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. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. 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. Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation. The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care.

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

  https://www.ncbi.nlm.nih.gov/sites/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. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. 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. Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation. The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care. Through effective collaboration, healthcare teams enhance early diagnosis, optimize surgical outcomes, and provide long-term management strategies that improve survival and quality of life for affected patients.

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

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

  When a normal heart squeezes, the right ventricle (the lower right pumping chamber) contracts, pushing the blood out to the lungs. The pulmonary valve is an opening on the right side of the heart that helps prevent blood from leaking back into the heart between beats. In the congenital heart disease pulmonary atresia, the pulmonary valve has not formed correctly: it is sealed and can’t open. […] Many children with pulmonary atresia also have a ventricular septal defect (VSD), a hole in the tissue between the lower chambers of the heart. This combination is often called tetralogy of Fallot with 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.

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

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

  Pulmonary atresia (PA) is a congenital heart defect in which the one-way valve (pulmonary valve) between the right ventricle (responsible for pumping oxygen-poor blood through the pulmonary artery and to the lungs) and pulmonary artery (responsible for carrying blood to the lungs) does not develop at all. […] In the case of pulmonary atresia with ventricle septal defect (PA with VSA), a hole between the two pumping chambers of the heart (ventricular septal defect) is also present. […] This combination of defects can also be classified as Tetralogy of Fallot. […] Pulmonary atresia with ventricular septal defect occurs when a hole between the two pumping chambers of the heart (ventricular septal defect) is also present. […] The ventricular septal defect allows the right ventricle to grow and develop despite the pulmonary valve abnormality.

• #5 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

  Pulmonary atresia with ventricular septal defect, also called PA-VSD, is often diagnosed during pregnancy or soon after birth. […] 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. […] 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.

• #6 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. […] After surgery, infants will return to the CVICU to be closely monitored during recovery. […] Pain medications, such as acetaminophen, may be recommended to keep the child comfortable. Your child’s physician will discuss pain control before the child is discharged from the hospital.

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

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

  Oxygen saturation is critical and should be monitored continuously. Similarly, fluid imbalance and acidosis, if present, need to be addressed urgently. Since the neonate is usually unable to feed well, nutritional rehabilitation might also be needed. 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 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. […] Education of parents regarding congenital heart diseases is important as the prognosis is particularly poor, and the patient usually requires multiple surgeries for correction of anomalies. Parents should be educated regarding performing cardiopulmonary resuscitation in children.

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

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

  Oxygen saturation is critical and should be monitored continuously. Similarly, fluid imbalance and acidosis, if present, need to be addressed urgently. Since the neonate is usually unable to feed well, nutritional rehabilitation might also be needed. 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. Symptomatic treatment with diuretics or digoxin is indicated if the patient is going into congestive heart failure. […] 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.

• #9 Pediatric Pulmonary Atresia – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/pulmonary-atresia

  Pulmonary atresia is an abnormal development of the pulmonary valve. […] Your child will get special care in the hospital until surgery can be done. […] 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. […] Your child will need surgery to improve blood flow to the lungs. The type of surgery your child has will depend on whether the tricuspid valve or right ventricle can send enough blood to the lungs. […] After the surgical repair and time for recovery in the hospital, your baby will be able to go home. Your child’s healthcare provider may advise pain medicines such as acetaminophen or ibuprofen to keep your baby comfortable.

• #9 Pediatric Pulmonary Atresia – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/pulmonary-atresia

  The nursing staff will show you how to give any special treatments at home, if needed. Or you may need a home health agency to help. Your child will likely need special formula and supplemental feedings to get enough nutrition. […] After surgery, infants will return to the ICU to be closely monitored during recovery. […] Your child may need to take medications for a while. The staff will give you written instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged. […] Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child’s physician will discuss pain control before your child is discharged from the hospital. […] You may receive additional instructions from your child’s physicians and the hospital staff.

• #10 Pulmonary Atresia

  https://healthlibrary.ecuhealth.org/Conditions/Pregnancy/90,P01809

  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. […] Your child’s healthcare provider may advise pain medicines such as acetaminophen or ibuprofen to keep your baby comfortable. Your child’s heart care team will talk about pain control before your child goes home. […] Your child will need regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of their life.

• #11 Pulmonary Atresia – Seattle Children’s

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

  Most babies with pulmonary atresia show symptoms during the first few hours after birth. They may be diagnosed after having a pulse oximetry screening in their birth hospital when they are about a day old. In some babies, it may take a few days for symptoms to appear. […] If your baby has pulmonary atresia symptoms, they may have these: Skin may look blue or purple tinged, mottled (different shades or colors), grayish or paler than usual; the lips, mouth, gums, fingernails or toenails may look bluish (cyanosis) […] Your child’s doctor will suggest some procedures and treatments right away to improve your baby’s 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 baby’s lung arteries when the normal path between the heart and lungs is closed.

• #11 Pulmonary Atresia – Seattle Children’s

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

  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). […] Your baby will need 1 or more surgeries to improve their blood flow. […] The exact procedures and timing depend on your child’s condition, including how serious it is and whether they have other heart defects. […] Your child may need other surgeries based on their condition, such as surgery to close a ventricular septal defect. […] We provide comprehensive care for children with pulmonary atresia.

• #12 Pulmonary Atresia

  https://healthlibrary.osfhealthcare.org/Conditions/COPD/90,P01809

  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 nursing staff will show you how to give any special treatments at home, if needed. Or you may need a home health agency to help. Your child will likely need special formula and supplemental feedings to get enough nutrition. […] Your child’s heart care team will talk about pain control before your child goes home. […] After each surgery, your pediatric cardiologist will follow your baby’s recovery. They will make changes to medicines, help you with feeding problems, measure oxygen levels, and determine when and if it is time for the next surgery. […] Your child will need regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of their life.

• #13 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).

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

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

  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. […] Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way.

• #14 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. […] 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.

• #15 Pulmonary atresia | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/pulmonary-atresia

  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. […] 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. […] A person born with pulmonary atresia needs regular checkups, even as an adult. A doctor trained in congenital heart diseases, called a congenital cardiologist, often provides care. Get recommended vaccines, including yearly flu vaccines.

• #16 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/

  Pulmonary atresia (PA) is when the pulmonary valve did not form, and no blood can go from the right ventricle into the lungs. […] 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. […] Babies born with pulmonary atresia may have a bluish tint to their skin (cyanosis), they may breathe fast or appear to be struggling to breathe, have poor feeding or be lethargic. […] Catheter based: 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. There are certain types of pulmonary atresia where a catheter can be used to create an opening in the valve. […] Surgical Interventions: 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.

• #17 Pulmonary Atresia | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/pulmonary-atresia/

  Surgery will be needed to create a way to get blood flow to the lungs. Different options include surgically reconstructing the right ventricular outflow tract. […] Another option is a Blalock-Taussig shunt (BT shunt), a small tube that connect the pulmonary artery and the subclavian artery. […] Subsequent surgeries will depend on a number of factors, including right ventricle size and other defects present.

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

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

  Pulmonary atresia with ventricular septal defect (PA/VSD) occurs when theres a hole between the two bottom pumping chambers of the heart and theres no direct connection from the pumping chamber of the heart to the lung arteries. […] Often, multiple heart catheterization procedures are necessary to define the anatomy of these blood arteries and to improve the flow to the lungs before and after surgery. […] Initially after birth, an assessment must be made as to how blood is getting to the lungs. […] An intravenous prostaglandin E1 (PGE1) infusion may also be started to keep the ductus arteriosus open. […] 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. […] 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.

• #19 Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand | Scientific Reports

  https://www.nature.com/articles/s41598-020-61879-2

  Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic congenital heart disease with a wide-range of presentations and treatment strategies, depending on the source of pulmonary circulation, anatomy of pulmonary arteries (PAs), and major aortopulmonary collateral arteries (MAPCAs). […] The goal of the surgical treatment is the relatively straight-forward complete repair with closure of VSD and constructed continuity from right ventricle to the central pulmonary artery. […] Strategies for treating PA/VSD with MAPCAs of single-stage and multi-stage repairs such as unifocalization have been long debated with regards to size and distribution of intrapulmonary arteries and the anatomy of the MAPCAs. […] The prevailing treatment strategy is primarily to reconnect the pulmonary artery tree (unifocalization) and create an appropriate source of pulmonary blood supply to optimize the segmental lung perfusion and avoid segmental pulmonary hypertension.

• #19 Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand | Scientific Reports

  https://www.nature.com/articles/s41598-020-61879-2

  In Thailand, the initial medical treatment for this lesion is usually initiated at a provincial hospital, followed by referral of patients to advanced cardiac centers according to the complexity of the disease and the repair strategies. […] The present study evaluates survival rates and mortality risks of children who were diagnosed with PA/VSD at Siriraj Hospital in the last decade. […] Of the 88 patients who underwent operations, 32 patients had complete repair at 8.4 ± 4.6 years old. […] During the follow-up [median time of 5.7 years (7 days-13.6 years)], 17 patients (18.9%) died. […] The survival rates at 1, 5, and 10 years of age were 95%, 83.7%, and 79.6%, respectively. […] Significant mortality risks were the presence of associated anomalies and non-confluent PAs. […] Overall survival rates of PA/VSD at 1, 5, and 10 years of age, were 95%, 83.7%, and 79.6%, respectively. […] In this study, the significant predictors of death were the presence of associated anomalies and non-confluent PAs. […] Children with PA/VSD have had a fairly good survival rate for the past decade.

• #20 Pulmonary atresia | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/pulmonary-atresia

  Pulmonary atresia (PA) is a complicated congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy. […] After birth, the placenta no longer provides oxygen for the newborn – the lungs must provide it. However, with no pulmonary valve opening present, blood must find another route to reach the lungs and receive oxygen. […] Your child will most likely be admitted to the cardiac intensive care unit (CICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen, and possibly even on a ventilator, to assist his/her breathing. Intravenous (IV) medications may be given to help the heart and lungs function more efficiently. […] After surgery, infants will return to the cardiac intensive care unit (CICU) to be closely monitored during recovery.

• #20 Pulmonary atresia | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/pulmonary-atresia

  Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child. […] Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child’s physician will discuss pain control before your child is discharged from the hospital.

• #21 Pulmonary Atresia | Nationwide Children’s Hospital

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

  Some heart conditions won’t need to be fixed right away. It can be better to give your baby the chance to grow, making recovery much easier. […] Your baby may need help with breathing, extra oxygen, or may need to be started on a medicine called prostaglandin E (PGE, or „prostins”). This medicine keeps the blood flowing through the patent ductus arteriosus (PDA). […] After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). […] Your medical team will help you decide on the safest feeding approach. […] Your hospital stay after birth may be a couple of days or as long as weeks to months. […] You will need to do CPR training in the Columbus Blue Jackets Family Resource Center before you leave. […] Your cardiologist will follow up with your baby’s heart needs even after they are discharged from the hospital.

• #22 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: […] 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. […] Even major surgery later in life cannot make the heart completely normal and it is very unlikely that any child with pulmonary atresia and VSD will have a normal lifespan. […] 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. […] All children with pulmonary atresia and VSD who have had a shunt operation as a baby will need further treatment when they are older. […] Even many years after apparently successful surgery new problems may arise which require treatment. […] Most children with major heart disease cannot manage as much physical exercise as other children. […] All patients with pulmonary atresia and VSD will be at risk of infection in the heart (called endocarditis), both before and after surgery.

• #23 Pulmonary Atresia: Symptoms, Causes and Treatment

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

  Pulmonary atresia is a congenital heart defect, which means its present at birth. […] Pulmonary atresia with a ventricular septal defect. This opening in the wall (septum) between the right and left ventricles allows oxygen-rich blood to mix with oxygen-poor blood. […] 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. […] Surgery for pulmonary atresia with a ventricular septal defect. Usually, the right ventricle is well developed and can pump blood to your babys lungs. Surgery involves closing your babys ventricular septal defect. […] 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. […] Your child will have regular follow-up appointments with their pediatric cardiologist. This starts two to four weeks after leaving the hospital. […] People with pulmonary atresia must remain under the care of a cardiologist for their entire lives.

• #24 Pulmonary Atresia | Phoenix Children’s Hospital

  https://phoenixchildrens.org/specialties-conditions/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 nursing staff will show you how to give any special treatments at home, if needed. Or you may need a home health agency to help. Your child will likely need special formula and supplemental feedings to get enough nutrition. […] Your child will need regular follow-up care at a center offering pediatric or adult congenital heart care for the rest of their life.

• #25 Single Ventricle Defects | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/single-ventricle-defects

  Children with pulmonary atresia need regular follow-up with a pediatric cardiologist and, once they reach adulthood, lifelong regular follow-up with a cardiologist who’s had special training in congenital heart defects. Some children may need medicines, heart catheterization or additional surgery. […] Children with pulmonary atresia are at increased risk for developing endocarditis. Ask your pediatric cardiologist about your child’s need to take antibiotics before certain dental procedures to help prevent endocarditis.

• #25 Single Ventricle Defects | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/single-ventricle-defects

  In pulmonary atresia no pulmonary valve exists. Blood can’t flow from the right ventricle into the pulmonary artery and on to the lungs. The right ventricle and tricuspid valve are often poorly developed. […] If the PDA narrows or closes, the lung blood flow is reduced to critically low levels. This can cause very severe cyanosis. Symptoms may develop soon after birth. […] Temporary treatment includes a drug to keep the PDA from closing. To increase blood flow to the lungs, there are two options. A surgeon can create a shunt between the aorta and the pulmonary artery. Alternatively the interventional catheterizer can place a stent in the patent ductus arteriosus to keep it open. A more complete repair depends on the size of the pulmonary artery and right ventricle. […] Children with pulmonary atresia may be advised to limit their physical activities to their own endurance. Some competitive sports may pose greater risk. Your child’s pediatric cardiologist will help determine the proper level of activity.

• #26 Pulmonary Atresia – Tiny Tickers

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

  Pulmonary atresia is a congenital heart defect in which the pulmonary valve that allows blood to flow to the lungs is not formed properly. […] In most cases, children with pulmonary atresia require intensive medical and nursing care soon after birth. […] Immediate treatment will be gaining intravenous access after birth and the medicine prostaglandin started to keep the ‘duct’ open. […] 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. […] Babies and children with pulmonary atresia will need lifelong follow-up. Whenever tubes are used for repairs, they will always require changing, usually before the 10th birthday and some children may need another intervention during adolescence or adulthood. […] They will need repeat tests like echocardiogram, ECG and sometimes cardiac Magnetic Resonance Imaging (MRI) scans. The aim of these tests is to monitor your child’s heart function so that any future heart problems are diagnosed and treated quickly.

• #27 Pulmonary Atresia: Overview, Causes, Treatment

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

  Pulmonary atresia with ventricular septal defect (PA-VSD): Theres a hole in the wall between the ventricles, allowing some blood to flow into the right ventricle. This can help the right ventricle develop better, but it still has difficulty pumping blood to the lungs. […] Management of pulmonary atresia typically requires lifelong care, especially in complex cases or with associated heart irregularities. […] 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.

• #28 CE Activity | Pulmonary Atresia With Ventricular Septal Defect | Nurses

  https://www.statpearls.com/nurse/ce/activity/105747

  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. This condition is often associated with major aortopulmonary collateral arteries, which supply pulmonary circulation without a normal pulmonary artery. […] Early detection through fetal ultrasound or postnatal echocardiography is crucial, as untreated cases can lead to severe cyanosis, heart failure, and life-threatening complications. 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. Participants gain insight into prenatal and postnatal screening techniques, imaging modalities, and individualized surgical strategies based on anatomical presentation.

• #28 CE Activity | Pulmonary Atresia With Ventricular Septal Defect | Nurses

  https://www.statpearls.com/nurse/ce/activity/105747

  The curriculum emphasizes the importance of an interprofessional approach, integrating expertise from pediatric cardiologists, cardiothoracic surgeons, neonatologists, and genetic counselors to ensure comprehensive care. Through effective collaboration, healthcare teams enhance early diagnosis, optimize surgical outcomes, and provide long-term management strategies that improve survival and quality of life for affected patients. […] At the conclusion of this activity, the learner will be better able to: Identify the etiology of pulmonary atresia with ventricular septal defect medical conditions and associated emergencies. […] Assess the diagnostic evaluation of pulmonary atresia with ventricular septal defect. […] Evaluate the management options available for pulmonary atresia with ventricular septal defect. […] Collaborate among patients, caregivers, and a multidisciplinary team to establish the best corrective action for patients with pulmonary atresia with ventricular septal defect.

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