Materiały źródłowe

• #1 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart anomaly characterized by the underdevelopment or complete obstruction of the pulmonary valve, preventing blood flow from the right ventricle to the lungs. […] Surgical intervention, often in multiple stages, is required to establish pulmonary blood flow and improve long-term survival. […] This course provides healthcare professionals with the knowledge to diagnose and manage pulmonary atresia with ventricular septal defect accurately. […] The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care. […] Oxygen saturation is critical and should be monitored continuously.

• #2 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: […] Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs. […] This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt.

• #3 Pulmonary atresia with ventricular septal defect | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-ventricular-septal-defect?content_id=CON-20380877

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: Catheter procedure. Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs.

• #4 Mayo Clinic Health Library – Pulmonary atresia with ventricular septal defect | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20380877

  A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: […] Catheter procedure. Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs.

• #5 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Pulmonary atresia treatment depends on how the condition affects your child and on their: […] Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs. […] Temporary treatments include medication and balloon atrial septostomy. Most children will probably need more surgery to improve blood flow to their lungs. […] Medication. Newborns with pulmonary atresia usually receive an IV drug (injected into a vein) called alprostadil. This drug prevents the ductus arteriosus (which connects the aorta to the pulmonary artery) from starting to close as it normally would within a few hours to days after birth. By keeping this open, oxygen-poor blood can flow from your baby’s aorta to their pulmonary arteries. Then it goes to their lungs to pick up oxygen.

• #6 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  In severe cases where the pulmonary valve is completely atretic, and the pulmonary circulation is entirely dependent on ductus arteriosus, prostaglandin E1 should be considered to keep the duct open until a surgical correction occurs. […] Conventional treatment, occurring in about 75% of cases, involves discharge of the neonates from the hospital to return at 3 to 6 months of age when their organs are more developed. […] The size of the pulmonary arteries, the presence/absence of MAPCAs, and PDA drive the surgical plan. […] The Nakata index (NI) is an angiographic technique used to standardize the cross-sectional area of the bilateral pulmonary arteries quantitatively. […] Early total correction […] In this less popular approach, which is preferred mostly in mild cases, surgeons usually correct the anatomy within the same admission if not the same operation. […] In patients with a completely atretic pulmonary system or who have failed the surgical corrective measures, a cardiac transplant can be a viable option.

• #7 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa

  Specific treatment for pulmonary atresia will be determined by your child’s doctor based on: […] After birth, it is important to make sure the ductus arteriosus stays open to allow blood to get to the lungs to pick up oxygen. An IV medicine called prostaglandin (PGE-1) is used to keep the ductus arteriosus open. […] Many babies will then undergo a procedure to provide more stable blood flow to the lungs and allow them to grow before they undergo further surgeries. This procedure can be done either with surgery or with a catheter-based procedure. […] Some patients, particularly those who had a pulmonary valve that did not form normally but do not have a lot of muscle under the valve, may be eligible for another procedure. […] After this initial procedure, the next steps are determined by the size of the right side of the heart, including the tricuspid valve between the atrium (collecting chamber) and the ventricle, the right ventricle itself, and any other associated abnormalities.

• #8 Pulmonary atresia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia/diagnosis-treatment/drc-20350733

  Babies need emergency medical care for pulmonary atresia symptoms. The choice of surgeries or procedures depends on how severe the condition is. […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a long-term treatment for pulmonary atresia. But it gives healthcare professionals more time to decide what type of surgery or procedure might be best. […] Sometimes, pulmonary atresia treatment can be done using a long, thin tube called a catheter. A doctor places the tube into a large blood vessel in a baby’s groin and guides it to the heart. Catheter-based procedures for pulmonary atresia include: […] Babies with pulmonary atresia often need many heart surgeries over time. The type of heart surgery depends on the size of the child’s lower right heart chamber and pulmonary artery. […] If the baby also has a ventricular septal defect (VSD), surgery is done to patch the hole. Then the surgeon makes a connection from the right pumping chamber to the pulmonary artery. This repair may use an artificial valve.

• #9 Pulmonary Atresia Surgery | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/treatments/surgical/pulmonary-atresia-treatment/

  Pulmonary atresia is a very rare birth defect affecting the pulmonary valve. Sometimes the pulmonary valve is missing completely; other times the valve is blocked. Normally, the pulmonary valve acts like a door that allows blood to flow from the right ventricle through the pulmonary artery to the lungs to pick up oxygen. Pulmonary atresia surgery will be needed to repair the condition. […] Initial treatment of pulmonary atresia focuses on keeping the ductus arteriosus open with a medication called prostaglandins. The ductus arteriosus, a connection between the pulmonary artery and aorta, normally closes soon after birth. Keeping it open allows blood to keep blood flowing to the lungs. If the baby does not have a ventricular septal defect (VSD) or an atrial septal defect (ASD), an emergency balloon atrial septostomy will be performed. This will allow blood on the right side of the heart to get to the left side of the heart so it can be pumped out to the body.

• #10 Pulmonary Atresia (PA) – Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/pulmonary-atresia-pa/

  Pulmonary atresia (PA) is a heart defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy. […] Specific treatment for pulmonary atresia will be determined by your child’s physician. Children with pulmonary atresia will most likely be admitted to the cardiovascular intensive care unit (CVICU) or neonatal intensive care unit (NICU) once symptoms are noted. Initially, the child may be placed on oxygen, and possibly even on a ventilator, to assist his or her breathing. IV medications may be given to help the heart and lungs function more efficiently. […] Ultimately, surgery is necessary to improve blood flow to the lungs on a permanent basis. The exact type of surgery needed depends on a number of factors, including the size of the right ventricle and whether there is a communication between the right and left ventricles (a ventricular septal defect or VSD). Your child’s cardiologist, heart surgeon and intensive care doctor will carefully review your child’s unique situation and explain their recommendations for treatment.

• #11 Pulmonary Atresia | Valley Children’s Healthcare

  https://www.valleychildrens.org/services/heart/conditions-we-treat/pulmonary-atresia

  Pulmonary atresia (PA) is a heart defect. It happens when the baby’s heart doesnt form as it should in the uterus. This can happen during the first 8 weeks of pregnancy. […] A pediatric cardiologist will treat your baby. This healthcare provider has special training to treat heart problems in children. Your baby will most likely be in the intensive care unit (ICU). At first, your baby may be put on oxygen, and possibly on a machine (ventilator), to help with breathing. Your child may get IV medicines to help their heart and lungs work better. […] The below treatments allow time for the oxygen levels in your baby’s heart to even out while other repairs are planned: An IV medicine called prostaglandin E1 is often given to keep the ductus arteriosus from closing. This lets blood get to the lungs for oxygen. A cardiac catheterization may be done to diagnose the defect. In some cases, the pulmonary valve may be able to be opened with catheterization to prevent the need for open heart surgery. This is only an option if the tricuspid valve and the right ventricle are normal size. A procedure can also be done to open or keep open the ductus arteriosus.

• #12 Pulmonary Atresia | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia

  If your baby is diagnosed with pulmonary atresia before birth, the Fetal Heart Program at Children’s Hospital can prepare a plan for delivery and care immediately after birth. Your baby will be admitted to our Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU) and may require a ventilator for help breathing. In the CICU, your baby will receive round-the-clock attention from a team of dedicated cardiac critical care medicine specialists. […] The exact treatment for pulmonary atresia depends on each child’s heart anatomy. Many cases can be treated with a cardiac catheterization technique called balloon valvuloplasty. In this procedure, doctors advance a thin tube (catheter) to the heart through a vein in the leg. A special catheter is used to create a hole in the closed-off pulmonary valve. Then, another catheter, which has a balloon on the end of it, is advanced across the valve. The balloon is briefly inflated to open up the valve, and then deflated and withdrawn.

• #13 Pulmonary Atresia – Seattle Children’s

  https://www.seattlechildrens.org/conditions/pulmonary-atresia/

  Your childs doctor will suggest some procedures and treatments right away to improve your babys blood flow. Other procedures may be done when your baby gets older, such as open-heart surgery to repair or replace the valve. Most babies can be helped with surgery. […] Your doctor may give your baby medicine (prostaglandin) to keep the ductus arteriosus from closing. This gives blood a way to get to your babys lung arteries when the normal path between the heart and lungs is closed. If your baby is diagnosed before birth, the delivery team will plan to start this medicine soon after birth. […] Your baby may need cardiac catheterization to enlarge the opening between their atria (foramen ovale). Doctors use a balloon to stretch open the narrow pulmonary valve (balloon valvuloplasty). This process may also be used to place a mesh-like tube, called a stent, in the ductus arteriosus to keep it open and let blood flow from the aorta into the lung arteries.

• #14 Pulmonary atresia with ventricular septal defect | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-ventricular-septal-defect?content_id=CON-20380877

  Pulmonary atresia with ventricular septal defect is life-threatening. A baby with pulmonary atresia eventually doesn’t get enough oxygen. One or more procedures or surgeries are needed to fix the heart. […] A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: Catheter procedure. Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs.

• #15 Mayo Clinic Health Library – Pulmonary atresia with ventricular septal defect | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20380877

  A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: […] Catheter procedure. Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs.

• #16 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: […] Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs. […] This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt.

• #17 Pulmonary Atresia with Ventricular Septal Defect | Children’s Mercy Kansas City

  https://www.childrensmercy.org/departments-and-clinics/heart-center/pulmonary-atresia-with-ventricular-septal-defect/

  For babies with pulmonary atresia, a medication called prostaglandin will be infused through an IV to keep the PDA open to provide pulmonary blood flow. […] Treatment options for PA-VSD include catheter based interventions and surgical interventions. […] During a cardiac catheterization, doctors can place a stent in the PDA so that blood can continue to flow to the lungs without the prostaglandins. […] A surgical shunt can be placed to connect the pulmonary artery and aorta to act as a PDA. […] Complete repair includes closure of the ventricular septal defect and placement of a valved conduit (tube) that connects the right ventricle to the pulmonary arteries for pulmonary blood flow.

• #18 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Balloon atrial septostomy. Your child’s healthcare provider can use cardiac catheterization to enlarge the opening in their septum (the wall between the right atrium and left atrium). This improves blood flow to your baby’s lungs. They insert a catheter into your child’s heart through a blood vessel and guide it through the foramen ovale. They inflate the balloon at the tip of the catheter. They quickly remove the catheter to make an opening in the atrial septum. Oxygen-poor blood can then flow from the right to the left atrium and into the left ventricle and aorta. […] The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including: […] Surgery for pulmonary atresia with a ventricular septal defect. Usually, the right ventricle is well developed and can pump blood to your baby’s lungs. Surgery involves:

• #19 Pulmonary atresia | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia?content_id=CON-20155225

  Pulmonary atresia is a life-threatening condition that needs emergency treatment. Treatment includes surgery to repair the heart and medicines to help the heart work better. […] Babies need emergency medical care for pulmonary atresia symptoms. The choice of surgeries or procedures depends on how severe the condition is. […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a long-term treatment for pulmonary atresia. But it gives healthcare professionals more time to decide what type of surgery or procedure might be best. […] Sometimes, pulmonary atresia treatment can be done using a long, thin tube called a catheter. A doctor places the tube into a large blood vessel in a baby’s groin and guides it to the heart. Catheter-based procedures for pulmonary atresia include: Balloon atrial septostomy. A balloon is used to enlarge the natural hole in the wall between the upper chambers of the heart. This hole, called the foramen ovale, most often closes soon after birth. Making the hole larger lets blood move easily from the right side of the heart to the left side. Stent placement. A doctor may put a rigid tube called a stent in the ductus arteriosus to prevent it from closing. This keeps blood flowing to the lungs.

• #20 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. […] Surgical options for neonates and young infants with PA/VSD have been under debate. Early primary repairs guarantee the restoration of serial circulation, and may prevent progression of right ventricular hypertrophy (RVH). However, early primary repairs present increased risks of cardiopulmonary bypass (CPB) for young and small patients as well as inevitable placement of a small RV-PA conduit, which necessitates early conduit replacement.

• #21 Mayo Clinic Health Library – Pulmonary atresia with ventricular septal defect | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20380877

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt. […] Complete repair. This treatment may be done when as a baby is young or after a shunt or stent is placed. During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery. […] Unifocalization. Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it’s the first step toward an eventual complete repair. […] Babies with pulmonary atresia with ventricular septal defect need regular checkups by a doctor trained in heart conditions present at birth. This type of doctor is called a congenital cardiologist.

• #22 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures. […] A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs. […] Surgeries or other procedures may include: […] Thin, flexible tubes called catheters are used to look at the baby’s heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs. […] This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt.

• #23 Pulmonary atresia with ventricular septal defect | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-ventricular-septal-defect?content_id=CON-20380877

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt. […] Complete repair. This treatment may be done when as a baby is young or after a shunt or stent is placed. During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery. […] Unifocalization. Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it’s the first step toward an eventual complete repair.

• #24 Partners in Care | Pulmonary Atresia With Ventricular Septal Defect…

  https://partnersincare.health/conditions/pulmonary-atresia-with-ventricular-septal-defect

  Treatment options for children with PA with VSA is tailored to their unique anatomy. […] Treatment options for PA with VSA may include: […] Children diagnosed with PA with VSD who do not have MAPCAs will usually undergo surgery as a newborn to ensure they have a reliable means of transporting blood to the lungs to receive oxygen. During this operation, a connection is made, which typically involves sewing a Gore-Tex tube between the right subclavian artery (the first artery that branched off the aorta) and the right pulmonary artery. Some of the blood traveling through the aorta toward the body will „shunt” through this connection and flow into the pulmonary artery to receive oxygen. […] Using the same method as for diagnosis, a small tube can be inserted through a blood vessel in the groin and guided to the heart where a stent can be placed in the ductus arteriosus (a blood vessel that connects the aorta and pulmonary artery). When the child is around 6-12 months of age, they will undergo another operation in which the ventricular septal defect will be closed by placing a patch over the hole and a larger tube (conduit) will be sewn between the right ventricle and the pulmonary artery to provide a stable source of pulmonary blood flow. The tube does not grow and over time stops working properly, which results in the need to be replaced by performing an additional surgery. Once the child is adult-sized, they may be able to undergo procedures in the cardiac catheterization lab to help restore the function of the conduit.

• #25 Conditions – Leeds Congenital Hearts

  https://leedscongenitalhearts.com/child/conditions/view/2/51/pulmonary-atresia-with-ventricular-septal-defect

  This is a serious heart condition in which there are two main abnormalities: the artery which normally allows blood to be pumped from the heart to the lungs (the pulmonary artery) is completely blocked […] Most babies with pulmonary atresia will need an operation within the first few weeks of life to make sure they can feed their lungs with blood. This operation involves connecting the artery leading to one of the arms to the artery which leads to the lungs, and is called a shunt operation. This doesnt make the babys heart normal, but it usually allows the baby to survive until he or she is old enough to consider more major surgery. […] All heart operations in babies are serious and will carry some risk of the baby dying or having major complications such as brain damage, kidney damage or lung complications such as pneumonia. For shunt operations the risk of a baby dying is small (about 2 in 100) and the risk of brain damage is also small (probably around 1 in 100).

• #26 Conditions – Leeds Congenital Hearts

  https://leedscongenitalhearts.com/child/conditions/view/2/51/pulmonary-atresia-with-ventricular-septal-defect

  This is a serious heart condition in which there are two main abnormalities: the artery which normally allows blood to be pumped from the heart to the lungs (the pulmonary artery) is completely blocked […] Most babies with pulmonary atresia will need an operation within the first few weeks of life to make sure they can feed their lungs with blood. This operation involves connecting the artery leading to one of the arms to the artery which leads to the lungs, and is called a shunt operation. This doesnt make the babys heart normal, but it usually allows the baby to survive until he or she is old enough to consider more major surgery. […] All heart operations in babies are serious and will carry some risk of the baby dying or having major complications such as brain damage, kidney damage or lung complications such as pneumonia. For shunt operations the risk of a baby dying is small (about 2 in 100) and the risk of brain damage is also small (probably around 1 in 100).

• #27 Pulmonary Atresia With Ventricular Septal Defect | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/105956

  Perioperative Care […] Improved outcomes have been associated with early anticoagulation to decrease thrombotic occlusion of shunts; 1 suggested dose is 10 mg/kg of aspirin (ASA). Another suggestion is improved post-surgical care with frequent clinic visits to re-dose ASA and caregiver education regarding the symptoms of shunt failure with 24-hour medical availability by phone. […] Cardiac Transplant […] In patients with a completely atretic pulmonary system or who have failed the surgical corrective measures, a cardiac transplant can be a viable option.

• #28 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  Initial palliation with staged repair enables neonates to defer the definitive operation until they can undergo repair with relatively lower risks of CPB-associated complications, and larger RV-PA conduit with prolonged durability can be used. However, high in-hospital and inter-stage mortality have been considered as caveats of the staged repair strategy. […] In group-SR, the median age at initial palliation was 22 days. The most common type of palliation was the right modified Blalock-Taussig (MBT) shunt. […] In group-PR, median age, body weight, and Nakata index at Rastelli-type repair was 29 days, 3.4 kg, and 175 mm2/m2, respectively. […] Post-repair re-intervention included numerous surgical or catheter-based interventions after Rastelli-type repair. […] When analyzing the risk factors for decreased time to adverse events using the Cox proportional hazards model, group-PR (against group-SR) was the only significant risk factor for decreased time to death or 1st re-intervention and death or 2nd re-intervention.

• #29 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to increase blood flow to the lungs. It creates a connection (shunt) for blood to flow using a small synthetic tube. An example is the Blalock-Taussig shunt (BT shunt). […] Neonatal complete repair. If a baby’s heart has well-developed pulmonary arteries and no MAPCAs, a surgeon can perform a complete repair at one time during the first 4 weeks of life. During the complete repair surgery, the ventricular septal defect is closed and a tube with an artificial valve is placed between the right lower heart chamber (ventricle) and the pulmonary artery. […] One-stage complete repair. Also called unifocalization, this procedure is done to connect all the MAPCAs together to create new pulmonary arteries. The surgeon then completes the repair with closure of the VSD. A tube graft (with or without a valve) is used to make a connection between the right lower heart chamber and the pulmonary artery. This procedure is usually done between 4 and 6 months of age.

• #30

  https://www.kuh.ku.edu.tr/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-ventricular-septal-defect

  Neonatal complete repair. If a baby’s heart has well-formed pulmonary arteries and no MAPCAs, a surgeon can do a complete repair at one time during the first four weeks of life. […] One-stage complete repair. Also called unifocalization, this treatment is done to connect all the MAPCAs together to make a new pulmonary artery. […] Staged unifocalization. If the MAPCAs are small or have many areas of narrowing, surgery to connect them may be done in stages. […] Babies with pulmonary atresia with ventricular septal defect (VSD) need regular checkups by a doctor with training in heart conditions present at birth.

• #31 Pulmonary Atresia with Ventricular Septal Defect, and Right Ventricle-to-Pulmonary Artery Conduits | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-ventricular-septal-defect-and-right-ventricle-to-pulmonary-artery-conduits/

  Unifocalization is a technique in which MAPCAs are disconnected from the descending aorta and anastomosed to a common confluence that can receive blood supply from a stable sourceusually the RV or a systemic shunt. […] The VSD may be closed with Dacron, polytetrafluoroethylene (PTFE), or pericardial patch material. […] An RV-to-PA conduit can be sewn to confluent central native pulmonary arteries or to unifocalized pulmonary vascular segments. […] Lung or heartlung transplantation may be applied selectively for certain patients, generally adolescents or adults, with irreversible pulmonary hypertension.

• #32 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to increase blood flow to the lungs. It creates a connection (shunt) for blood to flow using a small synthetic tube. An example is the Blalock-Taussig shunt (BT shunt). […] Neonatal complete repair. If a baby’s heart has well-developed pulmonary arteries and no MAPCAs, a surgeon can perform a complete repair at one time during the first 4 weeks of life. During the complete repair surgery, the ventricular septal defect is closed and a tube with an artificial valve is placed between the right lower heart chamber (ventricle) and the pulmonary artery. […] One-stage complete repair. Also called unifocalization, this procedure is done to connect all the MAPCAs together to create new pulmonary arteries. The surgeon then completes the repair with closure of the VSD. A tube graft (with or without a valve) is used to make a connection between the right lower heart chamber and the pulmonary artery. This procedure is usually done between 4 and 6 months of age.

• #33 What Is Pulmonary Atresia With Ventricular Septal Defect?

  https://www.icliniq.com/articles/heart-circulatory-health/pulmonary-atresia-with-ventricular-septal-defect

  Some Common Surgeries Performed Are: […] In this procedure, the blood vessels coming out of the pulmonary artery are reconstructed. A balloon is placed at the catheter’s tip, which helps widen any narrow region. A stent (a rigid tube) is placed between the aorta and pulmonary artery to open it, allowing the blood to flow to the lungs. […] This procedure is required in the initial days of life to enhance blood flow to the lungs. This forms a connection for the blood to flow through the synthetic tube. […] If the newborn has properly developed pulmonary arteries and no MAPCAs, the doctor can perform a repair during the initial four weeks of life. During the surgery, the ventricular septal defect is closed, and an artificial valve with a tube is placed between the ventricle and the pulmonary artery.

• #34 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to increase blood flow to the lungs. It creates a connection (shunt) for blood to flow using a small synthetic tube. An example is the Blalock-Taussig shunt (BT shunt). […] Neonatal complete repair. If a baby’s heart has well-developed pulmonary arteries and no MAPCAs, a surgeon can perform a complete repair at one time during the first 4 weeks of life. During the complete repair surgery, the ventricular septal defect is closed and a tube with an artificial valve is placed between the right lower heart chamber (ventricle) and the pulmonary artery. […] One-stage complete repair. Also called unifocalization, this procedure is done to connect all the MAPCAs together to create new pulmonary arteries. The surgeon then completes the repair with closure of the VSD. A tube graft (with or without a valve) is used to make a connection between the right lower heart chamber and the pulmonary artery. This procedure is usually done between 4 and 6 months of age.

• #35 Pulmonary Atresia With Ventricular Septal Defect | SCAI – Seconds Count

  https://www.secondscount.org/condition/pulmonary-atresia-ventricular-septal-defect

  After the child grows, a more definitive surgical repair is performed at 6 to 12 months of age, at which time the hole between the lower chambers of the heart (VSD) is often closed with a patch, the shunt will be closed off (ligated), and a tube containing a biologic valve is placed from the heart directly to the lung arteries. […] If MAPCAs exist, the options will be more complicated. If these blood arteries are suspected, the baby will undergo a heart catheterization or cardiac computerized tomography (CT) scan shortly after birth in order to map out where these arteries arise and to which part of the lungs they go. […] Instead, staged procedures may be considered, starting at 3 to 6 months of age. The purpose is to try to bring these blood arteries together surgically in order to recreate the lung arteriesso they will eventually be able to be connected back to the heart. These procedures are called unifocalization procedures. This process of rebuilding the lung arteries may require multiple surgical and catheter-based procedures. These arteries may become narrowed, at which time angioplasty, stenting, or further surgical repair may be considered.

• #36 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery. […] Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it’s the first step toward an eventual complete repair. […] Babies with pulmonary atresia with ventricular septal defect need regular checkups by a doctor trained in heart conditions present at birth. This type of doctor is called a congenital cardiologist.

• #37 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. […] Surgical options for neonates and young infants with PA/VSD have been under debate. Early primary repairs guarantee the restoration of serial circulation, and may prevent progression of right ventricular hypertrophy (RVH). However, early primary repairs present increased risks of cardiopulmonary bypass (CPB) for young and small patients as well as inevitable placement of a small RV-PA conduit, which necessitates early conduit replacement.

• #38 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  The modern approach to PA/VSD/MAPCAs is the synthesis of decades of experience of the world’s leading centers in the treatment of this disease. […] The ideal end goal of treatment is to establish pulmonary blood flow from the right ventricle (RV) to as normal a pulmonary vascular bed as possible to allow VSD closure with the lowest possible RV pressure after repair. […] The goals of surgical treatment are to achieve a single confluence of all sources of pulmonary blood flow (unifocalization) and to complete the repair by closing the VSD and creating a connection between the RV and pulmonary artery (PA). […] Pulmonary artery rehabilitation aimed at inducing native pulmonary artery growth was the first surgical treatment described for PA/VSD/MAPCAs. […] The unifocalization technique, particularly in single-stage constitutes the approach of choice advocated by the Stanford group.

• #39 Pulmonary atresia with ventricular septal defect – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-ventricular-septum-defect/diagnosis-treatment/drc-20580526

  During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery. […] Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it’s the first step toward an eventual complete repair. […] Babies with pulmonary atresia with ventricular septal defect need regular checkups by a doctor trained in heart conditions present at birth. This type of doctor is called a congenital cardiologist.

• #40 Midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries – Mainwaring – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/33245/html

  Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. […] The purpose of this chapter is to summarize the surgical algorithm used at Stanford University with a special focus on midline unifocalization. […] The dominant pathway is midline unifocalization, which was utilized in 80% of all patients. […] For the patients who underwent a midline unifocalization, 85% had a single-stage complete repair while 15% had a unifocalization and shunt. […] Ultimately, 97% of the midline unifocalization patients were repaired. […] The algorithm utilized at Stanford takes into account the anatomic heterogeneity and the results demonstrate that a high percentage of patients achieve full repair.

• #41 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to increase blood flow to the lungs. It creates a connection (shunt) for blood to flow using a small synthetic tube. An example is the Blalock-Taussig shunt (BT shunt). […] Neonatal complete repair. If a baby’s heart has well-developed pulmonary arteries and no MAPCAs, a surgeon can perform a complete repair at one time during the first 4 weeks of life. During the complete repair surgery, the ventricular septal defect is closed and a tube with an artificial valve is placed between the right lower heart chamber (ventricle) and the pulmonary artery. […] One-stage complete repair. Also called unifocalization, this procedure is done to connect all the MAPCAs together to create new pulmonary arteries. The surgeon then completes the repair with closure of the VSD. A tube graft (with or without a valve) is used to make a connection between the right lower heart chamber and the pulmonary artery. This procedure is usually done between 4 and 6 months of age.

• #42 What Is Pulmonary Atresia With Ventricular Septal Defect?

  https://www.icliniq.com/articles/heart-circulatory-health/pulmonary-atresia-with-ventricular-septal-defect

  This procedure, also called uni focalization, is a procedure that is performed to connect every MAPCA to form a new pulmonary artery. The surgeon completes the repair procedure by closing the VSD. A graft forms a connection between the ventricles and the pulmonary artery. This procedure is performed within four to six months of age. […] If MAPCs are small or have many narrowing regions, surgery is done stage-wise to correct them. This makes the arteries grow before complete repair.

• #43 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Staged unifocalization. If the MAPCAs are small or have multiple areas of narrowing, surgery to connect them may be done in stages. This allows the arteries to grow before the complete repair. A small shunt from the aorta to the newly created pulmonary arteries allows the blood to flow to the lungs. After a few months, cardiac imaging tests are done to determine whether the baby is ready for the complete repair. […] Babies with pulmonary atresia with ventricular septal defect (VSD) need regular checkups by a cardiologist with training in pediatric congenital heart disease.

• #44 Pulmonary atresia with ventricular septal defect | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-ventricular-septal-defect?content_id=CON-20380877

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt. […] Complete repair. This treatment may be done when as a baby is young or after a shunt or stent is placed. During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery. […] Unifocalization. Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it’s the first step toward an eventual complete repair.

• #45 Midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries – Mainwaring – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/33245/html

  Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a relatively rare and extremely heterogeneous form of congenital heart disease. […] The purpose of this chapter is to summarize the surgical algorithm used at Stanford University with a special focus on midline unifocalization. […] The dominant pathway is midline unifocalization, which was utilized in 80% of all patients. […] For the patients who underwent a midline unifocalization, 85% had a single-stage complete repair while 15% had a unifocalization and shunt. […] Ultimately, 97% of the midline unifocalization patients were repaired. […] The algorithm utilized at Stanford takes into account the anatomic heterogeneity and the results demonstrate that a high percentage of patients achieve full repair.

• #46 Midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries – Mainwaring – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/33245/html

  The introduction of the midline unifocalization approach represented a paradigm shift in the treatment of PA/VSD/MAPCAs. […] There are numerous groups that have subsequently adopted the midline unifocalization approach and have confirmed the success of this surgical algorithm relative to the untreated natural history. […] The majority of patients will have stable hemodynamics over time. […] The mid-line unifocalization approach is our choice for patients with predominantly single-supply MAPCAs and was used in the preponderance (80%) of the patients.

• #47 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  The modern approach to PA/VSD/MAPCAs is the synthesis of decades of experience of the world’s leading centers in the treatment of this disease. […] The ideal end goal of treatment is to establish pulmonary blood flow from the right ventricle (RV) to as normal a pulmonary vascular bed as possible to allow VSD closure with the lowest possible RV pressure after repair. […] The goals of surgical treatment are to achieve a single confluence of all sources of pulmonary blood flow (unifocalization) and to complete the repair by closing the VSD and creating a connection between the RV and pulmonary artery (PA). […] Pulmonary artery rehabilitation aimed at inducing native pulmonary artery growth was the first surgical treatment described for PA/VSD/MAPCAs. […] The unifocalization technique, particularly in single-stage constitutes the approach of choice advocated by the Stanford group.

• #48 Pulmonary Atresia With Ventricular Septal Defect | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/105956

  PAVSD with MAPCAs […] PAVSD with under-developed MAPCAs: […] Primary rehabilitation: An initial procedure targeting small MAPCAs, aiming at improving the central pulmonary arteries with either a prosthetic central shunt or the reimplantation of the main pulmonary artery on the side of the aorta, the Melbourne shunt, or patching of the outflow tract. […] Central shunt: Laks technique […] Side reimplantation: Melbourne […] Right ventricular outflow tract patching […] Secondary rehabilitation: […] Right ventricle to pulmonary artery shunt (Sano) […] Pulmonary artery patching […] Final repair: Involves repair patching of pulmonary arteries and VSD closure […] PAVSD with well-developed MAPCAs […] Unifocalization: This involves reattaching MAPCAs to central pulmonary arteries. Results from a follow-up study found a 95% patency rate of MAPCAs with the midline unifocalization approach by catheterization (although 20% of these had some degree of stenosis). Risk factors for needing reoperation of a uni-focalized bed include the following: Unifocalization/shunt rather than complete repair, Higher initial right ventricle-to-aorta pressure ratios, Absence of central pulmonary arteries.

• #49 Pulmonary Atresia With Ventricular Septal Defect | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/105956

  PAVSD with MAPCAs […] PAVSD with under-developed MAPCAs: […] Primary rehabilitation: An initial procedure targeting small MAPCAs, aiming at improving the central pulmonary arteries with either a prosthetic central shunt or the reimplantation of the main pulmonary artery on the side of the aorta, the Melbourne shunt, or patching of the outflow tract. […] Central shunt: Laks technique […] Side reimplantation: Melbourne […] Right ventricular outflow tract patching […] Secondary rehabilitation: […] Right ventricle to pulmonary artery shunt (Sano) […] Pulmonary artery patching […] Final repair: Involves repair patching of pulmonary arteries and VSD closure […] PAVSD with well-developed MAPCAs […] Unifocalization: This involves reattaching MAPCAs to central pulmonary arteries. Results from a follow-up study found a 95% patency rate of MAPCAs with the midline unifocalization approach by catheterization (although 20% of these had some degree of stenosis). Risk factors for needing reoperation of a uni-focalized bed include the following: Unifocalization/shunt rather than complete repair, Higher initial right ventricle-to-aorta pressure ratios, Absence of central pulmonary arteries.

• #50 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. […] Surgical options for neonates and young infants with PA/VSD have been under debate. Early primary repairs guarantee the restoration of serial circulation, and may prevent progression of right ventricular hypertrophy (RVH). However, early primary repairs present increased risks of cardiopulmonary bypass (CPB) for young and small patients as well as inevitable placement of a small RV-PA conduit, which necessitates early conduit replacement.

• #51

  https://link.springer.com/article/10.1007/s00246-020-02352-9

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset.

• #52 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. […] Surgical options for neonates and young infants with PA/VSD have been under debate. Early primary repairs guarantee the restoration of serial circulation, and may prevent progression of right ventricular hypertrophy (RVH). However, early primary repairs present increased risks of cardiopulmonary bypass (CPB) for young and small patients as well as inevitable placement of a small RV-PA conduit, which necessitates early conduit replacement.

• #53 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. […] Surgical options for neonates and young infants with PA/VSD have been under debate. Early primary repairs guarantee the restoration of serial circulation, and may prevent progression of right ventricular hypertrophy (RVH). However, early primary repairs present increased risks of cardiopulmonary bypass (CPB) for young and small patients as well as inevitable placement of a small RV-PA conduit, which necessitates early conduit replacement.

• #54 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  Initial palliation with staged repair enables neonates to defer the definitive operation until they can undergo repair with relatively lower risks of CPB-associated complications, and larger RV-PA conduit with prolonged durability can be used. However, high in-hospital and inter-stage mortality have been considered as caveats of the staged repair strategy. […] In group-SR, the median age at initial palliation was 22 days. The most common type of palliation was the right modified Blalock-Taussig (MBT) shunt. […] In group-PR, median age, body weight, and Nakata index at Rastelli-type repair was 29 days, 3.4 kg, and 175 mm2/m2, respectively. […] Post-repair re-intervention included numerous surgical or catheter-based interventions after Rastelli-type repair. […] When analyzing the risk factors for decreased time to adverse events using the Cox proportional hazards model, group-PR (against group-SR) was the only significant risk factor for decreased time to death or 1st re-intervention and death or 2nd re-intervention.

• #55

  https://link.springer.com/article/10.1007/s00246-020-02352-9

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset.

• #56 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  Initial palliation with staged repair enables neonates to defer the definitive operation until they can undergo repair with relatively lower risks of CPB-associated complications, and larger RV-PA conduit with prolonged durability can be used. However, high in-hospital and inter-stage mortality have been considered as caveats of the staged repair strategy. […] In group-SR, the median age at initial palliation was 22 days. The most common type of palliation was the right modified Blalock-Taussig (MBT) shunt. […] In group-PR, median age, body weight, and Nakata index at Rastelli-type repair was 29 days, 3.4 kg, and 175 mm2/m2, respectively. […] Post-repair re-intervention included numerous surgical or catheter-based interventions after Rastelli-type repair. […] When analyzing the risk factors for decreased time to adverse events using the Cox proportional hazards model, group-PR (against group-SR) was the only significant risk factor for decreased time to death or 1st re-intervention and death or 2nd re-intervention.

• #57 Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7223124/

  A staged repair strategy, compared with a primary repair strategy, was associated with comparable overall survival with less frequent re-interventions after Rastelli-type repair. Lowering inter-stage mortality rates after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes.

• #58

  https://link.springer.com/article/10.1007/s00246-020-02352-9

  The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. […] A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset.

• #59 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  In severe cases where the pulmonary valve is completely atretic, and the pulmonary circulation is entirely dependent on ductus arteriosus, prostaglandin E1 should be considered to keep the duct open until a surgical correction occurs. […] Conventional treatment, occurring in about 75% of cases, involves discharge of the neonates from the hospital to return at 3 to 6 months of age when their organs are more developed. […] The size of the pulmonary arteries, the presence/absence of MAPCAs, and PDA drive the surgical plan. […] The Nakata index (NI) is an angiographic technique used to standardize the cross-sectional area of the bilateral pulmonary arteries quantitatively. […] Early total correction […] In this less popular approach, which is preferred mostly in mild cases, surgeons usually correct the anatomy within the same admission if not the same operation. […] In patients with a completely atretic pulmonary system or who have failed the surgical corrective measures, a cardiac transplant can be a viable option.

• #60 Pulmonary Atresia With Ventricular Septal Defect | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/105956

  The MAPCAs are closed in the next step, usually at 6 months of age, if the respective lung segments receive enough supply through the now-developed pulmonary arteries. […] VSD is repaired at the end, usually at 1 to 3 years old. The major benefit of this approach is that the whole procedure becomes more tolerable by the already feeble neonate, and breaks are available to allow for healing. In contrast to staged repair, the other approach is complete repair in 1 surgery. This procedure is only suitable for limited candidates on the low end of the severity spectrum. One high-volume center found that infants with a basal oxygen saturation higher than 85% have well-developed pulmonary vasculature amenable to single-stage intervention. […] Early total correction […] In this less popular approach, which is preferred mostly in mild cases, surgeons usually correct the anatomy within the same admission if not the same operation. The patients are carefully picked, and risks versus benefits are critically analyzed before choosing this option. Some centers have found lower mortality and intervention rates with single-stage total correction. As noted above, neonates with frank CHF symptoms may require early correction. In addition, infants with persistent PDA or hemi-truncus can develop pulmonary vascular obstructive disease, also requiring early correction.

• #61 Pulmonary Atresia With Ventricular Septal Defect | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/105956

  The MAPCAs are closed in the next step, usually at 6 months of age, if the respective lung segments receive enough supply through the now-developed pulmonary arteries. […] VSD is repaired at the end, usually at 1 to 3 years old. The major benefit of this approach is that the whole procedure becomes more tolerable by the already feeble neonate, and breaks are available to allow for healing. In contrast to staged repair, the other approach is complete repair in 1 surgery. This procedure is only suitable for limited candidates on the low end of the severity spectrum. One high-volume center found that infants with a basal oxygen saturation higher than 85% have well-developed pulmonary vasculature amenable to single-stage intervention. […] Early total correction […] In this less popular approach, which is preferred mostly in mild cases, surgeons usually correct the anatomy within the same admission if not the same operation. The patients are carefully picked, and risks versus benefits are critically analyzed before choosing this option. Some centers have found lower mortality and intervention rates with single-stage total correction. As noted above, neonates with frank CHF symptoms may require early correction. In addition, infants with persistent PDA or hemi-truncus can develop pulmonary vascular obstructive disease, also requiring early correction.

• #62 Pulmonary Valve Atresia

  https://www.rwjbh.org/treatment-care/heart-and-vascular-care/diseases-conditions/congenital-heart-disease/pulmonary-valve-atresia/

  Your baby will need urgent medical attention once pulmonary atresia symptoms develop. The choice of surgeries or procedures will depend on the severity of your child’s condition. Some treatment options include: […] An intravenous drug called prostaglandin will prevent closure of the natural connection (ductus arteriosus) between the pulmonary artery and the aorta. […] Modified Blalock Taussig shunt […] Rastelli procedure […] Glenn operation […] Fontan procedure […] Pulmonary valve repair or replacement […] Heart transplantation.

• #63 Surgery for Pulmonary Atresia Without VSD for Children – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=surgery-for-pulmonary-atresia-without-vsd-for-children-161-107

  In some cases, an abnormal hole is present between the walls of the ventricles. Its called a ventricular septal defect (VSD). Children with VSD need special treatment, including surgery. For children who have pulmonary atresia without VSD, treatments vary based on other problems that are present. Sometimes the surgeon can fix the pulmonary valve with 2 working ventricles (biventricular repair). In other cases, the surgeon fixes this health problem using only 1 pumping ventricle in a series of surgeries. Those procedures may include a Glenn and Fontan palliation (single-ventricle repair). Other times, the surgeon may do a surgery that provides 2 functioning ventricles. But the right ventricle only pumps some of the blood it normally would (one-and-a-half ventricle repair). […] Your childs healthcare provider will carefully study your childs heart before deciding on the best type of surgery. If possible, he or she will likely choose to do a biventricular repair. It needs fewer surgeries. It also often leads to fewer long-term complications. But it may not be possible if the right ventricle is underdeveloped. Or if there are problems with the coronary arteries. Ask your childs healthcare provider about which procedure or surgery makes the most sense for your child. […] Your child will need follow-up care from a cardiologist after the surgery. That is especially true in the case of a single (or one-and-a-half) ventricle-style repair. Many children with pulmonary atresia without VSD do quite well. But follow-up surgery or other procedures may be needed.

• #64 Pulmonary Atresia Surgery | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/treatments/surgical/pulmonary-atresia-treatment/

  Another option is following the single-ventricle pathway with staged surgeries to separate the oxygenated arterial circulation from the deoxygenated venous circulation. Two more surgeries — the Glenn procedure and Fontan procedure — allow blood to flow through the lungs to pick up oxygen so that it can then be distributed throughout the body. […] The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute can identify the best surgical options for your child with pulmonary atresia.

• #65 Pulmonary Atresia – Tiny Tickers

  https://www.tinytickers.org/support-info/what-is-chd/pulmonary-atresia/

  This will be followed by several stages of surgery during the first few years of the child’s life to balance and optimise blood flow to the lungs and the body. […] Other surgeries may include: Glenn procedure: The second surgery is usually done when the baby is around 6-12 months of age and the surgeons move the superior vena cava from the heart and attach it to the pulmonary artery so that the blood can collect oxygen from the lungs without having to go through the heart. […] Fontan procedure: The third stage of surgery, usually done when the child is 2 – 6 years old. The timing of this surgery is dependent on oxygen levels and the child’s general well-being. This surgery involves a tube being attached from the inferior vena cava to the pulmonary artery, so most of the blood from the body now flows directly to the lungs-the surgeon will normally leave a little hole between the tube and the right atrium-this is called a fenestration. This procedure does not create a normal blood circulation in the body but it does improve the circulation.

• #66 Pulmonary Atresia – Tiny Tickers

  https://www.tinytickers.org/support-info/what-is-chd/pulmonary-atresia/

  This will be followed by several stages of surgery during the first few years of the child’s life to balance and optimise blood flow to the lungs and the body. […] Other surgeries may include: Glenn procedure: The second surgery is usually done when the baby is around 6-12 months of age and the surgeons move the superior vena cava from the heart and attach it to the pulmonary artery so that the blood can collect oxygen from the lungs without having to go through the heart. […] Fontan procedure: The third stage of surgery, usually done when the child is 2 – 6 years old. The timing of this surgery is dependent on oxygen levels and the child’s general well-being. This surgery involves a tube being attached from the inferior vena cava to the pulmonary artery, so most of the blood from the body now flows directly to the lungs-the surgeon will normally leave a little hole between the tube and the right atrium-this is called a fenestration. This procedure does not create a normal blood circulation in the body but it does improve the circulation.

• #67 Pulmonary Atresia With Ventricular Septal Defect – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK562277/

  In severe cases where the pulmonary valve is completely atretic, and the pulmonary circulation is entirely dependent on ductus arteriosus, prostaglandin E1 should be considered to keep the duct open until a surgical correction occurs. […] Conventional treatment, occurring in about 75% of cases, involves discharge of the neonates from the hospital to return at 3 to 6 months of age when their organs are more developed. […] The size of the pulmonary arteries, the presence/absence of MAPCAs, and PDA drive the surgical plan. […] The Nakata index (NI) is an angiographic technique used to standardize the cross-sectional area of the bilateral pulmonary arteries quantitatively. […] Early total correction […] In this less popular approach, which is preferred mostly in mild cases, surgeons usually correct the anatomy within the same admission if not the same operation. […] In patients with a completely atretic pulmonary system or who have failed the surgical corrective measures, a cardiac transplant can be a viable option.

• #68 Pulmonary Atresia With Ventricular Septal Defect – MD Searchlight

  https://mdsearchlight.com/heart-health/pulmonary-atresia-with-ventricular-septal-defect/

  Ultimately, surgical treatment is usually necessary. […] The use of aspirin is suggested before and after surgery to decrease the chances of blood clots forming in the vessels or any surgical shunts. […] In cases where the lung blood vessels are completely closed off, or where corrective measures have not worked, a heart transplant may be considered.

• #69 Pulmonary atresia | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20155225/

  Babies with pulmonary atresia often need many heart surgeries over time. The type of heart surgery depends on the size of the child’s lower right heart chamber and pulmonary artery. […] If the baby also has a ventricular septal defect (VSD), surgery is done to patch the hole. Then the surgeon makes a connection from the right pumping chamber to the pulmonary artery. This repair may use an artificial valve.

• #70 Pulmonary Atresia (PA) | Causes, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/patients/child/encyclopedia/defects/pa

  If the tricuspid valve and right ventricle are normal sized, the child should be able to have surgery to bring blood from the right ventricle to the lungs. […] If the tricuspid valve and right ventricle size is in between (not very small, but not normal sized), then the child may require a combination of the above surgeries so the right ventricle does not have to handle all the blood that would normally go through it to the lungs. […] Finally, in some patients with significant coronary abnormalities who do not have good blood supply to their heart muscle, a heart transplant may be required.

• #71 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Closing your baby’s ventricular septal defect. […] Placing a donated artery and valve between your child’s right ventricle and pulmonary artery. This lets blood flow through their right ventricle into their pulmonary artery and to their lungs. […] After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation. […] No. Medical procedures and surgeries can improve your child’s condition, but they aren’t cures.

• #72 Pulmonary Atresia | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/pulmonary-atresia

  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. Members of their health care team may make changes to your baby’s medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] 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 cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.

• #73 Pulmonary Atresia | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/pulmonary-atresia

  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. Members of their health care team may make changes to your baby’s medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] 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 cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.

• #74 Pulmonary Atresia | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/pulmonary-atresia

  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. Members of their health care team may make changes to your baby’s medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] 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 cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.

• #75 Pulmonary Atresia | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/pulmonary-atresia

  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. Members of their health care team may make changes to your baby’s medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] 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 cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.

• #76 Pulmonary Atresia | Nationwide Children’s Hospital

  https://www.nationwidechildrens.org/conditions/pulmonary-atresia

  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. Members of their health care team may make changes to your baby’s medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] 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 cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.

• #77 Pulmonary atresia with ventricular septal defect | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/pulmonary-atresia-with-ventricular-septal-defect?content_id=CON-20380877

  Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt. […] Complete repair. This treatment may be done when as a baby is young or after a shunt or stent is placed. During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery. […] Unifocalization. Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it’s the first step toward an eventual complete repair.

• #78 Pulmonary atresia with ventricular septal defect – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-ventricular-septal-defect/

  Staged unifocalization. If the MAPCAs are small or have multiple areas of narrowing, surgery to connect them may be done in stages. This allows the arteries to grow before the complete repair. A small shunt from the aorta to the newly created pulmonary arteries allows the blood to flow to the lungs. After a few months, cardiac imaging tests are done to determine whether the baby is ready for the complete repair. […] Babies with pulmonary atresia with ventricular septal defect (VSD) need regular checkups by a cardiologist with training in pediatric congenital heart disease.

• #79 Conditions – Leeds Congenital Hearts

  https://leedscongenitalhearts.com/teen/conditions/view/3/36/pulmonary-atresia-with-ventricular-septal-defect

  All heart operations in babies are serious and will carry some risk of the baby dying or having major complications such as brain damage, kidney damage or lung complications such as pneumonia. For shunt operations the risk of a baby dying is small (about 2 in 100) and the risk of brain damage is also small (probably around 1 in 100). […] All children with pulmonary atresia and VSD who have had a shunt operation as a baby will need further treatment when they are older. […] If they grow well it may be possible, with major surgery, to open up the blockage between the heart and the pulmonary artery (sometimes using a human or pigs valve) and to close the ventricular septal defect by sewing a patch over it. This is often referred to as corrective surgery, but it does not make the heart completely normal. […] All patients with pulmonary atresia and VSD will be at risk of infection in the heart (called endocarditis), both before and after surgery.

• #80 Pulmonary Atresia: Symptoms, Causes and Treatment

  https://my.clevelandclinic.org/health/diseases/14779-pulmonary-atresia

  Closing your baby’s ventricular septal defect. […] Placing a donated artery and valve between your child’s right ventricle and pulmonary artery. This lets blood flow through their right ventricle into their pulmonary artery and to their lungs. […] After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation. […] No. Medical procedures and surgeries can improve your child’s condition, but they aren’t cures.

• #81 Pulmonary atresia with ventricular septal defect – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_atresia_with_ventricular_septal_defect

  The surgical methods that can be used to treat (for the long-term) this condition include: Catheter procedure for pulmonary artery branches, Systemic-to-pulmonary artery shunt, Neonatal complete repair, One-stage complete repair, Balloon septostomy, Staged unifocalization. […] Prognosis after surgical intervention is generally good.

• #82 Pulmonary Atresia with VSD

  https://www.rch.org.au/cardiology/heart_defects/Pulmonary_Atresia_with_VSD/

  This defect is a form of Tetralogy of Fallot in which there is complete obstruction of the Pulmonary Artery resulting in total diversion of blood from the right ventricle into the aorta. Survival depends on the ductus remaining open in the early days of life (in order for blood to reach the lungs), or on the presence of other connecting blood vessels between the Aorta and the Pulmonary Arteries in the lungs (Collaterals). Most babies will need a 'Shunt’ operation during infancy, involving insertion of a tiny piece of artificial tube (made from Goretex) between the Aorta, or a branch (usually one of the arm arteries), and one of the branch Pulmonary Arteries. Complete Repair is carried out at two to three years. Complete repair for Pulmonary Atresia is usually carried out after the first year of life, though sometimes it may be performed earlier. […] Complete repair for Pulmonary Atresia usually necessitates the insertion of a 'Conduit’, which is a tube containing a valve, placed to connect the right ventricle to the pulmonary artery. The VSD is also closed and the pulmonary arteries may require enlargement with one or more patches.

• #83 Pulmonary Atresia With Ventricular Septal Defect | SCAI – Seconds Count

  https://www.secondscount.org/condition/pulmonary-atresia-ventricular-septal-defect

  After the child grows, a more definitive surgical repair is performed at 6 to 12 months of age, at which time the hole between the lower chambers of the heart (VSD) is often closed with a patch, the shunt will be closed off (ligated), and a tube containing a biologic valve is placed from the heart directly to the lung arteries. […] If MAPCAs exist, the options will be more complicated. If these blood arteries are suspected, the baby will undergo a heart catheterization or cardiac computerized tomography (CT) scan shortly after birth in order to map out where these arteries arise and to which part of the lungs they go. […] Instead, staged procedures may be considered, starting at 3 to 6 months of age. The purpose is to try to bring these blood arteries together surgically in order to recreate the lung arteriesso they will eventually be able to be connected back to the heart. These procedures are called unifocalization procedures. This process of rebuilding the lung arteries may require multiple surgical and catheter-based procedures. These arteries may become narrowed, at which time angioplasty, stenting, or further surgical repair may be considered.

• #84 Pulmonary Atresia (PA) – Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/pulmonary-atresia-pa/

  The long-term outlook after pulmonary atresia repair varies from child to child. Children should receive lifelong follow-up care at a center offering pediatric (and ultimately adult) congenital cardiac care. It is not unexpected for further surgery to be performed to replace conduits or revise a palliation.

• #85 Pulmonary Atresia: Overview, Causes, Treatment

  https://www.healthline.com/health/pulmonary-atresia

  In general, the goal of treatment is to improve blood flow to the lungs and alleviate symptoms. […] A common approach is surgery to create a pathway for blood to reach the lungs. This can involve creating a connection between the right ventricle and the pulmonary arteries (a shunt) or enlarging the pulmonary valve. In some cases, a series of surgeries may be necessary over time to further improve blood flow. […] Management of pulmonary atresia typically requires lifelong care, especially in complex cases or with associated heart irregularities. […] The success rates of surgery for pulmonary atresia depend on various factors, including the specific type of pulmonary atresia, the presence of other heart irregularities, and the overall health of the infant. […] A study of 107 infants with PA-VSD who underwent surgery between 1989 and 2019 found that most (85%) had a complete repair at around 1 year old. Survival rates were 90% at 6 months, 85% at 20 months, and 81% at 10 years after surgery.

• #86 Pulmonary Atresia: Overview, Causes, Treatment

  https://www.healthline.com/health/pulmonary-atresia

  Using smaller shunts during surgery led to quicker complete repairs but also increased the risk of complications between surgeries. Overall, staged repair for PA-VSD showed high success rates for complete repair and long-term survival. […] Pulmonary atresia can be a serious condition with a risk of death, especially without appropriate treatment. But with advances in medical and surgical care, the outlook for individuals with pulmonary atresia has improved significantly, and many can lead relatively normal lives with appropriate management. […] Treatment often involves surgery to create a pathway for blood flow. […] With modern medical and surgical advancements, most infants born with pulmonary atresia now survive into adulthood. The exact survival rate varies, but overall, there has been a significant improvement in survival rates in recent years.

• #87

  https://link.springer.com/article/10.1007/s11936-003-0047-3

  It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patients pericardium, and this patient continues to do well. […] Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. […] Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.

• #88

  https://link.springer.com/article/10.1007/s11936-003-0047-3

  It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patients pericardium, and this patient continues to do well. […] Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. […] Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.

• #89 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  Regardless of the strategy, the results have altered the natural history of the disease, with a complete cure rate of approximately 80% and low early and late mortality. […] The use of the intraoperative flow study as a functional measure of pulmonary vascular performance and suitability for VSD closure was first reported by Reddy and colleagues. […] At our institution, patients who did not receive VSD closure in the first instance are re-evaluated, also on the basis of the individual clinical condition, 6–12 months after surgery by cardiac catheterization, possibly associated with CMR.

• #90

  https://link.springer.com/article/10.1007/s11936-003-0047-3

  It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patients pericardium, and this patient continues to do well. […] Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. […] Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.

• #91 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  The modern approach to PA/VSD/MAPCAs is the synthesis of decades of experience of the world’s leading centers in the treatment of this disease. […] The ideal end goal of treatment is to establish pulmonary blood flow from the right ventricle (RV) to as normal a pulmonary vascular bed as possible to allow VSD closure with the lowest possible RV pressure after repair. […] The goals of surgical treatment are to achieve a single confluence of all sources of pulmonary blood flow (unifocalization) and to complete the repair by closing the VSD and creating a connection between the RV and pulmonary artery (PA). […] Pulmonary artery rehabilitation aimed at inducing native pulmonary artery growth was the first surgical treatment described for PA/VSD/MAPCAs. […] The unifocalization technique, particularly in single-stage constitutes the approach of choice advocated by the Stanford group.

• #92 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  The modern approach to PA/VSD/MAPCAs is the synthesis of decades of experience of the world’s leading centers in the treatment of this disease. […] The ideal end goal of treatment is to establish pulmonary blood flow from the right ventricle (RV) to as normal a pulmonary vascular bed as possible to allow VSD closure with the lowest possible RV pressure after repair. […] The goals of surgical treatment are to achieve a single confluence of all sources of pulmonary blood flow (unifocalization) and to complete the repair by closing the VSD and creating a connection between the RV and pulmonary artery (PA). […] Pulmonary artery rehabilitation aimed at inducing native pulmonary artery growth was the first surgical treatment described for PA/VSD/MAPCAs. […] The unifocalization technique, particularly in single-stage constitutes the approach of choice advocated by the Stanford group.

• #93 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  Regardless of the strategy, the results have altered the natural history of the disease, with a complete cure rate of approximately 80% and low early and late mortality. […] The use of the intraoperative flow study as a functional measure of pulmonary vascular performance and suitability for VSD closure was first reported by Reddy and colleagues. […] At our institution, patients who did not receive VSD closure in the first instance are re-evaluated, also on the basis of the individual clinical condition, 6–12 months after surgery by cardiac catheterization, possibly associated with CMR.

• #94 The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries

  https://www.mdpi.com/2227-9067/9/4/515

  Regardless of the strategy, the results have altered the natural history of the disease, with a complete cure rate of approximately 80% and low early and late mortality. […] The use of the intraoperative flow study as a functional measure of pulmonary vascular performance and suitability for VSD closure was first reported by Reddy and colleagues. […] At our institution, patients who did not receive VSD closure in the first instance are re-evaluated, also on the basis of the individual clinical condition, 6–12 months after surgery by cardiac catheterization, possibly associated with CMR.

• #95 Pulmonary Atresia With Ventricular Septal Defect – MD Searchlight

  https://mdsearchlight.com/heart-health/pulmonary-atresia-with-ventricular-septal-defect/

  Ultimately, surgical treatment is usually necessary. […] The use of aspirin is suggested before and after surgery to decrease the chances of blood clots forming in the vessels or any surgical shunts. […] In cases where the lung blood vessels are completely closed off, or where corrective measures have not worked, a heart transplant may be considered.

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