the size of the valve opening and allow the existing valve leaflets to come together to work properly. […] Tricuspid valve replacement: If the existing valve can’t be repaired, your surgeon may replace the valve by removing the deformed valve and inserting either a biological tissue (bioprosthetic) or mechanical valve. […] Closure of the atrial septal defect: If a hole is present between the two upper chambers of the heart (atrial septal defect), your surgeon can close the hole during surgery to repair or replace the defective valve. […] Maze procedure: If you have fast heart rhythms, your surgeon may perform the maze procedure to correct the fast heart rhythms during surgery to repair or replace the tricuspid valve. […] Radiofrequency catheter ablation: If you have fast or abnormal heart rhythms, your doctor may perform radiofrequency catheter ablation. […] Heart transplantation: If you have severe Ebstein anomaly and poor heart function, a heart transplant may be necessary.

• #79 Ebstein’s Anomaly Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/ebsteins-anomaly

  Starnes procedure: This surgery is typically performed in the first week of life. The surgeon sews over the leaking tricuspid valve (the valve between the right atrium and right ventricle), and then creates an extra connection (called a Blalock-Taussig shunt) to provide blood flow to the lungs. […] Glenn procedure: This procedure typically occurs between four and six months of age. This surgery creates a direct connection between the pulmonary artery and the superior vena cava (vessel returning oxygen-poor blood from the upper part of the body to the heart). This reduces the work the right ventricle has to do by allowing blood returning from the upper body to flow directly to the lungs. […] Fontan procedure: This procedure typically occurs between two and four years of age. This surgery connects the pulmonary artery and the inferior vena cava (vessel returning oxygen-poor blood from the lower part of the body to the heart), allowing the blood coming back from the lower body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart. The surgeon may leave a small connection between the oxygen rich and oxygen poor chambers (a fenestration).

• #80 Ebstein anomaly – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132

  If the valve can’t be repaired, surgery to replace the valve may be needed. […] This surgery is done to fix a hole between the upper chambers of the heart. […] If Ebstein anomaly causes irregular heartbeats, this treatment may be done during valve repair or replacement surgery. […] This treatment is done to correct fast or irregular heartbeats. […] If severe Ebstein anomaly is causing the heart to fail, a heart transplant might be needed.

• #81 Ebstein Anomaly treatment by the Irish Congenital Heart Centre

  https://congenitalheartcentre.ie/congenital-heart-conditions/ebstein-anomaly/

  Tricuspid valve repair: In this procedure, surgeons reduce the size of the valve opening and allow the existing valve leaflets to come together to work properly. […] Tricuspid valve replacement: If the existing valve can’t be repaired, your surgeon may replace the valve by removing the deformed valve and inserting either a biological tissue (bioprosthetic) or mechanical valve. […] Closure of the atrial septal defect: If a hole is present between the two upper chambers of the heart (atrial septal defect), your surgeon can close the hole during surgery to repair or replace the defective valve. […] Maze procedure: If you have fast heart rhythms, your surgeon may perform the maze procedure to correct the fast heart rhythms during surgery to repair or replace the tricuspid valve. […] Radiofrequency catheter ablation: If you have fast or abnormal heart rhythms, your doctor may perform radiofrequency catheter ablation. […] Heart transplantation: If you have severe Ebstein anomaly and poor heart function, a heart transplant may be necessary.

• #82 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Left ventricular dysfunction should not be considered a contraindication to tricuspid valve surgery. […] Functional status improves after surgery. […] Surgical treatments of arrhythmias include the following: ablation of the accessory pathways, modified right atrial maze procedure for atrial arrhythmias, in the presence of atrial fibrillation, the addition of left atrial Cox Maze III procedure can reduce the risk of recurrent atrial fibrillation. […] Cardiac transplantation is appropriate in selected patients.

• #83 Ebstein’s Anomaly: A Rare Disease Treated with World-class Care Here at Home | Newsroom

  https://news.unchealthcare.org/2021/10/ebsteins-anomaly-a-rare-disease-treated-with-world-class-care-here-at-home/

  In June 2018, Mahesh Sharma, MD, was recruited from the Childrens Hospital of Pittsburgh to lead the surgical program at UNC Childrens Heart Center. […] In August 2020, Drs. Sharma and da Silva performed open-heart surgery on Jahliah. […] Its our feeling that the Cone Procedure offers a good durable repair, explains Dr. Sharma. […] Along with the Cone procedure, Dr. Sharma and his team performed arrhythmia surgery, also referred to as the Maze procedure. […] The hope for Jahliah is that she is not going to require lifelong medication for the heart rhythm problem because doctors solved that issue with the Maze procedure. […] Since her surgery in August, Jahliah celebrated her 10th birthday. […] All of Jahliahs doctors stay in contact to discuss her case and make sure she progresses well in her recovery.

• #84 Ebstein’s Anomaly: Symptoms and Treatment | Doctor

  https://patient.info/doctor/ebsteins-anomaly-pro

  In patients over 50 years of age with Ebstein’s anomaly, surgery is associated with good long-term survival and improved functional status, although long-term survival might be improved by performing surgery earlier. […] Tricuspid valve repair is preferred over valve replacement. Bioprosthetic valves are preferred over mechanical prosthetic valves. […] Both the atrialised portion of the right ventricle and the very dilated, thin-walled right atrium can be resected. […] Palliative procedures are usually reserved for severely ill infants with an otherwise poor prognosis. These include: creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through an aorto-pulmonary shunt. […] Heart transplantation is appropriate in selected patients.

• #85 Ebstein’s Anomaly Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/ebsteins-anomaly

  Starnes procedure: This surgery is typically performed in the first week of life. The surgeon sews over the leaking tricuspid valve (the valve between the right atrium and right ventricle), and then creates an extra connection (called a Blalock-Taussig shunt) to provide blood flow to the lungs. […] Glenn procedure: This procedure typically occurs between four and six months of age. This surgery creates a direct connection between the pulmonary artery and the superior vena cava (vessel returning oxygen-poor blood from the upper part of the body to the heart). This reduces the work the right ventricle has to do by allowing blood returning from the upper body to flow directly to the lungs. […] Fontan procedure: This procedure typically occurs between two and four years of age. This surgery connects the pulmonary artery and the inferior vena cava (vessel returning oxygen-poor blood from the lower part of the body to the heart), allowing the blood coming back from the lower body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart. The surgeon may leave a small connection between the oxygen rich and oxygen poor chambers (a fenestration).

• #86 Ebstein’s Anomaly Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/ebsteins-anomaly

  Starnes procedure: This surgery is typically performed in the first week of life. The surgeon sews over the leaking tricuspid valve (the valve between the right atrium and right ventricle), and then creates an extra connection (called a Blalock-Taussig shunt) to provide blood flow to the lungs. […] Glenn procedure: This procedure typically occurs between four and six months of age. This surgery creates a direct connection between the pulmonary artery and the superior vena cava (vessel returning oxygen-poor blood from the upper part of the body to the heart). This reduces the work the right ventricle has to do by allowing blood returning from the upper body to flow directly to the lungs. […] Fontan procedure: This procedure typically occurs between two and four years of age. This surgery connects the pulmonary artery and the inferior vena cava (vessel returning oxygen-poor blood from the lower part of the body to the heart), allowing the blood coming back from the lower body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart. The surgeon may leave a small connection between the oxygen rich and oxygen poor chambers (a fenestration).

• #87 Partners in Care | Ebstein’s Anomaly treatment at the Texas Center…

  https://partnersincare.health/conditions/ebsteins-anomaly

  Blalock-Thomas-Taussig Shunt: if there is not enough blood moving through the heart and out to the lungs, a Blalock-Taussig shunt can be used to create a pathway for blood to reach the lungs. […] Glenn Procedure: To unload the volume of blood going through the right side of the heart, a bidirectional Glenn procedure can be performed. […] Cone Procedure: The Cone technique can be used to improve the valve function. […] Starnes Procedure: The Starnes procedure closes off the ill-functioning right ventricle, creating a single ventricle circulation. […] Valve Replacement: The valve can be replaced with an artificial valve.

• #88 Ebstein’s Anomaly – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/ebsteins-anomaly/

  In the most severe cases, Ebsteins anomaly affects the right ventricle so seriously that it will never be able to pump blood to the lungs. These children need 2 or 3 surgeries to make a path for blue (oxygen-poor) blood to flow into their lungs without passing through their right ventricle at all. These children receive complete care through our Single Ventricle Program. Those who have a Fontan procedure get coordinated, ongoing, team-based care through our Fontan Clinic.

• #89 Ebstein’s Anomaly Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/ebsteins-anomaly

  Starnes procedure: This surgery is typically performed in the first week of life. The surgeon sews over the leaking tricuspid valve (the valve between the right atrium and right ventricle), and then creates an extra connection (called a Blalock-Taussig shunt) to provide blood flow to the lungs. […] Glenn procedure: This procedure typically occurs between four and six months of age. This surgery creates a direct connection between the pulmonary artery and the superior vena cava (vessel returning oxygen-poor blood from the upper part of the body to the heart). This reduces the work the right ventricle has to do by allowing blood returning from the upper body to flow directly to the lungs. […] Fontan procedure: This procedure typically occurs between two and four years of age. This surgery connects the pulmonary artery and the inferior vena cava (vessel returning oxygen-poor blood from the lower part of the body to the heart), allowing the blood coming back from the lower body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart. The surgeon may leave a small connection between the oxygen rich and oxygen poor chambers (a fenestration).

• #90 Partners in Care | Ebstein’s Anomaly treatment at the Texas Center…

  https://partnersincare.health/conditions/ebsteins-anomaly

  Blalock-Thomas-Taussig Shunt: if there is not enough blood moving through the heart and out to the lungs, a Blalock-Taussig shunt can be used to create a pathway for blood to reach the lungs. […] Glenn Procedure: To unload the volume of blood going through the right side of the heart, a bidirectional Glenn procedure can be performed. […] Cone Procedure: The Cone technique can be used to improve the valve function. […] Starnes Procedure: The Starnes procedure closes off the ill-functioning right ventricle, creating a single ventricle circulation. […] Valve Replacement: The valve can be replaced with an artificial valve.

• #91 Ebstein Anomaly: Diagnosis & Treatment | NewYork-Presbyterian

  https://www.nyp.org/pediatrics/heart/ebstein-anomaly/treatment

  The Cone procedure is the most advanced form of tricuspid valve repair for Ebstein Anomaly. […] Bidirectional Glenn procedure. Sometimes when the right ventricle is not functioning, a procedure to direct the blood flow from the upper body veins to the lungs is performed. It replaces the normal way in which the heart pumps the blood to the lungs. […] Heart transplantation. In severe Ebstein anomaly cases with poor heart function, a heart transplant might be necessary. […] At NewYork-Presbyterians Congenital Heart Center, we are dedicated to helping children with congenital heart conditions. We collaborate with experts in other teams at NewYork-Presbyterian to provide treatment for children with complex heart conditions.

• #92 Ebstein’s Anomaly Diagnosis & Treatment | Cardinal Glennon

  https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/fetal-heart-program/heart-conditions/ebsteins-anomaly

  Starnes procedure: This surgery is typically performed in the first week of life. The surgeon sews over the leaking tricuspid valve (the valve between the right atrium and right ventricle), and then creates an extra connection (called a Blalock-Taussig shunt) to provide blood flow to the lungs. […] Glenn procedure: This procedure typically occurs between four and six months of age. This surgery creates a direct connection between the pulmonary artery and the superior vena cava (vessel returning oxygen-poor blood from the upper part of the body to the heart). This reduces the work the right ventricle has to do by allowing blood returning from the upper body to flow directly to the lungs. […] Fontan procedure: This procedure typically occurs between two and four years of age. This surgery connects the pulmonary artery and the inferior vena cava (vessel returning oxygen-poor blood from the lower part of the body to the heart), allowing the blood coming back from the lower body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart. The surgeon may leave a small connection between the oxygen rich and oxygen poor chambers (a fenestration).

• #93 Ebstein’s Anomaly – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/ebsteins-anomaly/

  In the most severe cases, Ebsteins anomaly affects the right ventricle so seriously that it will never be able to pump blood to the lungs. These children need 2 or 3 surgeries to make a path for blue (oxygen-poor) blood to flow into their lungs without passing through their right ventricle at all. These children receive complete care through our Single Ventricle Program. Those who have a Fontan procedure get coordinated, ongoing, team-based care through our Fontan Clinic.

• #94 Ebstein’s Anomaly – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/ebsteins-anomaly/

  In the most severe cases, Ebsteins anomaly affects the right ventricle so seriously that it will never be able to pump blood to the lungs. These children need 2 or 3 surgeries to make a path for blue (oxygen-poor) blood to flow into their lungs without passing through their right ventricle at all. These children receive complete care through our Single Ventricle Program. Those who have a Fontan procedure get coordinated, ongoing, team-based care through our Fontan Clinic.

• #95 Ebstein anomaly – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/drc-20352132

  If the valve can’t be repaired, surgery to replace the valve may be needed. […] This surgery is done to fix a hole between the upper chambers of the heart. […] If Ebstein anomaly causes irregular heartbeats, this treatment may be done during valve repair or replacement surgery. […] This treatment is done to correct fast or irregular heartbeats. […] If severe Ebstein anomaly is causing the heart to fail, a heart transplant might be needed.

• #96 Ebstein Anomaly treatment by the Irish Congenital Heart Centre

  https://congenitalheartcentre.ie/congenital-heart-conditions/ebstein-anomaly/

  Tricuspid valve repair: In this procedure, surgeons reduce the size of the valve opening and allow the existing valve leaflets to come together to work properly. […] Tricuspid valve replacement: If the existing valve can’t be repaired, your surgeon may replace the valve by removing the deformed valve and inserting either a biological tissue (bioprosthetic) or mechanical valve. […] Closure of the atrial septal defect: If a hole is present between the two upper chambers of the heart (atrial septal defect), your surgeon can close the hole during surgery to repair or replace the defective valve. […] Maze procedure: If you have fast heart rhythms, your surgeon may perform the maze procedure to correct the fast heart rhythms during surgery to repair or replace the tricuspid valve. […] Radiofrequency catheter ablation: If you have fast or abnormal heart rhythms, your doctor may perform radiofrequency catheter ablation. […] Heart transplantation: If you have severe Ebstein anomaly and poor heart function, a heart transplant may be necessary.

• #97 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #98 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. […] Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. […] Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves.

• #99 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #100 Ebstein’s Anomaly

  https://www.healio.com/cardiology/learn-the-heart/cardiology-review/topic-reviews/ebsteins-anomaly

  Ebstein’s anomaly is frequently associated with Wolff-Parkinson-White syndrome which can manifest as atrioventricular reentrant tachycardia (AVRT) or atrial fibrillation with pre-excitation. Treatment is emergent cardioversion, procainamide and eventually ablation. […] Tricuspid valve regurgitation can ensue and frequently requires surgical repair. Indications for repair of the Ebsteins anomaly and associated atrial septal defect (if present) include New York Heart Association Functional Class III or IV symptoms, marked right heart enlargement, cyanosis, and transient ischemic attack (TIA) or stroke.

• #101 Ebsteins anomaly of the tricuspid valve medical therapy – wikidoc

  https://www.wikidoc.org/index.php/Ebsteins_anomaly_of_the_tricuspid_valve_medical_therapy

  Medical management of patients with Ebsteins anomaly consists of supportive care such as control of the heart rhythm with antiarrhythmic drugs, inotropic agents and diuretics for heart failure, anticoagulation in patients with atrial fibrillation and paradoxical embolization. […] Some Ebstein’s anomaly patients present with an (antidromic) AV nodal reentrant tachycardia with associated pre-excitation. Among these patients, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers and digoxin are contraindicated. If there is atrial fibrillation with pre-excitation, treatment options include procainamide, flecainide, propafenone, dofetilide and ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response.

• #102 Ebstein’s anomaly – Wikipedia

  https://en.wikipedia.org/wiki/Ebstein%27s_anomaly

  Ebstein’s cardio physiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred medication treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated. […] If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response. […] The Canadian Cardiovascular Society (CCS) recommends surgical intervention for these indications: Limited exercise capacity (NYHA III-IV), Increasing heart size (cardiothoracic ratio greater than 65%), Important cyanosis (resting oxygen saturation less than 90% – level B), Severe tricuspid regurgitation with symptoms, Transient ischemic attack or stroke. […] The CCS further recommends patients who require operation for Ebstein’s anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.

• #103 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Class IC and III anti-arrhythmic drugs may help to control the arrhythmic burden, however, there is general agreement to avoid sotalol and amiodarone as long-term therapy due to their known proarrhythmic risk and side effects. […] Cardiac resynchronisation therapy can be applied in case of bundle branch block and either LV or RV dysfunction. […] Monocusp reconstruction for surgical treatment of EA had been used for many years. […] Nonetheless, there is nowadays a general consensus on adopting a more anatomical form of repair, described by da Silva et al in 2007. […] The concept of da Silvas cone repair (CR) is to perform a surgical delamination of all the rescuable leaflets in order to obtain a 360 TV annulus in anatomical position. […] Current indication to surgical intervention is severe TR with symptoms or, in the absence of symptoms, with progression in either right-sided heart dilation or fRV systolic dysfunction. […] Nonetheless, the surgical mortality risk for younger patients is not negligible even in experienced centres and, in the absence of randomized control studies, whether an early operation may effectively prevent complications of EA natural history is still debated.

• #104 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary. […] Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin. […] Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease.

• #105 Ebstein Anomaly (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/ebstein-anomaly.html

  Doctors may recommend an electrophysiology study (EP study) when there are serious abnormal heart rhythms. During this study, ablation (freezing or burning) can get rid of electrical pathway fibers that make the heart beat too fast. […] Babies with Ebstein anomaly need care from a pediatric cardiologist (a doctor who diagnoses and treats children’s heart problems) because the heart’s pumping ability may fall short as the child grows. The doctor will recommend treatment that is tailored to a child’s needs. […] Many children whose Ebstein anomaly was corrected by surgery can be as active as other kids.

• #106 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #107 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #108 Ebstein’s Anomaly: A Rare Disease Treated with World-class Care Here at Home | Newsroom

  https://news.unchealthcare.org/2021/10/ebsteins-anomaly-a-rare-disease-treated-with-world-class-care-here-at-home/

  In June 2018, Mahesh Sharma, MD, was recruited from the Childrens Hospital of Pittsburgh to lead the surgical program at UNC Childrens Heart Center. […] In August 2020, Drs. Sharma and da Silva performed open-heart surgery on Jahliah. […] Its our feeling that the Cone Procedure offers a good durable repair, explains Dr. Sharma. […] Along with the Cone procedure, Dr. Sharma and his team performed arrhythmia surgery, also referred to as the Maze procedure. […] The hope for Jahliah is that she is not going to require lifelong medication for the heart rhythm problem because doctors solved that issue with the Maze procedure. […] Since her surgery in August, Jahliah celebrated her 10th birthday. […] All of Jahliahs doctors stay in contact to discuss her case and make sure she progresses well in her recovery.

• #109 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Left ventricular dysfunction should not be considered a contraindication to tricuspid valve surgery. […] Functional status improves after surgery. […] Surgical treatments of arrhythmias include the following: ablation of the accessory pathways, modified right atrial maze procedure for atrial arrhythmias, in the presence of atrial fibrillation, the addition of left atrial Cox Maze III procedure can reduce the risk of recurrent atrial fibrillation. […] Cardiac transplantation is appropriate in selected patients.

• #110 Ebstein’s anomaly – congenital heart disease – BHF

  https://www.bhf.org.uk/informationsupport/conditions/ebsteins-anomaly

  The treatment for Ebsteins anomaly depends on: […] If you need treatment, you’ll usually have surgery to repair or replace your tricuspid valve. This could include: […] Some people with Ebsteins anomaly will also have abnormal heart rhythms (arrhythmias). Your doctor may recommend you have treatment at the same time, like a pacemaker or an implantable cardioverter defibrillator (ICD). […] If you cannot have surgery, or choose not to, your doctor may suggest medicine to help your heart work better and improve symptoms. You’ll also have regular appointments to check how your heart is working. […] Your doctor can give you more information on living with Ebsteins anomaly and how to manage your condition.

• #111 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Class IC and III anti-arrhythmic drugs may help to control the arrhythmic burden, however, there is general agreement to avoid sotalol and amiodarone as long-term therapy due to their known proarrhythmic risk and side effects. […] Cardiac resynchronisation therapy can be applied in case of bundle branch block and either LV or RV dysfunction. […] Monocusp reconstruction for surgical treatment of EA had been used for many years. […] Nonetheless, there is nowadays a general consensus on adopting a more anatomical form of repair, described by da Silva et al in 2007. […] The concept of da Silvas cone repair (CR) is to perform a surgical delamination of all the rescuable leaflets in order to obtain a 360 TV annulus in anatomical position. […] Current indication to surgical intervention is severe TR with symptoms or, in the absence of symptoms, with progression in either right-sided heart dilation or fRV systolic dysfunction. […] Nonetheless, the surgical mortality risk for younger patients is not negligible even in experienced centres and, in the absence of randomized control studies, whether an early operation may effectively prevent complications of EA natural history is still debated.

• #112 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Class IC and III anti-arrhythmic drugs may help to control the arrhythmic burden, however, there is general agreement to avoid sotalol and amiodarone as long-term therapy due to their known proarrhythmic risk and side effects. […] Cardiac resynchronisation therapy can be applied in case of bundle branch block and either LV or RV dysfunction. […] Monocusp reconstruction for surgical treatment of EA had been used for many years. […] Nonetheless, there is nowadays a general consensus on adopting a more anatomical form of repair, described by da Silva et al in 2007. […] The concept of da Silvas cone repair (CR) is to perform a surgical delamination of all the rescuable leaflets in order to obtain a 360 TV annulus in anatomical position. […] Current indication to surgical intervention is severe TR with symptoms or, in the absence of symptoms, with progression in either right-sided heart dilation or fRV systolic dysfunction. […] Nonetheless, the surgical mortality risk for younger patients is not negligible even in experienced centres and, in the absence of randomized control studies, whether an early operation may effectively prevent complications of EA natural history is still debated.

• #113 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Medical Management In infants with Ebstein anomaly, the mainstay of treatment is supportive to reduce pulmonary vascular resistance and hypoxemia. In symptomatic infants with either heart failure or cyanosis, inhaled nitric oxide can reduce pulmonary vascular resistance. In cyanotic infants, prostaglandin E1 (PGE1) infusion can maintain ductus arteriosus patency and lower pulmonary vascular resistance by increasing pulmonary vasodilation. Newborns with heart failure and cardiogenic shock may require inotropic therapy; milrinone is the drug of choice since it contributes to pulmonary vasodilation. Symptoms of heart failure are treated with loop diuretics and guideline-directed medical therapy. Patients with supraventricular tachyarrhythmias can receive rate-control medications such as beta blockers or calcium channel blockers; if ineffective, the class I antiarrhythmic procainamide or class III antiarrhythmic amiodarone or sotalol can be used to treat paroxysmal atrial fibrillation. Patients with intractable arrhythmias frequently require catheter ablation.

• #114 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #115 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Medical Management In infants with Ebstein anomaly, the mainstay of treatment is supportive to reduce pulmonary vascular resistance and hypoxemia. In symptomatic infants with either heart failure or cyanosis, inhaled nitric oxide can reduce pulmonary vascular resistance. In cyanotic infants, prostaglandin E1 (PGE1) infusion can maintain ductus arteriosus patency and lower pulmonary vascular resistance by increasing pulmonary vasodilation. Newborns with heart failure and cardiogenic shock may require inotropic therapy; milrinone is the drug of choice since it contributes to pulmonary vasodilation. Symptoms of heart failure are treated with loop diuretics and guideline-directed medical therapy. Patients with supraventricular tachyarrhythmias can receive rate-control medications such as beta blockers or calcium channel blockers; if ineffective, the class I antiarrhythmic procainamide or class III antiarrhythmic amiodarone or sotalol can be used to treat paroxysmal atrial fibrillation. Patients with intractable arrhythmias frequently require catheter ablation.

• #116 Ebstein Anomaly – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/ebstein-anomaly

  Treatment is with medications and surgery, and sometimes with interventional catheterization. […] Medications play a role in managing neonates who are severely cyanotic and have inadequate pulmonary blood flow. Maintaining ductal patency with prostaglandins allows time for the pulmonary vascular resistance to drop. The decrease in pulmonary artery pressure allows the compromised right ventricle to provide adequate pulmonary blood flow. Some neonates with very severe Ebstein anomaly and functional compromise of the right ventricle require more prolonged palliation with a Blalock-Thomas-Taussig (BTT) shunt or other complex surgical interventions. […] After the first several months of age, surgical repair of the tricuspid valve should be considered when any symptoms are present or there is evidence of progressive tricuspid valve dysfunction or enlargement of the right heart chambers. Surgical treatment of this anomaly has evolved over the years with developments such as the cone procedure. This approach to repair of the tricuspid valve involves delamination and mobilization of the tricuspid valve leaflets, plicating the atrialized portion of the right ventricle and the annulus and reattaching newly created „leaflet cone” to the true tricuspid annulus. These advances have greatly enhanced the surgeon’s ability to achieve an excellent functional repair of even the most deformed tricuspid valves. Any atrial defect is closed at the same time. […] Interventional catheterization techniques may be used to close an atrial communication when cyanosis is present, but the tricuspid valve dysfunction is only modest.

• #117 Ebstein Anomaly – Children’s Health Issues – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/ebstein-anomaly

  Treatment is with medication and surgery. […] Medication is needed when newborns have severe cyanosis. Medications called prostaglandins may be given to help keep the ductus arteriosus open to allow the right ventricle to maintain blood flow until surgery can be done. […] If the abnormal tricuspid valve causes severe symptoms or enlargement of the heart, the valve is surgically repaired.

• #118 Ebstein Anomaly – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/ebstein-anomaly

  Treatment is with medications and surgery, and sometimes with interventional catheterization. […] Medications play a role in managing neonates who are severely cyanotic and have inadequate pulmonary blood flow. Maintaining ductal patency with prostaglandins allows time for the pulmonary vascular resistance to drop. The decrease in pulmonary artery pressure allows the compromised right ventricle to provide adequate pulmonary blood flow. Some neonates with very severe Ebstein anomaly and functional compromise of the right ventricle require more prolonged palliation with a Blalock-Thomas-Taussig (BTT) shunt or other complex surgical interventions. […] After the first several months of age, surgical repair of the tricuspid valve should be considered when any symptoms are present or there is evidence of progressive tricuspid valve dysfunction or enlargement of the right heart chambers. Surgical treatment of this anomaly has evolved over the years with developments such as the cone procedure. This approach to repair of the tricuspid valve involves delamination and mobilization of the tricuspid valve leaflets, plicating the atrialized portion of the right ventricle and the annulus and reattaching newly created „leaflet cone” to the true tricuspid annulus. These advances have greatly enhanced the surgeon’s ability to achieve an excellent functional repair of even the most deformed tricuspid valves. Any atrial defect is closed at the same time. […] Interventional catheterization techniques may be used to close an atrial communication when cyanosis is present, but the tricuspid valve dysfunction is only modest.

• #119 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Surgical Management Newborns with Ebstein anomaly are initially observed as the pulmonary vascular resistance decreases. If surgery is required, a systemic-to-pulmonary artery shunt is typically employed to relieve cyanosis and secure adequate pulmonary and systemic circulations. Due to high periprocedural mortality, more definitive surgery is usually delayed unless the neonate meets specific indications. Data show that delaying surgery until heart failure or RV systolic dysfunction occurs is associated with worse outcomes. This is thought to be because Ebstein anomaly is a myopathic disorder in addition to abnormal valves, and the myopathic RV may be unable to tolerate increased volumes. Indications for surgery in neonates with Ebstein anomaly include: Right heart failure due to severe tricuspid regurgitation, A cardiothoracic ratio 80%, Severe cyanosis with dependency on PGE1, Mechanical ventilation dependency. In children and adults with Ebstein anomaly, indications for surgery include: Heart failure symptoms (New York Heart Association functional class III or IV), Progressive exercise intolerance, Evidence of RV dysfunction or progressive RV dilatation, Evidence of paradoxical emboli, Arrhythmias refractory to medical and catheter ablation therapies, Cyanosis with oxygen saturations 90%, Severe tricuspid regurgitation, Significant concomitant lesions such as pulmonic stenosis or atrial or ventricular septal defects, Progressive cardiomegaly, Cardiothoracic ratio 65%, Reduced left ventricular function. Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. For atrial arrhythmias in these patients, catheter ablation carries risks of paradoxical emboli, given the high incidence of interatrial shunts. A surgical biatrial Maze procedure during valvular repair is recommended. Multiple surgical approaches have been described for the treatment of Ebstein anomaly. Cone reconstruction is the currently preferred surgical approach in young children and adults. This approach involves mobilizing the anterior and posterior leaflets from their anomalous attachments, rotating the detached edges of these leaflets clockwise, and suturing them to the septal edge of the anterior leaflet at the level of the tricuspid valve annulus. The Danielson repair is the plication of the atrialized portion of the RV to narrow the size of the tricuspid valve and create a monoleaflet tricuspid valve that is competent. The Carpentier monocusp repair plicates the atrialized portion of the RV, implants a ring within the tricuspid annulus to provide support, mobilizes the anterior tricuspid valve leaflet, and attaches it to the annulus anteriorly. When the native tricuspid valve cannot be repaired, especially in older patients, it can be replaced with a bioprosthetic or mechanical valve. A bioprosthetic valve might require temporary anticoagulation; a mechanical valve will require lifelong anticoagulation. Mechanical valves are avoided when RV dysfunction is severe to mitigate disc motion abnormalities and the increased risk of valve thrombosis.

• #120 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Surgical options include a biventricular repair pathway with a valvuloplasty. […] Patients with EA with anatomic pulmonary atresia, a circular shunt, dependency on prostaglandins or mechanical ventilation, worsening cyanosis or heart failure with inability to tolerate feeds due to mesenteric congestion, and generally severe forms such as Carpentier type C/D or GOSE score 3 will require a surgical intervention during the neonatal period. […] The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, reduction right atrioplasty, fenestrated or partial closure of the atrial septum, establishment of RV-PA continuity in case of pulmonary valve atresia, and closure of the VSD if present. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #121 Ebstein’s Anomaly | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/ebsteins-anomaly

  Treatment for Ebsteins anomaly depends on how badly different parts of the heart are affected. Sometimes, if the problems arent too bad, a baby may not need surgery and can be watched closely for the first few years of their life. In some cases the tricuspid valve and right ventricle are not strong enough after birth, and surgery is needed sooner in the first week or two of life. In severe cases, multiple surgeries are needed. […] All babies with Ebsteins anomaly need extra monitoring after birth to make sure their heart is working normally and oxygen levels are stable. These may be able to be more space out as the child gets older. […] When your baby gets to the CTICU at Nationwide Childrens Hospital, they will have many tests. These tests include an echocardiogram and an EKG. These tests will help the team make a plan for your baby at the Heart Center Case Management Conference. This plan includes choices about surgeries, procedures, and timing.

• #122 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Surgical options include a biventricular repair pathway with a valvuloplasty. […] Patients with EA with anatomic pulmonary atresia, a circular shunt, dependency on prostaglandins or mechanical ventilation, worsening cyanosis or heart failure with inability to tolerate feeds due to mesenteric congestion, and generally severe forms such as Carpentier type C/D or GOSE score 3 will require a surgical intervention during the neonatal period. […] The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, reduction right atrioplasty, fenestrated or partial closure of the atrial septum, establishment of RV-PA continuity in case of pulmonary valve atresia, and closure of the VSD if present. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #123 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. […] Heart transplantation should be considered in patients with associated left ventricular dysfunction. […] Surgical options include a biventricular repair pathway with a valvuloplasty. […] Anatomic pulmonary atresia is not a contraindication to a biventricular approach as an RV-PA conduit or a transannular patch with a monocusp can be used to establish continuity depending upon how satisfactory the valvuloplasty is. […] Some neonates who are hemodynamically unstable with some degree of end-organ damage yet have a favorable morphology for a biventricular repair and thus are unlikely to tolerate a prolonged procedure with a valvuloplasty or concerns exist for a possibility of a tenuous repair given the friability of neonatal valvular tissues can be channeled down the single ventricle pathway initially with a modified Starnes procedure.

• #124 Ebstein’s Anomaly | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/ebsteins-anomaly/

  In the beginning, medications may be given to try to reduce the pressure in the right ventricle so that less leakiness occurs. […] Sometimes, prostaglandin needs to be given to keep the ductus arteriosus (PDA) open until the pressure in the right ventricle gets lower. […] Once the pressure in the lungs is lower, the right ventricle may be able to pump more blood to the lungs than leaks back across the tricuspid valve and the baby can survive. If this is not possible, heart surgery may be needed. […] The options for a newborn infant are few. One is to try to repair the valve. Traditionally, this has not been very successful. Another option is to sew the valve shut so it doesnt leak, and to perform a series of operations leading to a univentricular repair or Fontan operation. Sometimes there is a combination of approaches used. Another option is heart transplantation.

• #125 Ebstein Anomaly in Infants | Children’s Hospital Colorado

  https://www.childrenscolorado.org/conditions-and-advice/conditions-and-symptoms/conditions/ebstein-anomaly/

  Ebstein anomaly treatment depends on how your child’s tricuspid valve is working and your child’s heart rhythm. In all cases, we suggest long-term follow-up with our Heart Institute. In mild cases, your child usually won’t need treatment. In moderate cases, we typically use medications to treat rhythm problems and help the tricuspid valve work correctly. […] In severe cases, if medication doesn’t help and fixing the tricuspid valve is not possible (which is more common for newborns), we offer a procedure called the Starnes procedure. In the Starnes procedure, our surgeons create a different path for blood to go to the lungs so the blood flow avoids the tricuspid valve and right ventricle. We only use this in more severe cases and your child’s heart care team would explain this procedure and long-term effects in detail.

• #126 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Surgical options include a biventricular repair pathway with a valvuloplasty. […] Patients with EA with anatomic pulmonary atresia, a circular shunt, dependency on prostaglandins or mechanical ventilation, worsening cyanosis or heart failure with inability to tolerate feeds due to mesenteric congestion, and generally severe forms such as Carpentier type C/D or GOSE score 3 will require a surgical intervention during the neonatal period. […] The key steps in a biventricular repair are mobilization of the TV leaflets, plication and obliteration of the ARV, reduction of the true annulus, reduction right atrioplasty, fenestrated or partial closure of the atrial septum, establishment of RV-PA continuity in case of pulmonary valve atresia, and closure of the VSD if present. […] The two commonly described neonatal Tricuspid valvuloplasty techniques are the Knott-Craig monocusp technique and the da Silva Cone repair. […] Tricuspid valve replacement is necessary when a repair is not feasible or has failed. […] Heart transplantation should be given consideration when the LV function is severely depressed.

• #127 Ebstein’s Anomaly | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/ebsteins-anomaly/

  In the beginning, medications may be given to try to reduce the pressure in the right ventricle so that less leakiness occurs. […] Sometimes, prostaglandin needs to be given to keep the ductus arteriosus (PDA) open until the pressure in the right ventricle gets lower. […] Once the pressure in the lungs is lower, the right ventricle may be able to pump more blood to the lungs than leaks back across the tricuspid valve and the baby can survive. If this is not possible, heart surgery may be needed. […] The options for a newborn infant are few. One is to try to repair the valve. Traditionally, this has not been very successful. Another option is to sew the valve shut so it doesnt leak, and to perform a series of operations leading to a univentricular repair or Fontan operation. Sometimes there is a combination of approaches used. Another option is heart transplantation.

• #128 Ebstein anomaly: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/ebstein-anomaly-management-and-prognosis

  Ebstein anomaly: Management and prognosis […] The approach to the management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including the presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention. […] Indications for intervention or surgical repair of children and adults with Ebstein anomaly include worsening heart failure (HF), progressive cyanosis or uncontrolled arrhythmias. Surgical repair may also be considered if there is decline in functional capacity with evidence of right ventricular (RV) dysfunction and/or hypoxia. Occasionally, asymptomatic adult patients with severe tricuspid valve regurgitation and repairable tricuspid valves are considered for early repair to prevent progressive right heart enlargement.

• #129 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Although survival has significantly improved in the last four decades, the diagnosis of Ebsteins anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. […] A wait-and-see approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebsteins anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome. […] Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention. […] The effect of medical therapy on patients with EA has not yet been solidly investigated.

• #130

  https://link.springer.com/article/10.1007/s11936-016-0478-2

  Ebsteins anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. […] Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. […] Pre-pregnancy evaluation should be considered in all Ebsteins anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. […] Longitudinal clinical follow-up in all Ebsteins anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. […] Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery.

• #131 Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy | Heart

  https://heart.bmj.com/content/110/4/235

  Although survival has significantly improved in the last four decades, the diagnosis of Ebsteins anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. […] A wait-and-see approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebsteins anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome. […] Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention. […] The effect of medical therapy on patients with EA has not yet been solidly investigated.

• #132 Ebstein’s Anomaly: Symptoms and Treatment | Doctor

  https://patient.info/doctor/ebsteins-anomaly-pro

  Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. […] Tricuspid valve repair is the goal of operative intervention. Repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation. […] Non-surgical management of Ebstein’s anomaly includes: prevention of infective endocarditis, management of heart failure, and treatment of arrhythmias. […] Increasingly the trend is for operative intervention early in the development of heart failure. Complete repair of Ebstein’s anomaly in symptomatic neonates has been shown to be possible, with good survival and excellent functional status.

• #133 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Surgical Management Newborns with Ebstein anomaly are initially observed as the pulmonary vascular resistance decreases. If surgery is required, a systemic-to-pulmonary artery shunt is typically employed to relieve cyanosis and secure adequate pulmonary and systemic circulations. Due to high periprocedural mortality, more definitive surgery is usually delayed unless the neonate meets specific indications. Data show that delaying surgery until heart failure or RV systolic dysfunction occurs is associated with worse outcomes. This is thought to be because Ebstein anomaly is a myopathic disorder in addition to abnormal valves, and the myopathic RV may be unable to tolerate increased volumes. Indications for surgery in neonates with Ebstein anomaly include: Right heart failure due to severe tricuspid regurgitation, A cardiothoracic ratio 80%, Severe cyanosis with dependency on PGE1, Mechanical ventilation dependency. In children and adults with Ebstein anomaly, indications for surgery include: Heart failure symptoms (New York Heart Association functional class III or IV), Progressive exercise intolerance, Evidence of RV dysfunction or progressive RV dilatation, Evidence of paradoxical emboli, Arrhythmias refractory to medical and catheter ablation therapies, Cyanosis with oxygen saturations 90%, Severe tricuspid regurgitation, Significant concomitant lesions such as pulmonic stenosis or atrial or ventricular septal defects, Progressive cardiomegaly, Cardiothoracic ratio 65%, Reduced left ventricular function. Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. For atrial arrhythmias in these patients, catheter ablation carries risks of paradoxical emboli, given the high incidence of interatrial shunts. A surgical biatrial Maze procedure during valvular repair is recommended. Multiple surgical approaches have been described for the treatment of Ebstein anomaly. Cone reconstruction is the currently preferred surgical approach in young children and adults. This approach involves mobilizing the anterior and posterior leaflets from their anomalous attachments, rotating the detached edges of these leaflets clockwise, and suturing them to the septal edge of the anterior leaflet at the level of the tricuspid valve annulus. The Danielson repair is the plication of the atrialized portion of the RV to narrow the size of the tricuspid valve and create a monoleaflet tricuspid valve that is competent. The Carpentier monocusp repair plicates the atrialized portion of the RV, implants a ring within the tricuspid annulus to provide support, mobilizes the anterior tricuspid valve leaflet, and attaches it to the annulus anteriorly. When the native tricuspid valve cannot be repaired, especially in older patients, it can be replaced with a bioprosthetic or mechanical valve. A bioprosthetic valve might require temporary anticoagulation; a mechanical valve will require lifelong anticoagulation. Mechanical valves are avoided when RV dysfunction is severe to mitigate disc motion abnormalities and the increased risk of valve thrombosis.

• #134 Ebstein anomaly: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/ebstein-anomaly-management-and-prognosis

  Ebstein anomaly: Management and prognosis […] The approach to the management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including the presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention. […] Indications for intervention or surgical repair of children and adults with Ebstein anomaly include worsening heart failure (HF), progressive cyanosis or uncontrolled arrhythmias. Surgical repair may also be considered if there is decline in functional capacity with evidence of right ventricular (RV) dysfunction and/or hypoxia. Occasionally, asymptomatic adult patients with severe tricuspid valve regurgitation and repairable tricuspid valves are considered for early repair to prevent progressive right heart enlargement.

• #135 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. […] Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. […] Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves.

• #136 Ebstein anomaly: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/ebstein-anomaly-management-and-prognosis

  Ebstein anomaly: Management and prognosis […] The approach to the management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including the presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention. […] Indications for intervention or surgical repair of children and adults with Ebstein anomaly include worsening heart failure (HF), progressive cyanosis or uncontrolled arrhythmias. Surgical repair may also be considered if there is decline in functional capacity with evidence of right ventricular (RV) dysfunction and/or hypoxia. Occasionally, asymptomatic adult patients with severe tricuspid valve regurgitation and repairable tricuspid valves are considered for early repair to prevent progressive right heart enlargement.

• #137 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #138 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. […] Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. […] Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves.

• #139 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. […] Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. […] Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves.

• #140 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. […] Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. […] Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves.

• #141 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #142 Ebstein’s anomaly: contemporary management strategies – Sainathan – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/37143/html

  If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. […] Heart transplantation should be considered in patients with associated left ventricular dysfunction. […] Surgical options include a biventricular repair pathway with a valvuloplasty. […] Anatomic pulmonary atresia is not a contraindication to a biventricular approach as an RV-PA conduit or a transannular patch with a monocusp can be used to establish continuity depending upon how satisfactory the valvuloplasty is. […] Some neonates who are hemodynamically unstable with some degree of end-organ damage yet have a favorable morphology for a biventricular repair and thus are unlikely to tolerate a prolonged procedure with a valvuloplasty or concerns exist for a possibility of a tenuous repair given the friability of neonatal valvular tissues can be channeled down the single ventricle pathway initially with a modified Starnes procedure.

• #143 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #144

  https://link.springer.com/article/10.1007/s11936-016-0478-2

  Ebsteins anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. […] Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. […] Pre-pregnancy evaluation should be considered in all Ebsteins anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. […] Longitudinal clinical follow-up in all Ebsteins anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. […] Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery.

• #145 Ebstein Anomaly | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/ebstein-anomaly

  Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. […] If the deformity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. […] If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. […] If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. […] Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.

• #146 Ebstein anomaly | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/ebstein-anomaly/

  Many children whose Ebstein anomaly is corrected by surgery can be as active as other kids. […] As the leading providers of pediatric heart care in Louisville and Southern Indiana, cardiothoracic surgeons at Norton Children’s Heart Institute are experienced with successfully repairing Ebstein anomaly. […] Our specialists have the skill and experience to know when Ebstein anomaly needs surgery and when it can be treated without surgery. […] The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child. […] When treatment is needed, the most common treatments used initially are: Oxygen, IV medications, Surgery. […] Babies born with Ebstein anomaly need continued follow-up care from a pediatric cardiologist (a doctor who specializes in treating heart conditions) because the heart’s ability to pump blood may change as the child continues to grow. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist to provide care.

• #147 Ebstein Anomaly | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/ebstein-anomaly

  Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. […] If the deformity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. […] If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. […] If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. […] Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.

• #148 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #149 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

  Catheter Interventions Some patients with Ebstein anomaly have a functional tricuspid valve and are not candidates for surgical valve repair. They may have a secundum atrial septal defect amenable to transcatheter device closure or tachyarrhythmias that can be treated in the cardiac catheterization laboratory. Many patients with intractable arrhythmias need definitive ablative therapy for supraventricular arrhythmias. Definitive ablative therapy is often challenging due to the frequent presence of multiple accessory pathways in the setting of a markedly enlarged right heart with a displaced tricuspid annulus and distortion of the anatomic landmarks; these all complicate catheter stability. Therefore, catheter ablation has a higher arrhythmia recurrence rate in patients with Ebstein anomaly than in structurally normal hearts. However, surgical cryoablation for accessory pathway-mediated tachycardia at the time of tricuspid valve repair and ASD closure can yield excellent results. Patients with atrial arrhythmias with ventricular preexcitation and those with multiple accessory pathways are at significantly increased risk of complications. Holter monitoring studies should be performed for arrhythmia symptoms, such as palpitations or syncope, and referral for further electrophysiologic studies should be made if indicated. There is a low threshold for electrophysiologic intervention prior to cardiac surgery, given the susceptibility of patients with Ebstein abnormality to arrhythmias.

• #150 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

• #151 Ebstein Anomaly | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/ebstein-anomaly

  Babies born with Ebstein anomaly usually are treated surgically in infancy so most adults with the condition only have mild symptoms. […] If the deformity is severe at birth, it may require immediate surgery. In adults with milder cases, treatment may include medications or special procedures to manage arrhythmia. People with Ebstein anomaly should be prescribed antibiotics before surgery or dental procedures to protect against endocarditis. […] If medication no longer controls symptoms or if the heart enlarges and loses efficiency, surgery may be required. The surgery may either repair the tricuspid valve or replace it with an artificial valve. It also may involve repairing any associated condition such as atrial septal defect. […] If arrhythmia is present, radiofrequency ablation, a procedure in which a burst of energy destroys an abnormal electrical pathway in the heart, may be necessary. Rarely, a permanent pacemaker will be implanted. […] Adults with Ebstein anomaly should be followed regularly by a cardiologist with a special interest in adult congenital heart disease. Yearly echocardiograms are necessary in all but the mildest cases.

• #152 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

• #153 Ebstein Anomaly and Malformation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK534824/

• #154 Ebsteins anomaly of the tricuspid valve medical therapy – wikidoc

  https://www.wikidoc.org/index.php/Ebsteins_anomaly_of_the_tricuspid_valve_medical_therapy

  Warfarin is recommended for patients with Ebsteins anomaly with a history of paradoxical embolus or atrial fibrillation. […] Diuretics may aid in reducing volume overload. Cardiac glycosides like digoxin can be used to maintain proper heart rhythm and increase contractility. […] Anticoagulation with warfarin is recommended for patients with Ebsteins anomaly with a history of paradoxical embolus or atrial fibrillation.

• #155 Ebsteins anomaly of the tricuspid valve medical therapy – wikidoc

  https://www.wikidoc.org/index.php/Ebsteins_anomaly_of_the_tricuspid_valve_medical_therapy

  Warfarin is recommended for patients with Ebsteins anomaly with a history of paradoxical embolus or atrial fibrillation. […] Diuretics may aid in reducing volume overload. Cardiac glycosides like digoxin can be used to maintain proper heart rhythm and increase contractility. […] Anticoagulation with warfarin is recommended for patients with Ebsteins anomaly with a history of paradoxical embolus or atrial fibrillation.

• #156 Ebstein Anomaly (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/ebstein-anomaly.html

  Doctors may recommend an electrophysiology study (EP study) when there are serious abnormal heart rhythms. During this study, ablation (freezing or burning) can get rid of electrical pathway fibers that make the heart beat too fast. […] Babies with Ebstein anomaly need care from a pediatric cardiologist (a doctor who diagnoses and treats children’s heart problems) because the heart’s pumping ability may fall short as the child grows. The doctor will recommend treatment that is tailored to a child’s needs. […] Many children whose Ebstein anomaly was corrected by surgery can be as active as other kids.

• #157 Ebstein Anomaly | Rady Children’s Hospital

  https://www.rchsd.org/health-article/ebstein-anomaly/

  Doctors may recommend an electrophysiology study (EP study) when there are serious abnormal heart rhythms. During this study, ablation (freezing or burning) can get rid of electrical pathway fibers that make the heart beat too fast. […] Babies with Ebstein anomaly need care from a pediatric cardiologist (a doctor who diagnoses and treats childrens heart problems) because the hearts pumping ability may fall short as the child grows. The doctor will recommend treatment that is tailored to a childs needs. […] Many children whose Ebstein anomaly was corrected by surgery can be as active as other kids.

• #158 Ebstein anomaly | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/ebstein-anomaly/

  Many children whose Ebstein anomaly is corrected by surgery can be as active as other kids. […] As the leading providers of pediatric heart care in Louisville and Southern Indiana, cardiothoracic surgeons at Norton Children’s Heart Institute are experienced with successfully repairing Ebstein anomaly. […] Our specialists have the skill and experience to know when Ebstein anomaly needs surgery and when it can be treated without surgery. […] The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child. […] When treatment is needed, the most common treatments used initially are: Oxygen, IV medications, Surgery. […] Babies born with Ebstein anomaly need continued follow-up care from a pediatric cardiologist (a doctor who specializes in treating heart conditions) because the heart’s ability to pump blood may change as the child continues to grow. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist to provide care.

• #159 Cone Procedure for Ebstein’s Anomaly | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/heart-surgery/programs-services/cone-procedure-ebsteins-anomaly

  Traditional management of Ebsteins Anomaly has involved tricuspid valve replacement. While this surgery can alleviate symptoms, long-term outcomes have been poor because the patient either outgrows the valve or it degenerates, thereby prompting additional medical or surgical interventions. […] The Adult Congenital Heart Disease Program in Phoenix Childrens nationally ranked Heart Center now offers cone reconstruction surgery of the tricuspid valve as an alternative treatment for patients with congenital heart defects. […] Any patient with Ebsteins Anomaly can choose cone reconstruction as their treatment of choice over valve replacement, however, the surgery is complex and should only be performed by cardiothoracic surgeons who have specialized training and firsthand experience. […] Since its initial unveiling, results of this autologous procedure have continued to show incredible promise with no chance of rejection.

• #160 Ebstein Centre at the German Heart Centre Munich – Herz- und Gefäßchirugie – DHM

  https://www.deutsches-herzzentrum-muenchen.de/en/department-of-cardiovascular-surgery/ebstein-centre-at-the-german-heart-centre-munich/

  The treatment of Ebsteins anomaly depends on the extent of the malformation and the symptoms. Therefore, it is our claim to treat each patient individually. Ebstein conferences are held regularly with the participation of all departments. Here we clarify the need for treatment, recommend examination methods and draw up treatment plans for each patient. The aim of treatment is to alleviate symptoms where present and to prevent complications such as enlargement of the heart, heart failure and cardiac arrhythmias. […] In some cases, no specific treatment is necessary and the disease can be simply monitored for many years. In other cases, surgery is necessary to preserve the tricuspid valve, improve its function and avert later complications. The indication for surgery is made on an interdisciplinary basis between cardiologists for congenital heart defects and cardiac surgeons.

• #161 Healthcare Providers | ebsteinsanomaly

  https://www.ebsteinsanomaly.org/healthcare-providers

  If your infant or child has been diagnosed with Ebsteins anomaly, an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. […] While very serious, Ebsteins Anomaly is treatable surgically at Boston Childrens Hospital, and the outlook for many babies with this cardiac defect is very promising! […] Their surgeons have a strong record of excellence in heart-valve repair and replacement, including minimally-invasive techniques. […] The cone procedure has been the worldwide standard for the surgical treatment of Ebsteins anomaly for years.

• #162 Ebstein’s Anomaly | Da Silva Center for Ebstein’s Anomaly | UPMC

  https://www.chp.edu/our-services/heart/cardiothoracic-surgery/da-silva-center/ebsteins-anomaly

  Ebstein’s anomaly treatments depend on the extent of the defect. Some potential treatments include: […] A variety of medications may help with treating Ebstein’s anomaly or to manage side effects from the condition. For example: […] A cone procedure, pioneered by Jose da Silva, MD, is the standard treatment for children with Ebsteins anomaly. […] It uses tissue from a child’s own malfunctioning tricuspid valve to create a new, cone-shaped valve. […] Other, less common surgeries for Ebsteins anomaly include: […] The Da Silva Center for Ebstein’s Anomaly at UPMC Children’s Hospital of Pittsburgh provides world-class care for people with congenital heart defects. Our experts are among the most experienced in the world at treating children with Ebsteins anomaly and other heart valve problems.

• #163 Ebstein Centre at the German Heart Centre Munich – Herz- und Gefäßchirugie – DHM

  https://www.deutsches-herzzentrum-muenchen.de/en/department-of-cardiovascular-surgery/ebstein-centre-at-the-german-heart-centre-munich/

  The treatment of Ebsteins anomaly depends on the extent of the malformation and the symptoms. Therefore, it is our claim to treat each patient individually. Ebstein conferences are held regularly with the participation of all departments. Here we clarify the need for treatment, recommend examination methods and draw up treatment plans for each patient. The aim of treatment is to alleviate symptoms where present and to prevent complications such as enlargement of the heart, heart failure and cardiac arrhythmias. […] In some cases, no specific treatment is necessary and the disease can be simply monitored for many years. In other cases, surgery is necessary to preserve the tricuspid valve, improve its function and avert later complications. The indication for surgery is made on an interdisciplinary basis between cardiologists for congenital heart defects and cardiac surgeons.

• #164 Ebstein’s Anomaly | Schneeweiss Adult Congenital Heart Center

  https://www.congenitalheart.cuimc.columbia.edu/conditions-we-treat/ebsteins-anomaly

  Such patients should be evaluated at an experienced Adult Congenital Heart Disease center to determine if they should undergo cardiac surgery and to assess arrhythmia treatment options. […] Although most of these arrhythmias can be successfully treated with catheter ablation, management is more complicated because of the anatomy and should be approached in conjunction with an assessment of the tricuspid valve and right ventricle. […] Some of these patients may be candidates for device closure of the atrial septal defect to eliminate cyanosis from the right to left shunt. However, before embarking on this approach it should be certain that the degree of tricuspid regurgitation is only mild. […] Ebstein’s anomaly is a complicated lesion, and management of adolescents and adults at an experienced center is advisable.

• #165

  https://journals.lww.com/cmj/fulltext/2024/05200/strategy_and_technique_for_surgical_treatment_of.10.aspx

  Ebstein’s anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in management strategies, surgical techniques, and operative indications for patients with Ebstein’s anomaly. […] Anatomical correction was performed in 203 (78.7%) operations, 1 ventricle repair in 38 (14.7%) operations, tricuspid valve repair only in four operations (1.6%), tricuspid valve replacement in ten (3.9%), total cavopulmonary connection (TCPC) in two (0.8%), and Glenn operation in one operation (0.4%). […] Based on our management strategies and operative principles and techniques, anatomical correction of EA is capable of achieving excellent long-term results, and low rates of TCPC, 1 ventricle repair and valvular replacement.

• #166

  https://journals.lww.com/cmj/fulltext/2024/05200/strategy_and_technique_for_surgical_treatment_of.10.aspx

  In line with other complex congenital heart diseases, biventricular repair technique should be always preferred for patients with EA. The severity of the patient’s malformation is the most important factor affecting the outcome of the operation. The strategy, surgical principles and techniques for patients with EA should be individualized. […] Reconstruction of tricuspid valve is a very important factor to the good results for surgical treatment of EA patients. It is imperative to providing sufficient surface area of valvular leaflets to cover the orifice of the tricuspid valve and to rebuild the sub-valvular apparatus. […] Due to the significant variety of pathological changes in EA patients, these factors exert a profound influence on the surgical outcomes. Therefore, we have to select different procedures for patients based on the findings before or during the operation. […] In conclusion, based on the management strategies, operative principles, and surgical techniques outlined above, anatomical correction procedures can achieve excellent long-term results with low rates of TCPC, 1 ventricle repair, and valvular replacement.

• #167 Cone Procedure for Ebstein’s Anomaly | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/heart-surgery/programs-services/cone-procedure-ebsteins-anomaly

  Traditional management of Ebsteins Anomaly has involved tricuspid valve replacement. While this surgery can alleviate symptoms, long-term outcomes have been poor because the patient either outgrows the valve or it degenerates, thereby prompting additional medical or surgical interventions. […] The Adult Congenital Heart Disease Program in Phoenix Childrens nationally ranked Heart Center now offers cone reconstruction surgery of the tricuspid valve as an alternative treatment for patients with congenital heart defects. […] Any patient with Ebsteins Anomaly can choose cone reconstruction as their treatment of choice over valve replacement, however, the surgery is complex and should only be performed by cardiothoracic surgeons who have specialized training and firsthand experience. […] Since its initial unveiling, results of this autologous procedure have continued to show incredible promise with no chance of rejection.

• #168

  https://link.springer.com/article/10.1007/s11936-014-0338-x

  Ebstein anomaly is a developmental abnormality of the tricuspid valve and right ventricle that results in tricuspid regurgitation and right heart enlargement. […] management of adults with Ebstein anomaly should take place in a center with expertise in adult congenital heart disease. In many patients, the decision regarding if and when to perform surgery remains controversial, largely because of a lack of published data demonstrating improved postoperative symptoms and survival compared to the natural history of the disease. […] Surgical tricuspid valve repair historically has not been highly successful because of the marked distortion of tricuspid valve leaflets and right ventricular pathology. Over the last several years, reports of newer surgical techniques to repair the valve, with concurrent advances in arrhythmia management of patients hold promise for improved long term outcomes of patients with Ebstein anomaly. […] However, because Ebstein anomaly is rare and tricuspid valve repair remains technically challenging, the newer valve repair techniques have not yet gained widespread acceptance throughout the adult congenital heart disease community.

• #169 Ebstein Centre at the German Heart Centre Munich – Herz- und Gefäßchirugie – DHM

  https://www.deutsches-herzzentrum-muenchen.de/en/department-of-cardiovascular-surgery/ebstein-centre-at-the-german-heart-centre-munich/

  At the German Heart Centre Munich, we have been offering the most modern surgical method for the reconstruction of the tricuspid valve, the Cone operation, since 2010. Prof. Rdiger Lange learned this surgical method in person from the first person to describe it, Prof. Da Silva, and introduced it in Munich. It is a very complicated operation that is only performed in a few centres worldwide. Here, the valve is reconstructed with its own tissue, thus avoiding an artificial valve replacement. We have already treated many patients with very good results.

• #170

  https://journals.lww.com/cmj/fulltext/2024/05200/strategy_and_technique_for_surgical_treatment_of.10.aspx

  Ebstein’s anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in management strategies, surgical techniques, and operative indications for patients with Ebstein’s anomaly. […] Anatomical correction was performed in 203 (78.7%) operations, 1 ventricle repair in 38 (14.7%) operations, tricuspid valve repair only in four operations (1.6%), tricuspid valve replacement in ten (3.9%), total cavopulmonary connection (TCPC) in two (0.8%), and Glenn operation in one operation (0.4%). […] Based on our management strategies and operative principles and techniques, anatomical correction of EA is capable of achieving excellent long-term results, and low rates of TCPC, 1 ventricle repair and valvular replacement.

• #171

  https://journals.lww.com/cmj/fulltext/2024/05200/strategy_and_technique_for_surgical_treatment_of.10.aspx

  In line with other complex congenital heart diseases, biventricular repair technique should be always preferred for patients with EA. The severity of the patient’s malformation is the most important factor affecting the outcome of the operation. The strategy, surgical principles and techniques for patients with EA should be individualized. […] Reconstruction of tricuspid valve is a very important factor to the good results for surgical treatment of EA patients. It is imperative to providing sufficient surface area of valvular leaflets to cover the orifice of the tricuspid valve and to rebuild the sub-valvular apparatus. […] Due to the significant variety of pathological changes in EA patients, these factors exert a profound influence on the surgical outcomes. Therefore, we have to select different procedures for patients based on the findings before or during the operation. […] In conclusion, based on the management strategies, operative principles, and surgical techniques outlined above, anatomical correction procedures can achieve excellent long-term results with low rates of TCPC, 1 ventricle repair, and valvular replacement.

• #172 EBSTEIN ANOMALY: A COMPLEX HEART DEFECT | Mya Care

  https://myacare.com/blog/ebstein-anomaly-a-complex-heart-defect

  The treatment for Ebstein Anomaly depends on the severity of the tricuspid regurgitation and the symptoms experienced by the individual. In some instances, treatment is not necessary, and the condition only requires close monitoring. […] If symptoms are present, surgery is often necessary to correct the defect. If left untreated, those with Ebstein anomaly may live to an average of 32 years old, whereas with surgical repair, they can live an average lifespan. […] Medications can assist with managing symptoms and improving heart function until surgery takes place. This benefits infants until they are old enough to receive surgery or older patients on a waiting list. […] Numerous surgeries can repair Ebstein’s Anomaly. Some of the main procedures include: […] Cone reconstruction surgery is an open heart surgery that involves reshaping the tricuspid valve to improve its function and reduce regurgitation, resulting in better long-term outcomes and improved heart function for those with Ebstein anomaly.

• #173 Ebstein Anomaly: A Comprehensive Guide

  https://longmoreclinic.org/ebstein-anomaly-a-comprehensive-guide/

  Treatment Options […] Treatment depends on the severity: […] Mild cases: Often only require regular monitoring. […] Medications: To address heart rhythm abnormalities or heart failure symptoms. […] Surgery: Especially in more severe cases, to repair or replace the tricuspid valve. […] Pacemakers: For those with significant heart rhythm issues. […] […] […] Current research is looking at the genetic markers and potential preventive strategies during pregnancy. With advances in regenerative medicine, there is hope that in the future, treatments may involve tissue engineering or even stem cell therapies to repair or replace the affected valve.

• #174 Ebstein’s Anomaly | Schneeweiss Adult Congenital Heart Center

  https://www.congenitalheart.cuimc.columbia.edu/conditions-we-treat/ebsteins-anomaly

  Such patients should be evaluated at an experienced Adult Congenital Heart Disease center to determine if they should undergo cardiac surgery and to assess arrhythmia treatment options. […] Although most of these arrhythmias can be successfully treated with catheter ablation, management is more complicated because of the anatomy and should be approached in conjunction with an assessment of the tricuspid valve and right ventricle. […] Some of these patients may be candidates for device closure of the atrial septal defect to eliminate cyanosis from the right to left shunt. However, before embarking on this approach it should be certain that the degree of tricuspid regurgitation is only mild. […] Ebstein’s anomaly is a complicated lesion, and management of adolescents and adults at an experienced center is advisable.

• #175 Ebstein Anomaly Treatment & Management: Medical Care, Surgical Care, Long-Term Monitoring

  https://emedicine.medscape.com/article/154447-treatment

  Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary. […] Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin. […] Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease.

• #176 Ebstein anomaly: Management and prognosis – UpToDate

  https://www.uptodate.com/contents/ebstein-anomaly-management-and-prognosis

  Ebstein anomaly: Management and prognosis […] The approach to the management of patients with Ebstein anomaly is determined by the patient’s age and clinical presentation including the presence of symptoms of heart failure secondary to tricuspid regurgitation and right heart failure, cyanosis, and right ventricular dilation or dysfunction. The components of management are monitoring, medical management (including temporizing relief of symptoms prior to surgery), management of arrhythmias, and surgical or catheter intervention. Many patients with Ebstein anomaly have no symptoms and require only monitoring while others are symptomatic and require supportive medical therapy and surgical intervention. […] Indications for intervention or surgical repair of children and adults with Ebstein anomaly include worsening heart failure (HF), progressive cyanosis or uncontrolled arrhythmias. Surgical repair may also be considered if there is decline in functional capacity with evidence of right ventricular (RV) dysfunction and/or hypoxia. Occasionally, asymptomatic adult patients with severe tricuspid valve regurgitation and repairable tricuspid valves are considered for early repair to prevent progressive right heart enlargement.

• #177 Ebstein’s anomaly: contemporary management strategies

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7139083/

  Ebsteins anomaly (EA) is a rare congenital cardiac anomaly. […] The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. […] The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. […] If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

• #178 Cone Procedure for Ebstein’s Anomaly | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/heart-surgery/programs-services/cone-procedure-ebsteins-anomaly

  Traditional management of Ebsteins Anomaly has involved tricuspid valve replacement. While this surgery can alleviate symptoms, long-term outcomes have been poor because the patient either outgrows the valve or it degenerates, thereby prompting additional medical or surgical interventions. […] The Adult Congenital Heart Disease Program in Phoenix Childrens nationally ranked Heart Center now offers cone reconstruction surgery of the tricuspid valve as an alternative treatment for patients with congenital heart defects. […] Any patient with Ebsteins Anomaly can choose cone reconstruction as their treatment of choice over valve replacement, however, the surgery is complex and should only be performed by cardiothoracic surgeons who have specialized training and firsthand experience. […] Since its initial unveiling, results of this autologous procedure have continued to show incredible promise with no chance of rejection.

• #179

  https://link.springer.com/article/10.1007/s11936-016-0478-2

  Ebsteins anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. […] Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. […] Pre-pregnancy evaluation should be considered in all Ebsteins anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. […] Longitudinal clinical follow-up in all Ebsteins anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. […] Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery.

• #180 Ebstein anomaly // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/ebstein-anomaly

  Surgery also may be recommended if other symptoms, such as some irregular heartbeats, are severe or affecting quality of life. […] If surgery is needed, it’s important to choose a surgeon who’s familiar with Ebstein anomaly. The surgeon should have training and experience performing procedures to correct the problem. […] Surgery to treat Ebstein anomaly and related heart problems may include: Tricuspid valve repair. This open-heart surgery fixes a damaged tricuspid valve. […] Tricuspid valve replacement. If the valve can’t be repaired, surgery to replace the valve may be needed. […] Closure of the atrial septal defect. This surgery is done to fix a hole between the upper chambers of the heart. […] Maze procedure. If Ebstein anomaly causes irregular heartbeats, this procedure may be done during valve repair or replacement surgery. […] Radiofrequency catheter ablation. This procedure treats fast or irregular heartbeats. […] Heart transplant. If severe Ebstein anomaly is causing the heart to fail, a heart transplant might be needed.

• #181 Ebstein’s Anomaly | Schneeweiss Adult Congenital Heart Center

  https://www.congenitalheart.cuimc.columbia.edu/conditions-we-treat/ebsteins-anomaly

  Such patients should be evaluated at an experienced Adult Congenital Heart Disease center to determine if they should undergo cardiac surgery and to assess arrhythmia treatment options. […] Although most of these arrhythmias can be successfully treated with catheter ablation, management is more complicated because of the anatomy and should be approached in conjunction with an assessment of the tricuspid valve and right ventricle. […] Some of these patients may be candidates for device closure of the atrial septal defect to eliminate cyanosis from the right to left shunt. However, before embarking on this approach it should be certain that the degree of tricuspid regurgitation is only mild. […] Ebstein’s anomaly is a complicated lesion, and management of adolescents and adults at an experienced center is advisable.

• #182 Ebstein’s anomaly – Wikipedia

  https://en.wikipedia.org/wiki/Ebstein%27s_anomaly

  Ebstein’s cardio physiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred medication treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated. […] If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response. […] The Canadian Cardiovascular Society (CCS) recommends surgical intervention for these indications: Limited exercise capacity (NYHA III-IV), Increasing heart size (cardiothoracic ratio greater than 65%), Important cyanosis (resting oxygen saturation less than 90% – level B), Severe tricuspid regurgitation with symptoms, Transient ischemic attack or stroke. […] The CCS further recommends patients who require operation for Ebstein’s anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.

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