Materiały źródłowe

• #1 Double outlet right ventricle : MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/007328.htm

  Treatment requires surgery to close the hole in the heart and direct blood from the left ventricle into the aorta. Surgery may also be needed to move the pulmonary artery or aorta. […] Factors that determine the type and number of operations your baby needs include: The type of DORV, The severity of the defect, The presence of other problems in the heart, Your child’s overall condition.

• #2 Double Outlet Right Ventricle (DORV)

  https://my.clevelandclinic.org/health/diseases/14733-double-outlet-right-ventricle

  Double outlet right ventricle (DORV) describes a heart with two major arteries linking to its right ventricle (heart chamber). Surgery repairs the problem, but children born with DORV need lifelong follow-up care. […] Almost all babies with DORV need open-heart surgery within their first year of life. Your healthcare provider will help you make decisions about surgery by considering: Other issues with your baby’s heart or other organs. Their overall health. The type of DORV they have. […] Your baby’s surgeon may take one of the following approaches to double outlet right ventricle repair: Intraventricular repair: The surgeon creates a tunnel through the VSD to connect the aorta to the left ventricle. Biventricular repair or arterial switch: If both ventricles are in good shape and the DORV has a subpulmonary VSD (Taussig-Bing heart), the surgeon may recommend moving the aorta to the left ventricle. Univentricular repair: For more complex cases of DORV, the surgeon may recommend a Fontan operation. It redirects blood flow from your child’s lower body to their lungs.

• #3 Double Outlet Right Ventricle Surgery Treatment & Management: Approach Considerations, Medical Therapy, Surgical Indications and Contraindications

  https://emedicine.medscape.com/article/904397-treatment

  Medical management in the treatment of double outlet right ventricle (DORV) is based on the combination of anatomic lesions and the underlying physiology. A spectrum of presentations is possible with double outlet right ventricle, ranging from inadequate pulmonary blood flow with associated cyanosis to excessive pulmonary blood flow with congestive heart failure. However, double outlet right ventricle is a disorder that cannot spontaneously resolve, and the diagnosis alone is a sufficient indication for surgery. […] When double outlet right ventricle repair (DORV) is planned, several anatomic and physiologic factors are reviewed. The location of the ventriculoseptal defect (VSD) and its size are critical to the repair. […] Double outlet right ventricle (DORV) is a disorder that cannot spontaneously resolve, therefore the diagnosis alone is a sufficient indication for surgery. In general, palliative operations are performed only in patients who require short-term treatment, whereas noncardiac disease is managed (eg, sepsis) when anatomic features do not allow for definitive correction.

• #4 Double Outlet Right Ventricle (DORV) (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/dorv.html

  Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery. […] The main types of surgery used are intraventricular repair, arterial switch, and single ventricle pathway. More than one surgery might be needed to get the heart working as well as possible. […] This surgery uses a patch to make a tunnel from the VSD to the aorta. Then, when the heart contracts, the oxygen-rich blood in the left ventricle flows through the VSD into the aorta. After the repair, there is no longer mixing of oxygen-rich and oxygen-poor blood in the right ventricle. […] The arterial switch repair moves the aorta from the right ventricle to the left ventricle. The VSD is closed to keep oxygen-rich and oxygen-poor blood from mixing in the ventricles. […] In some cases many surgical steps (called the single ventricle pathway) are needed to balance blood flow in a baby with DORV. […] In very rare cases, surgical repair isn’t possible and a heart transplant may be recommended.

• #5 Double outlet right ventricle : MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/007328.htm

  Treatment requires surgery to close the hole in the heart and direct blood from the left ventricle into the aorta. Surgery may also be needed to move the pulmonary artery or aorta. […] Factors that determine the type and number of operations your baby needs include: The type of DORV, The severity of the defect, The presence of other problems in the heart, Your child’s overall condition.

• #6 Double Outlet Right Ventricle Surgery Treatment & Management: Approach Considerations, Medical Therapy, Surgical Indications and Contraindications

  https://emedicine.medscape.com/article/904397-treatment

  Medical management in the treatment of double outlet right ventricle (DORV) is based on the combination of anatomic lesions and the underlying physiology. A spectrum of presentations is possible with double outlet right ventricle, ranging from inadequate pulmonary blood flow with associated cyanosis to excessive pulmonary blood flow with congestive heart failure. However, double outlet right ventricle is a disorder that cannot spontaneously resolve, and the diagnosis alone is a sufficient indication for surgery. […] When double outlet right ventricle repair (DORV) is planned, several anatomic and physiologic factors are reviewed. The location of the ventriculoseptal defect (VSD) and its size are critical to the repair. […] Double outlet right ventricle (DORV) is a disorder that cannot spontaneously resolve, therefore the diagnosis alone is a sufficient indication for surgery. In general, palliative operations are performed only in patients who require short-term treatment, whereas noncardiac disease is managed (eg, sepsis) when anatomic features do not allow for definitive correction.

• #7 Double-outlet right ventricle | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/double-outlet-right-ventricle?content_id=CON-20359709

  Most babies with double-outlet right ventricle have heart repair surgery within a few months of age. More than one type of surgery may be done. The type of surgery depends on the specific heart structure. […] The heart surgeon may do one or more of the following to treat double-outlet right ventricle: […] Create a pathway in the heart to connect the lower left heart chamber to the body’s main artery, called the aorta. […] Use a patch, called a baffle, to fix the hole in the heart. […] Fix the aorta and pulmonary artery positions if they’re reversed. […] Connect the lower right heart chamber to the pulmonary artery, if necessary. […] Widen the natural pathway to the lungs if it’s narrowed. […] Other surgeries might be needed later in life. A person born with double-outlet right ventricle needs regular health checkups for life. Adults should see a doctor trained in evaluating and treating congenital heart conditions. This type of doctor is called an adult congenital cardiologist.

• #8 Double Outlet Right Ventricle (DORV) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/dorv

  Double outlet right ventricle (DORV) is a rare congenital heart defect, meaning its a condition a baby is born with. […] Although DORV is a serious condition, it is treatable with surgery. […] Children with double outlet right ventricle (DORV) will need surgery to close the hole in the heart and connect the aorta to the left ventricle and the pulmonary artery to the right ventricle. […] The most common surgical procedure for DORV with a ventricular septal defect (VSD) is called intraventricular tunnel repair. […] Another procedure, called an arterial switch operation, is necessary if the aorta and the pulmonary artery are reversed in relation to each other. […] If your child has other heart conditions associated with DORV, he or she may also need other types of surgery. […] The success rates of the operations used to repair most forms of DORV are in the same range.

• #9 Double Outlet Right Ventricle (DORV) (for Parents) | Nemours KidsHealth

  https://kidshealth.org/en/parents/dorv.html

  Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery. […] The main types of surgery used are intraventricular repair, arterial switch, and single ventricle pathway. More than one surgery might be needed to get the heart working as well as possible. […] This surgery uses a patch to make a tunnel from the VSD to the aorta. Then, when the heart contracts, the oxygen-rich blood in the left ventricle flows through the VSD into the aorta. After the repair, there is no longer mixing of oxygen-rich and oxygen-poor blood in the right ventricle. […] The arterial switch repair moves the aorta from the right ventricle to the left ventricle. The VSD is closed to keep oxygen-rich and oxygen-poor blood from mixing in the ventricles. […] In some cases many surgical steps (called the single ventricle pathway) are needed to balance blood flow in a baby with DORV. […] In very rare cases, surgical repair isn’t possible and a heart transplant may be recommended.

• #10 Double Outlet Right Ventricle Surgery Treatment & Management: Approach Considerations, Medical Therapy, Surgical Indications and Contraindications

  https://emedicine.medscape.com/article/904397-treatment

  The staged, palliative bidirectional Glenn procedure has been used for patients with univentricular hearts or complex congenital heat disease, including double outlet right ventricle. […] Absolute contraindications of double outlet right ventricle biventricular repair include significant left ventricular hypoplasia, and major overriding or straddling of atrioventricular valve. In those patients who are unsuitable for biventricular repair, single ventricle palliation would be indicated. […] Repair of double outlet right ventricle with a subaortic VSD is accomplished by creating an intraventricular tunnel that channels left ventricular blood through the VSD to the aorta. […] The preferred surgical repair of double outlet right ventricle with subpulmonary VSD (ie, the Taussig-Bing heart) is anatomic repair (ie, the arterial switch operation).

• #11 Double Outlet Right Ventricle (DORV) | Rady Children’s Hospital

  https://www.rchsd.org/health-article/double-outlet-right-ventricle-dorv/

  Double outlet right ventricle (DORV) is a heart defect where the aorta connects to the heart in the wrong place. DORV is a congenital heart defect, which means a baby who has it is born with it. […] Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery. […] The main types of surgery used are intraventricular repair, arterial switch, and single ventricle pathway. More than one surgery might be needed to get the heart working as well as possible. […] This surgery uses a patch to make a tunnel from the VSD to the aorta. Then, when the heart contracts, the oxygen-rich blood in the left ventricle flows through the VSD into the aorta. After the repair, there is no longer mixing of oxygen-rich and oxygen-poor blood in the right ventricle.

• #12 Double Outlet Right Ventricle Surgery Treatment & Management: Approach Considerations, Medical Therapy, Surgical Indications and Contraindications

  https://emedicine.medscape.com/article/904397-treatment

  The staged, palliative bidirectional Glenn procedure has been used for patients with univentricular hearts or complex congenital heat disease, including double outlet right ventricle. […] Absolute contraindications of double outlet right ventricle biventricular repair include significant left ventricular hypoplasia, and major overriding or straddling of atrioventricular valve. In those patients who are unsuitable for biventricular repair, single ventricle palliation would be indicated. […] Repair of double outlet right ventricle with a subaortic VSD is accomplished by creating an intraventricular tunnel that channels left ventricular blood through the VSD to the aorta. […] The preferred surgical repair of double outlet right ventricle with subpulmonary VSD (ie, the Taussig-Bing heart) is anatomic repair (ie, the arterial switch operation).

• #13 Partners in Care | Double Outlet Right Ventricle treatment at the…

  https://partnersincare.health/conditions/double-outlet-right-ventricle

  While medications can be used to help children, who have too much blood going to their lungs, feel better and grow larger in size, surgery is needed to repair the defect and connect the great arteries with their correct pumping chambers. […] Treatment options for DORV may include: […] Arterial Switch: A type of surgery in which the position of the arteries are reversed so that the pulmonary artery is connected to the right ventricle and the aorta is connected to the left ventricle. If the child also has a ventricular septal defect, this is typically closed by placing a patch over the hole during the same operation. […] Intraventricular Repair: A type of surgery in which a tunnel (baffle) is created through the ventricular septal defect to connect the aorta to the left ventricle.

• #14 Double Outlet Right Ventricle | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/double-outlet-right-ventricle

  DORV requires open heart surgery. Medication may also be necessary early on to help the heart function better before and after surgical intervention. […] Surgeons have a variety of methods to repair this heart defect, depending on the anatomy (structure) of your child’s heart. Sometimes a patch made of a synthetic fiber is used to create a tunnel (called a baffle) through the VSD (the hole between the right and left ventricles). The baffle connects to the aorta. This means all of the oxygenated blood from the left ventricle is channeled through the right ventricle into the aorta, without mixing with the deoxygenated blood in the right ventricle. […] In other cases, surgeons reverse the double outlet right ventricle by detaching the aorta from the right ventricle and reattaching it to the left ventricle. They also move the coronary arteries to the new aorta and close the VSD. This surgery is called the arterial switch operation.

• #15 Double Outlet Right Ventricle | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/double-outlet-right-ventricle

  In other cases, the left or right ventricle will be too small or malformed to ever function correctly. Surgeons will reconfigure the heart and circulatory system so that the heart functions with one ventricle (pumping chamber). This type of surgical management requires multiple open-heart surgeries, called staged reconstruction. […] Follow-up care for double outlet right ventricle depends on the type of surgical procedure that was performed, and whether additional surgeries are expected to be needed throughout the child’s life. Regardless, children who were born with double outlet right ventricle must continue to see a pediatric cardiologist regularly.

• #16 Double Outlet Right Ventricle (DORV)

  https://my.clevelandclinic.org/health/diseases/14733-double-outlet-right-ventricle

  Double outlet right ventricle (DORV) describes a heart with two major arteries linking to its right ventricle (heart chamber). Surgery repairs the problem, but children born with DORV need lifelong follow-up care. […] Almost all babies with DORV need open-heart surgery within their first year of life. Your healthcare provider will help you make decisions about surgery by considering: Other issues with your baby’s heart or other organs. Their overall health. The type of DORV they have. […] Your baby’s surgeon may take one of the following approaches to double outlet right ventricle repair: Intraventricular repair: The surgeon creates a tunnel through the VSD to connect the aorta to the left ventricle. Biventricular repair or arterial switch: If both ventricles are in good shape and the DORV has a subpulmonary VSD (Taussig-Bing heart), the surgeon may recommend moving the aorta to the left ventricle. Univentricular repair: For more complex cases of DORV, the surgeon may recommend a Fontan operation. It redirects blood flow from your child’s lower body to their lungs.

• #17 Double Outlet Right Ventricle Treatment: Symptoms & Causes Explained – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/double-outlet-right-ventricle-treatment-symptoms-causes-explained/

  As the primary treatment for DORV, surgery is typically performed during infancy or early childhood. […] Surgical repair offers the best chance for a normal or near-normal life, with most children experiencing significant improvement after recovery. […] The arterial switch operation repositions the aorta and pulmonary artery to their correct locations. […] Successful arterial switch operations restore normal blood circulation and oxygenation, greatly enhancing the child’s quality of life. […] The Rastelli procedure redirects blood flow from the left ventricle to the aorta using a conduit or patch. […] The Rastelli procedure effectively restores proper blood flow, though additional surgeries may be needed as the patient grows. […] The Fontan procedure directs blood flow from the lower body to the lungs without passing through the heart. […] The Fontan procedure improves oxygen levels and reduces symptoms, though lifelong monitoring and care are often required.

• #18 Double-outlet right ventricle | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/double-outlet-right-ventricle?content_id=CON-20359709

  Most babies with double-outlet right ventricle have heart repair surgery within a few months of age. More than one type of surgery may be done. The type of surgery depends on the specific heart structure. […] The heart surgeon may do one or more of the following to treat double-outlet right ventricle: […] Create a pathway in the heart to connect the lower left heart chamber to the body’s main artery, called the aorta. […] Use a patch, called a baffle, to fix the hole in the heart. […] Fix the aorta and pulmonary artery positions if they’re reversed. […] Connect the lower right heart chamber to the pulmonary artery, if necessary. […] Widen the natural pathway to the lungs if it’s narrowed. […] Other surgeries might be needed later in life. A person born with double-outlet right ventricle needs regular health checkups for life. Adults should see a doctor trained in evaluating and treating congenital heart conditions. This type of doctor is called an adult congenital cardiologist.

• #19 Double Outlet Right Ventricle With Transposition Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/896082-treatment

  The two surgical approaches to double outlet right ventricle (DORV) are palliative and corrective. […] Similar to medical management, palliative therapy helps improve the patient’s clinical condition, allowing him or her to gain weight and achieve optimal conditions for definitive surgical repair. […] Patients with subaortic or subpulmonary VSD with PS are cyanotic and have decreased PBF; therefore, they should undergo a systemic-to-PA shunt, usually a modified Blalock-Taussig anastomosis, to increase PBF. […] If biventricular repair is feasible, the 2 basic surgical steps to follow according to certain authors are (1) creation of an intracardiac tunnel to connect the LV to usually the aorta or, less commonly, the main pulmonary artery, where the conal septum is resected and any abnormal AV valve insertion on such conal septum are preserved; and (2) creation of an intracardiac or an extracardiac reconstruction to connect the RV to the main pulmonary artery. […] If the anatomy of associated lesions is too complex to consider an anatomic repair or if a repair results in unsatisfactory hemodynamics and intractable symptoms, consider heart transplantation.

• #20 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  The ventriculotomy should began inferiorly, as the orifice of the aortic valve can always be lower than expected from external inspection. […] Resection of the muscular ventricular septum may be required to create an unobstructed tunnel to the aorta should the interventricular communication be restrictive. […] When the construction of a straight tunnel is complex, with a high chance of residual or recurrent left ventricular outflow tract obstruction, the alternative options of an arterial switch or REV procedure are safer and easier to perform. […] In the recent years, it has been appreciated that it is often possible to achieve en bloc rotation of the outflow tracts. […] The procedure is advantageous in allowing for growth of all tubular structures, and in appropriate cases permitting preservation of the native pulmonary valve.

• #21 Double Outlet Right Ventricle With Transposition Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/896082-treatment

  Medical treatment depends on the clinical presentation, which is determined by the differences in physiology of each type of double outlet right ventricle (DORV). […] In DORV with no pulmonary valve stenosis (PS), medical management to control congestive heart failure (CHF) and improve the patient’s condition prior to surgery should be instituted. Management of CHF requires medications such as loop diuretics (eg, furosemide), potassium-sparing diuretics (eg, spironolactone), after-load reducing agents, and digitalis. […] In neonates with DORV and PS with marked cyanosis and hypoxemia, initial medical management consists of administration of intravenous prostaglandin E1 (PGE1) to open the ductus. […] Maintain patency of the ductus arteriosus with prostaglandin E1 in newborns with markedly diminished pulmonary blood flow (PBF) from severe pulmonary valve stenosis (PS).

• #22 Double Outlet Right Ventricle With Transposition Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/896082-treatment

  Medical treatment depends on the clinical presentation, which is determined by the differences in physiology of each type of double outlet right ventricle (DORV). […] In DORV with no pulmonary valve stenosis (PS), medical management to control congestive heart failure (CHF) and improve the patient’s condition prior to surgery should be instituted. Management of CHF requires medications such as loop diuretics (eg, furosemide), potassium-sparing diuretics (eg, spironolactone), after-load reducing agents, and digitalis. […] In neonates with DORV and PS with marked cyanosis and hypoxemia, initial medical management consists of administration of intravenous prostaglandin E1 (PGE1) to open the ductus. […] Maintain patency of the ductus arteriosus with prostaglandin E1 in newborns with markedly diminished pulmonary blood flow (PBF) from severe pulmonary valve stenosis (PS).

• #23 Double Outlet Right Ventricle – Children’s Health Issues – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/double-outlet-right-ventricle

  Medications to treat cyanosis and heart failure […] Surgery is required to repair the defect. […] During the newborn period, medications called prostaglandins may be needed to keep the ductus arteriosus open if the infant has severe cyanosis. If too much blood flows to the lungs, medications are needed to improve heart function and treat the resulting heart failure.

• #24 Double Outlet Right Ventricle (DORV): Overview, Symptoms and Treatment | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/double-outlet-right-ventricle

  The Heart Center at Nationwide Childrens Hospital has special methods to save (conserve) as much blood as possible during surgery. This can decrease the need for a blood transfusion later. […] After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). They may be on a breathing machine and have many tubes, wires, and equipment to keep them stable and make sure they are okay. […] Other treatments your baby will probably get after surgery are: Antibiotic medicine to prevent infection, Fluids and nutrition through an IV or PICC line, Heart medicines by IV or by mouth, Chest drainage tube, Oxygen. […] Your medical team will help you decide on the safest feeding approach. […] Your cardiologist will follow up with your babys heart needs even after they are discharged from the hospital.

• #25 Double Outlet Right Ventricle

  https://healthlibrary.wjmc.org/library/diseasesconditions/adult/Infectious/3,90590

  Some children with DORV also need medicine. The kind of medicine depends on the type of DORV and how serious the symptoms are. Medicine may include: Water pills (diuretics) to reduce swelling, ACE inhibitors or digoxin to make it easier for the heart to pump blood to the rest of the body, Beta-blockers to reduce how hard the heart pumps, Blood thinners (anticoagulants) to prevent blood clots, Antibiotics before certain medical and dental procedures to prevent infection. […] Most children with DORV go on to lead normal and active lives. But they will need to see a heart specialist regularly. Some may need more surgery as adults.

• #26

  https://journals.lww.com/cmj/fulltext/2017/02200/surgical_treatment_of_double_outlet_right.6.aspx

  Overall, infants and children in this study had a significant reduction in PH after surgery. However, the decline was not significant for a few infants 1 year old, which may be related to hypoplastic development of the pulmonary vascular bed. […] The postoperative management of these patients is very important. Routine use of inhaled nitric oxide and prostaglandins is helpful. Diuretic drugs and other supportive agents are essential.

• #27 Double Outlet Right Ventricle (DORV)

  https://my.clevelandclinic.org/health/diseases/14733-double-outlet-right-ventricle

  Most babies have good outcomes from surgery for DORV. But any heart surgery has risks, such as: Bleeding. Blood clots, which can cause a stroke or heart attack. Infection. Problems coming out of anesthesia. Respiratory failure. Unusual heart rhythms and heart block. […] With surgery, most babies who have double outlet right ventricle live to be adults. Anyone who’s had surgery for DORV needs lifelong care from a cardiologist or provider who specializes in taking care of your heart.

• #28 Double Outlet Right Ventricle Surgery Treatment & Management: Approach Considerations, Medical Therapy, Surgical Indications and Contraindications

  https://emedicine.medscape.com/article/904397-treatment

  Surgical correction of double outlet right ventricle with a doubly committed VSD (an uncommon variant of this disorder) is performed in a fashion similar to that described above for double outlet right ventricle with subaortic VSD. […] Of the types of double outlet right ventricle, the most difficult to correct is the defect that requires repair of the noncommitted VSD. […] Two groups of investigators reported a mortality rate of approximately 10% for double outlet right ventricle noncommitted VSD, but the incidence of late subaortic stenosis is reasonably high. […] In a large series analyzing outcomes in patients with double outlet right ventricle (DORV) from 1980 to 2000, Brown et al reported a 56% 15-year overall survival (including the patients who underwent no surgical intervention). […] Three-dimensional printed heart models are potentially useful in congenital heart surgery, In particular for demonstrating the association between intraventricular communications and great vessels, and in simulation for creating intracardiac pathways.

• #29 Double outlet right ventricle: opinions regarding management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16138957/

  In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the „Taussig-Bing anomaly” (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventricular valve abnormalities, or unbalanced ventricles, a staged palliative approach is warranted. This approach will result in the patient having „single ventricle” physiology, and „Fontan anatomy” after the staging surgeries. Outcomes are determined by the surgery performed. Patients who require conduit placement from right ventricle to pulmonary artery will need subsequent surgery for replacement of the conduit. Complex intraventricular tunnels are at risk for developing subaortic obstruction. Patients who had arterial switch operations have thus far had excellent results. However, these patients may be at risk for development of neoaortic valve regurgitation requiring subsequent reoperation. Complex biventricular intracardiac repairs have been associated with a higher risk of reoperation. In the past 10 to 15 years, patients undergoing Fontan operations have had improved early and late survival. However, long-term problems after the Fontan operation include arrhythmia and development of protein-losing enteropathy.

• #30 Double outlet right ventricle: opinions regarding management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16138957/

  In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the „Taussig-Bing anomaly” (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventricular valve abnormalities, or unbalanced ventricles, a staged palliative approach is warranted. This approach will result in the patient having „single ventricle” physiology, and „Fontan anatomy” after the staging surgeries. Outcomes are determined by the surgery performed. Patients who require conduit placement from right ventricle to pulmonary artery will need subsequent surgery for replacement of the conduit. Complex intraventricular tunnels are at risk for developing subaortic obstruction. Patients who had arterial switch operations have thus far had excellent results. However, these patients may be at risk for development of neoaortic valve regurgitation requiring subsequent reoperation. Complex biventricular intracardiac repairs have been associated with a higher risk of reoperation. In the past 10 to 15 years, patients undergoing Fontan operations have had improved early and late survival. However, long-term problems after the Fontan operation include arrhythmia and development of protein-losing enteropathy.

• #31 Double outlet right ventricle: opinions regarding management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/16138957/

  In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the „Taussig-Bing anomaly” (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventricular valve abnormalities, or unbalanced ventricles, a staged palliative approach is warranted. This approach will result in the patient having „single ventricle” physiology, and „Fontan anatomy” after the staging surgeries. Outcomes are determined by the surgery performed. Patients who require conduit placement from right ventricle to pulmonary artery will need subsequent surgery for replacement of the conduit. Complex intraventricular tunnels are at risk for developing subaortic obstruction. Patients who had arterial switch operations have thus far had excellent results. However, these patients may be at risk for development of neoaortic valve regurgitation requiring subsequent reoperation. Complex biventricular intracardiac repairs have been associated with a higher risk of reoperation. In the past 10 to 15 years, patients undergoing Fontan operations have had improved early and late survival. However, long-term problems after the Fontan operation include arrhythmia and development of protein-losing enteropathy.

• #32 Double-outlet right ventricle | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/double-outlet-right-ventricle?content_id=CON-20359709

  Most babies with double-outlet right ventricle have heart repair surgery within a few months of age. More than one type of surgery may be done. The type of surgery depends on the specific heart structure. […] The heart surgeon may do one or more of the following to treat double-outlet right ventricle: […] Create a pathway in the heart to connect the lower left heart chamber to the body’s main artery, called the aorta. […] Use a patch, called a baffle, to fix the hole in the heart. […] Fix the aorta and pulmonary artery positions if they’re reversed. […] Connect the lower right heart chamber to the pulmonary artery, if necessary. […] Widen the natural pathway to the lungs if it’s narrowed. […] Other surgeries might be needed later in life. A person born with double-outlet right ventricle needs regular health checkups for life. Adults should see a doctor trained in evaluating and treating congenital heart conditions. This type of doctor is called an adult congenital cardiologist.

• #33 Double outlet right ventricle (DORV) | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/double-outlet-right-ventricle/

  Double outlet right ventricle (DORV) is a congenital heart defect that causes the aorta to be connected to the heart in the wrong place. […] Surgery is needed to correct DORV. Medicines might help the heart work better, but a baby with DORV cannot get better for long without surgery. […] DORV can be corrected in a single-ventricle palliation approach, likely with multiple surgeries, or a complete two-ventricle repair. The decision on which repair is best will depend on the size of the ventricles and other heart defects that are present. […] Infants who have a repaired DORV will need to see a pediatric cardiologist throughout childhood, adolescence and adulthood. When the child becomes an adult, an adult congenital heart defect specialist can continue to provide care.

• #34 Double Outlet Right Ventricle

  https://healthlibrary.wjmc.org/library/diseasesconditions/adult/Infectious/3,90590

  Some children with DORV also need medicine. The kind of medicine depends on the type of DORV and how serious the symptoms are. Medicine may include: Water pills (diuretics) to reduce swelling, ACE inhibitors or digoxin to make it easier for the heart to pump blood to the rest of the body, Beta-blockers to reduce how hard the heart pumps, Blood thinners (anticoagulants) to prevent blood clots, Antibiotics before certain medical and dental procedures to prevent infection. […] Most children with DORV go on to lead normal and active lives. But they will need to see a heart specialist regularly. Some may need more surgery as adults.

• #35 Double Outlet Right Ventricle Surgery Treatment & Management: Approach Considerations, Medical Therapy, Surgical Indications and Contraindications

  https://emedicine.medscape.com/article/904397-treatment

  Surgical correction of double outlet right ventricle with a doubly committed VSD (an uncommon variant of this disorder) is performed in a fashion similar to that described above for double outlet right ventricle with subaortic VSD. […] Of the types of double outlet right ventricle, the most difficult to correct is the defect that requires repair of the noncommitted VSD. […] Two groups of investigators reported a mortality rate of approximately 10% for double outlet right ventricle noncommitted VSD, but the incidence of late subaortic stenosis is reasonably high. […] In a large series analyzing outcomes in patients with double outlet right ventricle (DORV) from 1980 to 2000, Brown et al reported a 56% 15-year overall survival (including the patients who underwent no surgical intervention). […] Three-dimensional printed heart models are potentially useful in congenital heart surgery, In particular for demonstrating the association between intraventricular communications and great vessels, and in simulation for creating intracardiac pathways.

• #36

  http://www.nni.com.sg/news/patient-care/hybrid-cardiac-management-for-a-child-with-rare-dorv-and-ventricular-inversion

  These advances have propelled a joint venture of care involving cardiac surgeons and interventional cardiologists collaborating in the same setting termed hybrid procedure to harmoniously amalgamate invasive and less invasive treatment modalities to minimise risks and optimise treatment outcomes for patients. […] A hybrid cardiac procedure is defined as a treatment strategy that concomitantly adopts surgical and interventional approaches, and is done concurrently or sequentially within 24 hours. Less invasive hybrid procedures potentially allow for a reduction in cardiopulmonary bypass time and overall risks, and improved treatment outcomes for patients. […] Despite a paucity of data addressing hybrid procedures in the context of DORV in current literature, this demonstrates a first reported case of a paediatric patient in Singapore with DORV using a hybrid treatment modality, with a good treatment outcome thus far.

• #37 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  The individualized surgical approach in individuals with both arterial trunks arising from the morphologically right ventricle is dictated by the extreme morphological variability encountered in this setting, with each patient being unique. […] An individualized surgical approach has been designed to take account of the morphological variations, identifying the anatomy with the preoperative three-dimensional CT scan reconstruction. […] The surgical approach is tailored, whenever feasible, to create a connection between left ventricle and aorta, but primarily to achieve biventricular repair. […] Our approach, being tailored to the individual cardiac morphology, can be offered to any patient with this ventriculo-arterial connection. […] Given the difficulties of diagnosis, and the multiple therapeutic indications, very close collaboration between cardiologists and surgeons is indispensable for further progress in the understanding and management of this complex congenital cardiac lesion.

• #38 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  The optimal surgical approach, of course, is to connect the left ventricle in unobstructed fashion to the aorta, leaving the right ventricle in free communication with the pulmonary trunk, without needing to interpose a prosthetic conduit. […] The critical point is to understand whether the right ventricle remains in free communication with the pulmonary trunk subsequent to construction of the interventricular tunnel. […] In this setting, the creation of an unrestricted tunnel from the left ventricle to the aorta can compromise the remaining connection between the tricuspid and pulmonary valves. […] The feasibility of an intraventricular repair, in which the left ventricle is connected to the aorta, depends on the distance between the orifice of the tricuspid valve and the orifice of the pulmonary valve.

• #39 Narrative review of assessing the surgical options for double outlet right ventricle – Corno – Translational Pediatrics

  https://tp.amegroups.org/article/view/60794/html

  The individualized surgical approach in individuals with both arterial trunks arising from the morphologically right ventricle is dictated by the extreme morphological variability encountered in this setting, with each patient being unique. […] An individualized surgical approach has been designed to take account of the morphological variations, identifying the anatomy with the preoperative three-dimensional CT scan reconstruction. […] The surgical approach is tailored, whenever feasible, to create a connection between left ventricle and aorta, but primarily to achieve biventricular repair. […] Our approach, being tailored to the individual cardiac morphology, can be offered to any patient with this ventriculo-arterial connection. […] Given the difficulties of diagnosis, and the multiple therapeutic indications, very close collaboration between cardiologists and surgeons is indispensable for further progress in the understanding and management of this complex congenital cardiac lesion.

• #40 Double outlet right ventricle

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10560996/

  The clinical outcomes in patients with DORV have improved significantly over time with the degree of success depending on the complexity of the underlying lesions and the institutions experience. For example, outcomes among patients with DORV-VSD are excellent. […] While any of these repairs can result in good outcomes, patient selection and determining the best operation for the patho-anatomy and the patients condition are essential.

• #41 Double outlet right ventricle

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10560996/

  The clinical outcomes in patients with DORV have improved significantly over time with the degree of success depending on the complexity of the underlying lesions and the institutions experience. For example, outcomes among patients with DORV-VSD are excellent. […] While any of these repairs can result in good outcomes, patient selection and determining the best operation for the patho-anatomy and the patients condition are essential.

• #42 Double Outlet Right Ventricle (DORV) | Rady Children’s Hospital

  https://www.rchsd.org/health-article/double-outlet-right-ventricle-dorv/

  The arterial switch repair moves the aorta from the right ventricle to the left ventricle. The VSD is closed to keep oxygen-rich and oxygen-poor blood from mixing in the ventricles. […] In some cases many surgical steps (called the single ventricle pathway) are needed to balance blood flow in a baby with DORV. […] In very rare cases, surgical repair isn’t possible and a heart transplant may be recommended.

• #43 Double-Outlet Right Ventricle – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/double-outlet-right-ventricle/

  All children with double-outlet right ventricle will need lifelong follow-up with a cardiologist who specializes in congenital heart defects. […] In rare cases, the heart cannot be repaired, and doctors may recommend a heart transplant. The heart transplant team at Seattle Children’s is one of the best in the nation, caring for children with this or other heart problems that cannot be controlled using other treatments.

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