Materiały źródłowe

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

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

  Ebstein anomaly is a rare congenital heart disease characterized by apical displacement of the tricuspid valve, adherence of the septal and posterior leaflets to the myocardium, and atrialization of the inlet portion of the right ventricle. […] While most patients with Ebstein anomaly will be symptomatic at birth or in early childhood, some patients will not be diagnosed until their sixth or seventh decade. […] The clinical manifestations of Ebstein anomaly range from asymptomatic to severe as dictated by the degree of tricuspid valve displacement, severity of tricuspid regurgitation, effective right ventricular volume, and the presence of associated malformations such as an atrial septal defect or pulmonary valve stenosis or atresia. […] This activity for healthcare professionals reviews the etiology, epidemiology, pathophysiologic mechanisms, clinical presentation, evaluation, and management of Ebstein anomaly and highlights the role of the interprofessional team in improving outcomes for patients with this rare but complex cardiac malformation.

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

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

  Ebsteins anomaly is a rare birth defect. […] If you’re diagnosed as an adult, the condition is usually mild, although some people may need surgery. […] A provider will review your symptoms and check your vital signs. You may need tests to find out how well your heart is functioning, such as: […] A chest X-ray can show an enlarged heart. A cardiac MRI provides greater detail of the valves and chambers. […] An echocardiogram helps your provider check the structure and function of your heart valves and chambers. […] This test measures the electrical impulses of your heart. An electrocardiogram can detect an abnormal heart rhythm. […] 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:

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

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

  To diagnose Ebstein anomaly, a healthcare professional examines the baby and listens to the heart and lungs. A heart sound called a murmur may be heard. […] Tests that are done to help diagnose Ebstein anomaly include: […] Pulse oximetry. In this test, a sensor attached to a finger or toe measures the amount of oxygen in the blood. […] Echocardiogram. Sound waves are used to create pictures of the beating heart. An echocardiogram can show how blood flows through the heart and heart valves. […] Electrocardiogram (ECG or EKG). This simple test checks the heartbeat. Sticky patches attach to the chest and sometimes the arms and legs. Wires connect the patches to a computer, which prints or displays the results. […] Holter monitor. This portable ECG device is worn for a day or more to record the heart’s activity during daily activities.

• #4 Ebstein Anomaly: Practice Essentials, Background, Pathophysiology

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

  Physical findings in patients with Ebstein anomaly span a spectrum from subtle to dramatic. They may include the following: […] A 12-lead electrocardiogram may demonstrate the following findings in patients with Ebstein anomaly: […] The following imaging studies may be used to assess patients with suspected Ebstein anomaly: […] 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. Treatment options include medical therapy, radiofrequency ablation, and surgical therapy. […] 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.

• #5 Ebstein Anomaly | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/e/ebstein

  Ebstein anomaly is a defect of the tricuspid valve. […] A chest X-ray will be taken to look at the size of the heart, which may be larger than normal. Often, the diagnosis of Ebstein anomaly is suspected because of the large heart on chest X-ray. […] An echocardiogram is used to diagnose Ebstein anomaly. It also helps identify any additional heart defects. This test allows the pediatric cardiologist (heart doctor) to determine the degree of valve displacement, the severity of valve leakage (insufficiency) or valve narrowing (stenosis). it also helps see the size of the heart chambers, and if an open foramen ovale is present. […] An electrocardiogram (ECG) records the heart’s rhythm. If your child has complained about a racing heart and the answer is not found in this initial test, they may go home with a recorder. Your child may also have an exercise stress test done to better look at their heart function during activity. Some patients with abnormal heart rhythms may need more testing to identify and treat their heart rhythm problems. […] Invasive diagnostic testing is not as commonly performed today. Certain patients with Ebstein anomaly may need cardiac catheterization to fully look at their cardiac anatomy and function.

• #6 Diagnosis and natural history of Ebstein’s anomaly.

  https://pmc.ncbi.nlm.nih.gov/articles/PMC481939/

  Thirty five cases of Ebstein’s anomaly were diagnosed by cardiac catheter study or echocardiography between 1962 and 1984. […] Echocardiography is the procedure of choice for diagnosis of Ebstein’s anomaly. […] Diagnosis at initial catheter study was incorrect in four patients. […] Earlier M mode measurements of time delay of tricuspid closure compared with mitral closure did not always lead to the correct diagnosis. […] Cross sectional studies gave good images of tricuspid leaflet displacement, its tethering, and the atrialised portion of the right ventricle and have facilitated the diagnosis of more cases in recent years.

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

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

  Our pediatric cardiology specialists are prepared to make an accurate diagnosis. […] Through ultrasound imaging during pregnancy, we may be able to diagnose Ebstein’s anomaly before your baby is born. We may also diagnose the condition after birth. In either case, we will carefully evaluate your child and help you decide next steps. Your child’s diagnostic work-up may include: […] Echocardiogram—a safe, noninvasive procedure that uses high frequency ultrasound to show the structure of your child’s heart, how blood flows through the heart, and how your child’s heart and circulatory system are working overall. […] In addition to Ebstein’s anomaly, it is possible that your child’s heart may have other defects (such as an atrial septal defect) or an abnormal heart beat from an electrical problem in the heart. Our pediatric cardiology specialists will get the full picture during our diagnostic process and develop a treatment plan for your child.

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

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

  Assess patients with Ebstein anomaly through the interpretation of results from routine and advanced cardiac imaging techniques. […] Echocardiography is the imaging study of choice to evaluate Ebstein anomaly. […] The first echocardiographic criteria to detect Ebstein anomaly is apical displacement of the septal leaflet of the tricuspid valve greater than 8 mm/m2 in the apical 4-chamber view. […] In infants with Ebstein anomaly, the mainstay of treatment is supportive to reduce pulmonary vascular resistance and hypoxemia. […] 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.

• #9 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. […] The present review sought to shed light on the wide grey zone of post-neonatal Ebsteins manifestations, highlighting current gaps and achievements in knowledge for adequate risk assessment and appropriate therapeutic strategy. […] In order to tackle the extreme heterogeneity of Ebsteins anomaly, this review displays the multimodality imaging assessment necessary for a proper anatomical classification and the multidisciplinary approach needed for a comprehensive risk stratification and monitoring strategy. […] The quantitative criterion for EA diagnosis is an apical displacement of the SL hinge point by at least 8mm/m2 from the anterior mitral leaflet insertion, assessed in four-chamber view.

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

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

  An absolute distance in atrioventricular valves offsetting of 15mm in children and 20mm in adults is also considered diagnostic. […] Multimodality imaging is pivotal not only for anatomical and functional assessment of TV and right-sided chambers but also for identifying associated lesions. […] Transthoracic echocardiography (TTE) is usually the first diagnostic tool. […] Second-line imaging includes transoesophageal echocardiography (TOE) and cardiovascular magnetic resonance (CMR), the former with particular usefulness in TV assessment, the latter in right-sided chamber volumes calculation and myocardial characterization. […] CMR is superior to TTE in the detection of PL and extra-cardiac abnormalities, while TTE reveals small septal communications more frequently. […] Echocardiography and CMR are both recommended for evaluating unoperated patients, informing on progressive disease and anatomies suitable of repair, as well as for monitoring operated patients, revealing surgical results and possible complications.

• #11 Ebstein’s anomaly

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/ebstein-s-anomaly.html

  This leaflet is to help you understand what Ebsteins anomaly is, what tests you need, and the implication of being diagnosed for your baby and your family. […] Your caregiver may refer you for genetic counseling and genetic testing. This can provide essential information regarding your individual case. In addition, your caregiver may refer you to specialists in fetal heart problems, such as a maternal-fetal medicine specialist and/or a pediatric cardiologist. They can monitor your babys progress with specialized ultrasound scans focused on the fetal heart and the blood vessels around it. Those specialized ultrasounds are called fetal echocardiography. The tests offered by your caregiver will depend on many associated factors. […] Your caregiver may order serial ultrasound scans to assess whether the Ebsteins anomaly is affecting your babys growth and well-being. Your caregivers will advise you as the pregnancy progresses.

• #12 Ebstein’s Anomaly | Texas Children’s

  https://www.texaschildrens.org/content/conditions/ebsteins-anomaly

  Ebstein’s anomaly may be detected during a routine prenatal ultrasound. Additional testing, including fetal echocardiography, is typically needed to confirm the diagnosis and learn more about the defect. A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and expertise required by the baby at birth, optimizing outcomes. […] In other cases, the condition may not be diagnosed until after the baby is born. The doctor may detect a heart murmur, an abnormal sound caused by blood flow problems in the heart. Testing may include an electrocardiogram (ECG or EKG), an echocardiogram, a chest X-ray, and cardiac catheterization.

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

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

  If the fetal tricuspid valve appears abnormal or is leaking and the heart is enlarged on a fetal ultrasound, Ebstein’s Anomaly may be suspected and a fetal echocardiogram (echo) will be performed. […] Severe cases of Ebsteins Anomaly carry an increased risk of fetal demise compared to other congenital heart defects, so close monitoring is essential. […] Most babies with a mild form of Ebsteins Anomaly can be delivered vaginally, unless there are obstetric indications for another mode of delivery. […] For mild cases, medication may be all your child needs. For more severe cases, surgery in the newborn period may be necessary. […] Children who have Ebsteins Anomaly will require monitoring and medication throughout their life. Children with more severe cases will need additional surgeries throughout their lifetime. […] The long-term prognosis after surgery for Ebsteins Anomaly depends on the severity of the defect. Your child will need to continue to take medication and go to follow-up visits with a cardiologist.

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

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

  Chest X-ray. A chest X-ray is a picture of the heart, lungs and blood vessels. It can show if the heart is enlarged. […] Cardiac MRI. A cardiac MRI uses magnetic fields and radio waves to create images of the heart. This test can give a detailed view of the tricuspid valve. It also shows the size of the heart chambers and how well they work. […] Exercise stress tests. These tests often involve walking on a treadmill or riding a stationary bike while the heart is checked. An exercise stress test can show how the heart reacts to exercise. […] Electrophysiology study (EP study). A doctor threads a thin, flexible tube called a catheter into a blood vessel and guides it to the heart. More than one catheter may be used. Sensors on the tip of the catheter give off electrical signals and record the heart’s electricity. This test helps show which part of the heart is causing a fast or irregular heartbeat. Treatment of an irregular heartbeat may be done during this test.

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

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

  Ebsteins anomaly is a rare birth defect. […] If you’re diagnosed as an adult, the condition is usually mild, although some people may need surgery. […] A provider will review your symptoms and check your vital signs. You may need tests to find out how well your heart is functioning, such as: […] A chest X-ray can show an enlarged heart. A cardiac MRI provides greater detail of the valves and chambers. […] An echocardiogram helps your provider check the structure and function of your heart valves and chambers. […] This test measures the electrical impulses of your heart. An electrocardiogram can detect an abnormal heart rhythm. […] 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:

• #16 Ebstein anomaly – WikEM

  https://wikem.org/wiki/Ebstein_anomaly

  Ebstein anomaly showing the downward displacement of the tricuspid valve from its normal position in the fibrous ring down into the right ventricle. […] ECG of a woman with Ebstein’s anomaly showing 1) signs of right atrial enlargement (best seen in V1), 2) broad and tall P waves („Himalayan” P waves), and 3) right bundle-branch block pattern and a first-degree atrioventricular block (prolonged PR-interval) due to intra-atrial conduction delay. […] Echocardiography […] Apical displacement of septal leaflet of tricuspid valve […] Tethering of tricuspid valve […] Right atrial enlargement […] Atrialization of right ventricle […] Tricuspid regurgitation. […] Cardiac MR […] Useful when echo image quality is inadequate. […] ECG […] Tall, broad P waves due to right atrial enlargement […] Complete or incompletely right bundle branch block […] Small R wave in V1 and V2 […] Bizarre QRS due to conduction abnormalities. […] CXR […] Enlarged cardiac silhouette […] 'Globe-shaped’ heart with narrow waist.

• #17 Narrative review of Ebstein’s anomaly beyond childhood: Imaging, surgery, and future perspectives – Neumann – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/62524/html

  Ebsteins anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. […] Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. […] Surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. […] The assessment of right ventricular function plays an important role in the timing of surgical intervention. […] CMR is an important adjunct to echocardiography and the more accurate and arguably preferable modality to quantify ventricular function and volumes as valuable follow-up parameters. […] In summary, contemporary magnetic resonance imaging scanners offer a comprehensive, multiplane evaluation of cardiac morphology, function and tissue (viability) status independent of body habitus and acoustic windows, making it particularly attractive for adult patients with CHD. […] Timing of surgery remains subject of further evaluation to improve postoperative right ventricular performance.

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

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

  An absolute distance in atrioventricular valves offsetting of 15mm in children and 20mm in adults is also considered diagnostic. […] Multimodality imaging is pivotal not only for anatomical and functional assessment of TV and right-sided chambers but also for identifying associated lesions. […] Transthoracic echocardiography (TTE) is usually the first diagnostic tool. […] Second-line imaging includes transoesophageal echocardiography (TOE) and cardiovascular magnetic resonance (CMR), the former with particular usefulness in TV assessment, the latter in right-sided chamber volumes calculation and myocardial characterization. […] CMR is superior to TTE in the detection of PL and extra-cardiac abnormalities, while TTE reveals small septal communications more frequently. […] Echocardiography and CMR are both recommended for evaluating unoperated patients, informing on progressive disease and anatomies suitable of repair, as well as for monitoring operated patients, revealing surgical results and possible complications.

• #19 Ebstein Anomaly | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/e/ebstein

  Ebstein anomaly is a defect of the tricuspid valve. […] A chest X-ray will be taken to look at the size of the heart, which may be larger than normal. Often, the diagnosis of Ebstein anomaly is suspected because of the large heart on chest X-ray. […] An echocardiogram is used to diagnose Ebstein anomaly. It also helps identify any additional heart defects. This test allows the pediatric cardiologist (heart doctor) to determine the degree of valve displacement, the severity of valve leakage (insufficiency) or valve narrowing (stenosis). it also helps see the size of the heart chambers, and if an open foramen ovale is present. […] An electrocardiogram (ECG) records the heart’s rhythm. If your child has complained about a racing heart and the answer is not found in this initial test, they may go home with a recorder. Your child may also have an exercise stress test done to better look at their heart function during activity. Some patients with abnormal heart rhythms may need more testing to identify and treat their heart rhythm problems. […] Invasive diagnostic testing is not as commonly performed today. Certain patients with Ebstein anomaly may need cardiac catheterization to fully look at their cardiac anatomy and function.

• #20 Ebstein anomaly – WikEM

  https://wikem.org/wiki/Ebstein_anomaly

  Ebstein anomaly showing the downward displacement of the tricuspid valve from its normal position in the fibrous ring down into the right ventricle. […] ECG of a woman with Ebstein’s anomaly showing 1) signs of right atrial enlargement (best seen in V1), 2) broad and tall P waves („Himalayan” P waves), and 3) right bundle-branch block pattern and a first-degree atrioventricular block (prolonged PR-interval) due to intra-atrial conduction delay. […] Echocardiography […] Apical displacement of septal leaflet of tricuspid valve […] Tethering of tricuspid valve […] Right atrial enlargement […] Atrialization of right ventricle […] Tricuspid regurgitation. […] Cardiac MR […] Useful when echo image quality is inadequate. […] ECG […] Tall, broad P waves due to right atrial enlargement […] Complete or incompletely right bundle branch block […] Small R wave in V1 and V2 […] Bizarre QRS due to conduction abnormalities. […] CXR […] Enlarged cardiac silhouette […] 'Globe-shaped’ heart with narrow waist.

• #21 Ebstein’s anomaly – causes, symptoms, diagnosis and treatment | Longevity

  https://vocal.media/longevity/ebstein-s-anomaly-causes-symptoms-diagnosis-and-treatment

  Radiological signs indicating Ebstein’s anomaly are represented by a sharp increase in the right parts of the heart, a spherical shape of the heart shadow, increased transparency of the pulmonary fields. […] During echocardiography, a downward displacement of the tricuspid valve flaps, an increase in the size of the right atrium, delayed closure of the tricuspid valve, a displacement of the flaps, the presence of an atrialized right ventricle, a shunting blood duct from right to left through ASD (according to Doppler echocardiography) is visible. […] To clarify the form and severity of Ebstein’s anomaly, MRI, probing of the heart cavities, ventriculography are performed. The diagnosis of Ebstein’s anomaly requires differentiation with exudative pericarditis, Abramov-Fiedler myocarditis, isolated ASD and pulmonal stenosis, Fallot’s tetrad.

• #22 Ebstein anomaly – WikEM

  https://wikem.org/wiki/Ebstein_anomaly

  Ebstein anomaly showing the downward displacement of the tricuspid valve from its normal position in the fibrous ring down into the right ventricle. […] ECG of a woman with Ebstein’s anomaly showing 1) signs of right atrial enlargement (best seen in V1), 2) broad and tall P waves („Himalayan” P waves), and 3) right bundle-branch block pattern and a first-degree atrioventricular block (prolonged PR-interval) due to intra-atrial conduction delay. […] Echocardiography […] Apical displacement of septal leaflet of tricuspid valve […] Tethering of tricuspid valve […] Right atrial enlargement […] Atrialization of right ventricle […] Tricuspid regurgitation. […] Cardiac MR […] Useful when echo image quality is inadequate. […] ECG […] Tall, broad P waves due to right atrial enlargement […] Complete or incompletely right bundle branch block […] Small R wave in V1 and V2 […] Bizarre QRS due to conduction abnormalities. […] CXR […] Enlarged cardiac silhouette […] 'Globe-shaped’ heart with narrow waist.

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

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

  Diagnosis is with ECG and imaging. […] The ECG is usually abnormal with right axis deviation, low voltage right bundle branch block, and first-degree atrioventricular block. […] Chest x-ray findings include a variable degree of cardiomegaly with prominent right atrial borders. […] Echocardiography clearly delineates the anatomic and functional abnormalities of the tricuspid valve and any associated defects. […] MRI provides valuable additional detail regarding the tricuspid valve, which is helpful in planning surgical intervention.

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

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

  To diagnose Ebstein anomaly, a healthcare professional examines the baby and listens to the heart and lungs. A heart sound called a murmur may be heard. […] Tests that are done to help diagnose Ebstein anomaly include: […] Pulse oximetry. In this test, a sensor attached to a finger or toe measures the amount of oxygen in the blood. […] Echocardiogram. Sound waves are used to create pictures of the beating heart. An echocardiogram can show how blood flows through the heart and heart valves. […] Electrocardiogram (ECG or EKG). This simple test checks the heartbeat. Sticky patches attach to the chest and sometimes the arms and legs. Wires connect the patches to a computer, which prints or displays the results. […] Holter monitor. This portable ECG device is worn for a day or more to record the heart’s activity during daily activities.

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

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

  To diagnose Ebstein anomaly, a healthcare professional examines the baby and listens to the heart and lungs. A heart sound called a murmur may be heard. […] Tests that are done to help diagnose Ebstein anomaly include: […] Pulse oximetry. In this test, a sensor attached to a finger or toe measures the amount of oxygen in the blood. […] Echocardiogram. Sound waves are used to create pictures of the beating heart. An echocardiogram can show how blood flows through the heart and heart valves. […] Electrocardiogram (ECG or EKG). This simple test checks the heartbeat. Sticky patches attach to the chest and sometimes the arms and legs. Wires connect the patches to a computer, which prints or displays the results. […] Holter monitor. This portable ECG device is worn for a day or more to record the heart’s activity during daily activities.

• #26

  https://www.sgh.com.sg/patient-care/conditions-treatments/ebstein-anomaly

  Your doctor may review your signs and symptoms and conduct a physical examination. If your doctor suspects Ebstein Anomaly, your doctor may recommend several tests, including: […] Echocardiogram assesses the structure of your heart, the tricuspid valve and the blood flow through your heart. […] Your doctor may also order a transesophageal echocardiogram to provide a detailed image of your heart. […] Electrocardiogram (ECG) can help your doctor detect irregularities in your heart’s rhythm and structure, and offer clues as to the presence of an extra accessory pathway. […] Chest X-ray can reveal if your heart is enlarged, which may be due to Ebstein Anomaly. […] Cardiac MRI may be used to determine the severity of your condition, get a detailed view of the tricuspid valve, and assess the size and function of your lower right heart chamber (right ventricle). […] Holter monitor may be used to diagnose rhythm disturbances that occur at unpredictable times. You wear the monitor under your clothing and it records information about the electrical activity of your heart as you go about your normal activities for a day or two.

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

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

  Chest X-ray. A chest X-ray is a picture of the heart, lungs and blood vessels. It can show if the heart is enlarged. […] Cardiac MRI. A cardiac MRI uses magnetic fields and radio waves to create images of the heart. This test can give a detailed view of the tricuspid valve. It also shows the size of the heart chambers and how well they work. […] Exercise stress tests. These tests often involve walking on a treadmill or riding a stationary bike while the heart is checked. An exercise stress test can show how the heart reacts to exercise. […] Electrophysiology study (EP study). A doctor threads a thin, flexible tube called a catheter into a blood vessel and guides it to the heart. More than one catheter may be used. Sensors on the tip of the catheter give off electrical signals and record the heart’s electricity. This test helps show which part of the heart is causing a fast or irregular heartbeat. Treatment of an irregular heartbeat may be done during this test.

• #28 Ebstein Anomaly | Conditions | UCSF Health

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

  Check-ups with a specialist in congenital heart disease in adults would include: […] Electrocardiogram (EKG or ECG), which records the heart’s electrical activity […] Chest X-ray to see the heart’s size […] Echocardiogram, which is an ultrasound examination of the heart […] Exercise test, measures how much oxygen the heart can provide to your muscles while you exercise […] Holter monitor or event recorder in case of arrhythmia […] Electrophysiology studies.

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

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

  Chest X-ray. A chest X-ray is a picture of the heart, lungs and blood vessels. It can show if the heart is enlarged. […] Cardiac MRI. A cardiac MRI uses magnetic fields and radio waves to create images of the heart. This test can give a detailed view of the tricuspid valve. It also shows the size of the heart chambers and how well they work. […] Exercise stress tests. These tests often involve walking on a treadmill or riding a stationary bike while the heart is checked. An exercise stress test can show how the heart reacts to exercise. […] Electrophysiology study (EP study). A doctor threads a thin, flexible tube called a catheter into a blood vessel and guides it to the heart. More than one catheter may be used. Sensors on the tip of the catheter give off electrical signals and record the heart’s electricity. This test helps show which part of the heart is causing a fast or irregular heartbeat. Treatment of an irregular heartbeat may be done during this test.

• #30 Ebstein Anomaly | Conditions | UCSF Health

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

  Check-ups with a specialist in congenital heart disease in adults would include: […] Electrocardiogram (EKG or ECG), which records the heart’s electrical activity […] Chest X-ray to see the heart’s size […] Echocardiogram, which is an ultrasound examination of the heart […] Exercise test, measures how much oxygen the heart can provide to your muscles while you exercise […] Holter monitor or event recorder in case of arrhythmia […] Electrophysiology studies.

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

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

  Ebstein anomaly might be seen on ultrasound scans before birth. It may be recognized at birth because the baby’s skin looks blue or the baby’s heart makes unusual sounds. […] The best test to confirm Ebstein anomaly is an echocardiogram (ultrasound of the heart). […] Other tests may include: Chest X-ray, which often shows a large heart; Electrocardiogram (ECG or EKG), a recording of the heart’s electrical activity. It may show abnormal heartbeats or signs that the heart’s right chambers are enlarged. […] Exercise test (for older kids who can follow instructions).

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

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

  To diagnose Ebstein anomaly, a healthcare professional examines the baby and listens to the heart and lungs. A heart sound called a murmur may be heard. […] Tests that are done to help diagnose Ebstein anomaly include: […] Pulse oximetry. In this test, a sensor attached to a finger or toe measures the amount of oxygen in the blood. […] Echocardiogram. Sound waves are used to create pictures of the beating heart. An echocardiogram can show how blood flows through the heart and heart valves. […] Electrocardiogram (ECG or EKG). This simple test checks the heartbeat. Sticky patches attach to the chest and sometimes the arms and legs. Wires connect the patches to a computer, which prints or displays the results. […] Holter monitor. This portable ECG device is worn for a day or more to record the heart’s activity during daily activities.

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

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

  If your newborn baby is born with a bluish tint to the skin, or your child’s pediatrician suspects a heart condition, you will be referred to a pediatric cardiologist for a physical exam. The doctor will listen to your baby’s heart and lungs, measure the oxygen level in his or her blood (non-invasively), and make other observations to help determine the diagnosis. […] In some cases, Ebstein’s anomaly is found before birth during a fetal ultrasound. […] The doctor may order one or more of the following tests to help with the diagnosis: Echocardiogram (cardiac ultrasound), Cardiac magnetic resonance imaging (MRI), Chest X-ray, Electrocardiogram (EKG or ECG).

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

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

  Cardiac catheterization. A doctor places a long, thin flexible tube into a blood vessel, usually in the groin or wrist. It’s guided to the heart. Dye flows through the tube to arteries in the heart. The dye helps the arteries show up more clearly on X-ray images and video. During the test, pressures and oxygen levels can be measured in different parts of the heart. Some heart disease treatments also can be done during this test.

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

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

  Heart Catheterization: This is a test to measure pressures in the heart. It can also take pictures of the heart and open a narrowing with a balloon or small metal coil, called a stent. Heart catheterizations are done while your child is asleep with medicines called anesthesia. […] Cardiac MRI: A test that uses radio waves, magnets, and a computer to make detailed pictures of the heart and blood vessels. Cardiac MRI can provide detailed information on the type and severity of heart disease.

• #36 Cardiac Cath to Diagnose Ebstein’s Anomaly | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/ebsteins-anomaly/diagnosis/cardiac-catheterization.html

  Chest X-ray […] Electrocardiogram […] Echocardiogram […] Exercise electrocardiogram […] Cardiac MRI […] Cardiac catheterization […] In cardiac catheterization (often called cardiac cath), a very small hollow tube, or catheter, is advanced from a blood vessel in the groin or arm through the aorta into the heart. Once the catheter is in place, several diagnostic techniques may be used. The tip of the catheter can be placed into various parts of the heart to measure the pressures within the chambers.

• #37 Ebstein Anomaly | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/e/ebstein

  Ebstein anomaly is a defect of the tricuspid valve. […] A chest X-ray will be taken to look at the size of the heart, which may be larger than normal. Often, the diagnosis of Ebstein anomaly is suspected because of the large heart on chest X-ray. […] An echocardiogram is used to diagnose Ebstein anomaly. It also helps identify any additional heart defects. This test allows the pediatric cardiologist (heart doctor) to determine the degree of valve displacement, the severity of valve leakage (insufficiency) or valve narrowing (stenosis). it also helps see the size of the heart chambers, and if an open foramen ovale is present. […] An electrocardiogram (ECG) records the heart’s rhythm. If your child has complained about a racing heart and the answer is not found in this initial test, they may go home with a recorder. Your child may also have an exercise stress test done to better look at their heart function during activity. Some patients with abnormal heart rhythms may need more testing to identify and treat their heart rhythm problems. […] Invasive diagnostic testing is not as commonly performed today. Certain patients with Ebstein anomaly may need cardiac catheterization to fully look at their cardiac anatomy and function.

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

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

  Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. […] The Celermajer index is an established score that uses echocardiography to predict mortality. […] The main predictors of mortality in patients with Ebstein anomaly include the degree of tricuspid displacement, amount of tricuspid regurgitation, and duration and degree of RV dysfunction.

• #39 Ebstein anomaly: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/ebstein-anomaly-clinical-manifestations-and-diagnosis

  Ebstein anomaly is a congenital malformation that is characterized primarily by abnormalities of the tricuspid valve and right ventricle. The clinical presentation of Ebstein anomaly varies widely, ranging from the critically ill fetus to the asymptomatic adult, depending upon the degree of anatomic abnormality. The clinical manifestations and diagnosis of Ebstein anomaly are discussed here. […] […] DIAGNOSIS AND EVALUATION: When to suspect Ebstein anomaly, How to diagnose Ebstein anomaly, Approach to diagnosis and evaluation, Key imaging tests: Echocardiography, Key features, GOSE score (Celermajer index), Cardiovascular magnetic resonance. […] […] The morphology of the tricuspid valve in Ebstein anomaly, and consequently the clinical presentation, is highly variable. The tricuspid valve leaflets demonstrate variable degrees of failed delamination (separation of the valve tissue from the myocardium) with fibrous and muscular attachments to the right ventricular myocardium. […] […] The displacement of hinge points of septal and posterior (inferior) leaflets into the right ventricle toward the apex and right ventricular outflow tract is the hallmark finding of Ebstein anomaly.

• #40

  https://journals.lww.com/jiae/fulltext/2019/03020/prenatal_diagnosis_of_ebstein_s_anomaly__an.9.aspx

  Fetal echocardiogram is an essential diagnostic tool for the early detection of EA and its severity. The histopathology studies proved that lung hypoplasia may not be a feature of EA, but severe cardiomegaly and effusion can restrict the lung growth in later gestation. Looking at the routine essential echo parameters in the diagnosis of EA such as, cardiothoracic ratio, severity of TR, TV annulus diameter z score, pulmonary valve regurgitation, ductal reversal flow, no antegrade PV flow, pericardial effusion, hydrops, and fetal arrhythmia would be helpful in predicting severity and outcome, even intrauterine death, warranted for safe termination.

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

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

  Surgical intervention comprises tricuspid valve repair and patch closure of the atrial septal defect. […] The Celermajer index is an established score that uses echocardiography to predict mortality. […] The main predictors of mortality in patients with Ebstein anomaly include the degree of tricuspid displacement, amount of tricuspid regurgitation, and duration and degree of RV dysfunction.

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

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

  Over time, various classifications of EA severity had been proposed. […] Imaging parameters predictive of adverse outcome in EA are reported in online supplemental tables 2 and 3. […] A clinical follow-up is recommended at least yearly in patients with EA, although the monitoring strategy is tailored according to patients specific features. […] EA diagnosis or diagnostic suspect should guide patients referral to a specialised multidisciplinary team. EA spectrum of heterogeneous anatomical and clinical features requires a patient-tailored monitoring strategy.

• #43 Prenatal Diagnosis of Ebstein¿s Anomaly – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-prenatal-diagnosis-ebsteins-anomaly-articulo-13125895

  Ebstein’s anomaly accounts for 0.5% of all congenital heart diseases. The condition is characterized by 8 mm/m2 displacement of the septal and posterobasal tricuspid leaflets toward the apex of the right ventricle (RV). This causes „atrialization” of the RV inflow tract, with decreased size of the RV and right atrial (RA) dilatation. The anterior valve is rarely displaced, but tends to be large and redundant. Associated tricuspid regurgitation (TR) and atrial shunt are common. […] Fetal echocardiography allows the diagnosis of Ebstein disease and is an aid to planning management of the condition during gestation and after birth. Pulmonary hypoplasia, severe TR, and pericardial effusion are signs of a poor prognosis. […] The fetus was severely affected, with signs of congestive heart failure, severe cardiomegaly (cardiothoracic index [C/T] = 0.61), pericardial effusion, and pulmonary hypoplasia. This type of presentation is associated with elevated intrauterine mortality.

• #44

  https://link.springer.com/article/10.1007/s00246-012-0355-z

  Tricuspid valve malformation is a rare congenital heart disease. Prenatal diagnosis of Ebsteins anomaly (EA) and tricuspid valve dysplasia (TVD) is associated with high mortality. […] The aim of our study was to assess prognostic factors based on our experience. […] Flow through the pulmonary valve on the first echocardiogram is a simple and excellent prognostic factor when major tricuspid valve disease is diagnosed in utero. […] Fetuses should be monitored throughout pregnancy, particularly those with retrograde ductus arteriosus, because several hemodynamic factors may worsen the prognosis.

• #45

  https://journals.lww.com/jiae/fulltext/2019/03020/prenatal_diagnosis_of_ebstein_s_anomaly__an.9.aspx

  Ebstein’s anomaly (EA) is rare but not an uncommon congenital heart disease in the fetus. Fetal echocardiogram has progressed to detect many forms of congenital heart diseases, especially to assess the prognosis of EA based on their anatomy and presentation in utero causing nonimmune hydrops. Fetal echocardiographic findings of cardiomegaly, apical displacement of tricuspid valve with severe regurgitation, can confirm the diagnosis of EA in utero. Severe cardiomegaly and nonimmune hydrops further contribute poor prognosis leading to fetal demise. Identifying fetal risk factors for predicting perinatal mortality is important, including the lack of antegrade flow across the pulmonary valve and retrograde duct flow. Here, we describe fetal echocardiographic findings of a case of EA causing severe cardiomegaly, restricted growth of the lungs, and hydrops in a 26-year-old primigravida at 20 weeks of gestation. The diagnosis was further correlated with the autopsy, characterized by severe cardiomegaly, huge right atrium, apical displacement, and tethering of septal and posterior leaflet, with markedly reduced lung volumes. Autopsy findings of dilated ductus further suggestive of possible reversed ductal flow causing circular shunt in EA raising the possibility of the poor postnatal outcome, even intrauterine death, and warranted for safe termination. Poor prognostic factors in EA have also been discussed with autopsy findings.

• #46 Ebstein Anomaly Differential Diagnoses

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

  Ebstein anomaly should not be confused with Uhl anomaly, a congenital heart disease characterized by a hypoplastic, underdeveloped inflow segment of the right ventricle with tricuspid leaflets arising appropriately from the annulus. […] Rule out Ebstein anomaly in patients with the following: […] Atrial septal defect (ASD) […] Cases of severe right heart failure […] Isolated severe tricuspid regurgitation […] L-transposition of the great vessels.

• #47 📃 Tricuspid valve, Ebstein anomaly

  https://thefetus.net/content/tricuspid-valve-ebstein-anomaly

  Ebstein anomaly is a congenital defect characterized by downward displacement of the septal and posterior leaflets of the tricuspid valve, with dysplasia of this valve. […] The main criterion for the antenatal diagnosis of Ebstein anomaly is the demonstration of downward displacement of the tricuspid value into the right ventricle. […] Doppler color flow mapping reveals precise abnormal intracardiac hemodynamics in various congenital heart diseases which are almost consistent with postnatal Doppler echocardiographic and angiographic findings. […] We conducted color Doppler examination for confirmation of suspected fetal cardiac anomaly, and typical tricuspid regurgitation was identified. Consequently, the antenatal diagnosis of Ebstein anomaly was made. […] The differential diagnosis include tricuspid insufficiency without displacement, Uhl anomaly, and pulmonary atresia with intact ventricular septum and tricuspid insufficiency.

• #48 Ebstein’s anomaly – causes, symptoms, diagnosis and treatment | Longevity

  https://vocal.media/longevity/ebstein-s-anomaly-causes-symptoms-diagnosis-and-treatment

  Radiological signs indicating Ebstein’s anomaly are represented by a sharp increase in the right parts of the heart, a spherical shape of the heart shadow, increased transparency of the pulmonary fields. […] During echocardiography, a downward displacement of the tricuspid valve flaps, an increase in the size of the right atrium, delayed closure of the tricuspid valve, a displacement of the flaps, the presence of an atrialized right ventricle, a shunting blood duct from right to left through ASD (according to Doppler echocardiography) is visible. […] To clarify the form and severity of Ebstein’s anomaly, MRI, probing of the heart cavities, ventriculography are performed. The diagnosis of Ebstein’s anomaly requires differentiation with exudative pericarditis, Abramov-Fiedler myocarditis, isolated ASD and pulmonal stenosis, Fallot’s tetrad.

• #49 Ebstein anomaly | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/ebstein-anomaly?embed_domain=external.radpair.com%2525252527%252525255b0%252525255dfavicon.ico&lang=us

  Ebstein anomaly is an uncommon congenital cardiac anomaly, characterized by a variable developmental anomaly of the tricuspid valve. […] Diagnosis relies on visualization of the septal leaflet of the tricuspid valve, typically appreciated from transthoracic parasternal and apical windows. Apical displacement the septal leaflet in excess of 8 mm per m2 (body surface area) with sail-like elongation is considered diagnostic. […] If you find Ebstein anomaly, also look for other associated defects: right ventricular outflow tract (RVOT) abnormalities, ASD (especially ostium secundum type), VSD, and tetralogy of Fallot. […] In your report, mention the position of tricuspid valve leaflets, assess the degree of regurgitation, and measure right ventricular volume and function. Be sure that you quote „true” RV volume (volume of the ventricular side of the tricuspid valve). […] The differential on a chest radiograph is extremely broad, particularly since the findings in Ebstein anomaly are so variable. With echocardiography and MRI, the diagnosis is usually self-evident, once the apically displaced tricuspid valve in identified.

• #50

  https://www.omim.org/entry/224700

  Of the 32 patients with nonsyndromic Ebstein anomaly, 10 (31%) had additional congenital heart defects (CHDs), including 7 atrial septal defects, 2 ventricular septal defects, 2 pulmonary stenoses, 1 dextrocardia, 1 aortic coarctation, and 1 patent ductus arteriosus. […] Familial recurrence of CHD was seen in 1 family, in which a mother with nonsyndromic Ebstein anomaly had a son with persistence of left ventricular noncompaction. […] In a study of 44 consecutive patients with Ebstein anomaly, Digilio et al. (2011) performed standard chromosome analysis and array CGH in the 12 patients in whom the anomaly was part of a syndrome, and identified chromosomal anomalies in 3 of them. […] In 28 patients with nonsyndromic Ebstein anomaly, Digilio et al. (2011) screened the candidate genes GATA4 and NKX2.5, but did not find any mutations.

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

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

  An absolute distance in atrioventricular valves offsetting of 15mm in children and 20mm in adults is also considered diagnostic. […] Multimodality imaging is pivotal not only for anatomical and functional assessment of TV and right-sided chambers but also for identifying associated lesions. […] Transthoracic echocardiography (TTE) is usually the first diagnostic tool. […] Second-line imaging includes transoesophageal echocardiography (TOE) and cardiovascular magnetic resonance (CMR), the former with particular usefulness in TV assessment, the latter in right-sided chamber volumes calculation and myocardial characterization. […] CMR is superior to TTE in the detection of PL and extra-cardiac abnormalities, while TTE reveals small septal communications more frequently. […] Echocardiography and CMR are both recommended for evaluating unoperated patients, informing on progressive disease and anatomies suitable of repair, as well as for monitoring operated patients, revealing surgical results and possible complications.

• #52

  https://link.springer.com/article/10.1007/s11936-012-0209-2

  Ebstein anomaly (EA) is a rare congenital heart defect that may not be detected until late in adolescence or adulthood. […] Over the past several decades, advances in diagnostic imaging and surgical techniques have contributed to our current management of this challenging congenital heart defect. […] The clinical manifestations of EA in the adult depend on several factors, including the extent of TV leaflet distortion, degree of tricuspid regurgitation (TR), right atrial pressure, and presence of a right-to-left atrial level shunt. […] Advances in diagnostic imaging and surgical techniques have contributed to our current management of this challenging congenital heart defect. […] The right ventricle may be difficult to image by echocardiography and this paper will allow for standardized measurements.

• #53 Antenatal Diagnosis of a Partial Atrioventricular Canal with Ebstein’s Anomaly

  https://www.mdpi.com/2227-9067/8/11/1029

  The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein’s anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. […] This patient’s antenatal diagnosis of both Ebstein’s anomaly and partial AVCD was made at 25 weeks of gestation. […] To our knowledge, we reported the first antenatal documented case of Ebstein’s anomaly with a partial AVCD. […] The antenatal diagnosis allowed us to prevent the occurrence of the complications expected in the immediate postnatal period (i.e., increasing resistance vascular pulmonary and inhibition of pulmonary flow). […] The initial management consisted of inhaled NO and prostaglandins infusion to increase pulmonary blood flow. […] The postnatal management of this case follows the current line reported by several centers with a lot of experience that has improved the survival results. […] We reported the first antenatal documented case of Ebstein anomaly with a partial AVCD. The postnatal evolution was unpredictable, but the antenatal diagnosis and multidisciplinary meetings allowed for the optimization of the management, probably contributing to the good surgical result.

• #54 SciELO Brazil – Ebstein’s anomaly with imperforate tricuspid valve. Prenatal diagnosis Ebstein’s anomaly with imperforate tricuspid valve. Prenatal diagnosis

  https://www.scielo.br/j/abc/a/H6vgJFLcF7LDVVmKtg9xcjy/?lang=en

  This report describes for the first time the diagnosis of Ebstein’s anomaly with imperforation of the tricuspid valve in a fetus documented with fetal echocardiography. […] After birth, sequential echocardiograms confirmed the presence of Ebstein’s anomaly with imperforation of the tricuspid valve. […] At the present time, no reports of the diagnosis of Ebstein’s anomaly with imperforate valve in the prenatal period using fetal echocardiography have been found. […] The case here reported focuses not only on the characteristic image of dysplasia and caudal displacement of the tricuspid valve, but also on the absence of right atrioventricular flow, in spite of the movement of the leaflets, thus documenting the imperforate valve. […] Prenatal diagnosis of this anomaly was decisive in the management of the patient, as its birth could be planned to take place in an adequately prepared, environment from a cardiological point of view. […] This report demonstrates the potential of fetal echocardiography in detection of rare defects, with an important impact on perinatal cardiologic management.

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

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

  Over time, various classifications of EA severity had been proposed. […] Imaging parameters predictive of adverse outcome in EA are reported in online supplemental tables 2 and 3. […] A clinical follow-up is recommended at least yearly in patients with EA, although the monitoring strategy is tailored according to patients specific features. […] EA diagnosis or diagnostic suspect should guide patients referral to a specialised multidisciplinary team. EA spectrum of heterogeneous anatomical and clinical features requires a patient-tailored monitoring strategy.

• #56 Ebstein’s Anomaly | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/ebsteins-anomaly

  Ebstein’s anomaly is a malformed heart valve between the right upper and lower heart chambers that does not properly close to keep the blood flow moving in the right direction. Blood may leak back from the lower to upper chambers on the right side of the heart. […] Children with Ebstein’s anomaly should receive continued care from a pediatric cardiologist (and an adult cardiologist as they move into adulthood). Besides getting information from routine exams, the cardiologist may use tests such as electrocardiograms (EKG), chest X-ray, MRI of the heart and echocardiograms.

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