Materiały źródłowe

• #1 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK546666/?report=reader

  Pulmonary atresia with intact ventricular septum is a rare congenital heart disease comprising less than 1% of all heart defects, characterized by membranous or muscular atresia of the right ventricular outflow tract without any ventricular communication, often involving underdevelopment of the right ventricle and tricuspid valve. […] Newborns with pulmonary atresia with intact ventricular septum frequently exhibit cyanosis and desaturation, especially after the closure of the patent ductus arteriosus, which is crucial for their pulmonary circulation. […] This activity for healthcare professionals is designed to enhance the learner’s competence in identifying pulmonary atresia with intact ventricular septum, performing the recommended evaluation, and implementing an appropriate interprofessional approach when managing this condition.

• #2 Pulmonary Atresia with Intact Ventricular Septum (IVS) | Texas Children’s

  https://www.texaschildrens.org/content/conditions/pulmonary-atresia-with-intact-ventricular-septum-ivs

  Pulmonary atresia with intact ventricular septum is a congenital heart defect in which: The pulmonary valve doesn’t form properly. The pulmonary valve allows blood to flow from the heart to the lungs to get oxygen. In this rare condition, instead of a normal pulmonary valve with three leaflets that open and close, a thickened, abnormal valve forms that does not open, blocking the blood flow to the lungs. […] Babies born with pulmonary atresia don’t have enough oxygen in their blood to support the body’s needs. Treatment at birth is necessary for survival. […] If pulmonary atresia with intact ventricular septum is detected during pregnancy, prompt referral to a fetal cardiac center is important for further evaluation and proper treatment planning. […] Because pulmonary atresia with intact ventricular septum is a life-threatening condition, delivery should take place at a center with the highest quality congenital heart program, ensuring the expertise and resources required to treat your baby at birth, including an advanced neonatal intensive care unit (NICU) and a cardiac intensive care unit (CICU).

• #3 Pulmonary Atresia With Intact Ventricular Septum | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28009

  Pulmonary atresia with intact ventricular septum (PA-IVS) is one of the rare forms of congenital heart disease, accounting for <1% of total heart defects. [...] Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. Intervention, either catheter-based or surgical, is necessary for neonates. Treatment is highly individualized, encompassing biventricular repair, systemic-to-pulmonary shunts, single-ventricular repair, or heart transplantation, depending on the characteristics of the right ventricle and tricuspid valve, as well as the anatomy of the coronary arteries. [...] Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. This action is vital for the preoperative survival of these patients, as the ductus arteriosus is the sole source of pulmonary blood flow. Also, manipulating pulmonary and systemic vascular resistance is crucial for achieving optimal pulmonary and systemic blood flow.

• #4 SSA – POMS: DI 23022.585 – Pulmonary Atresia – 08/28/2020

  https://secure.ssa.gov/poms.nsf/%20lnx/0423022585

  Pulmonary atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen. […] Infants born with this condition also tend to have multiple other cardiac conditions, such as poorly developed tricuspid valves, underdeveloped (hypoplastic) right ventricle, and abnormal blood vessels leading into the heart. Surgery is necessary shortly after birth to form a shunt from the systemic circulation to the pulmonary circulation in order to oxygenate the blood. […] Poor feeding during nursing; […] Infants are given medication (i.e. Prostaglandin) to help keep the blood vessel open between the pulmonary artery and the aorta (patent ductus arteriosis or PDA) until the initial shunt surgery can occur. Later, multi-staged surgeries to repair the vessel, open-heart surgery to repair or replace a pulmonary valve, reconstruction of the heart chambers, and heart transplant have been used to treat patients with this defect. Infants surviving into adulthood should be monitored by a cardiologist specializing in the care of adults with congenital heart disease to assess medication needs, surgery, and infection throughout their lifetime.

• #5 Pulmonary atresia with intact ventricular septum – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-intact-ventricular-septum/cdc-20396714

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, is a rare heart condition that’s present at birth. […] Treatment for pulmonary atresia with intact ventricular septum may include medicines and one or more procedures or surgeries to fix the heart. […] Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart conditions present at birth. […] Treatment for pulmonary atresia with intact ventricular septum, also called PA/IVS, may include medicines and one or more procedures or surgeries. […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it keeps the baby stable until a surgery or procedure can be done to permanently fix blood flow.

• #6 Pulmonary atresia with intact ventricular septum

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/pulmonary-atresia-with-intact-ventricular-septum.html

  This leaflet is to help you understand what Pulmonary atresia is, what tests you need, and the implication of being diagnosed for you, your baby, and your family. […] PA-IVS can be associated with important anomalies in the heart and, less frequently, abnormalities in other body parts. In addition, it could be associated with chromosomal and genetic abnormalities. In type I there is a risk in pregnancy that the right ventricle will not develop properly, and in type II there is a risk of fetal heart failure and intrauterine death. […] Prenatal diagnosis of a PA-IVS involves a detailed fetal ultrasound and an advanced echocardiography (a specialized ultrasound of the baby’s heart during pregnancy) to rule out other associated heart defects or anomalies in other body parts. […] All patients with a prenatal diagnosis of PA-IVS should be born in a centre with high-risk pregnancy specialists, access to pediatric heart specialists, and an intensive care nursery.

• #7 Prenatal diagnosis of pulmonary atresia with intact ventricular septum: a single-center study in China | Cardiovascular Ultrasound | Full Text

  https://cardiovascularultrasound.biomedcentral.com/articles/10.1186/s12947-025-00348-0

  Fetal echocardiography is an effective tool for diagnosing PA/IVS. […] Given the progressive nature of PA/IVS in utero, sequential ultrasound examinations during the second and third trimesters are essential for monitoring disease progression and hemodynamic changes. […] Additionally, a comprehensive evaluation for associated intracardiac and extracardiac anomalies should be systematically conducted throughout the pregnancy.

• #8

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  A baby with pulmonary atresia doesn’t get enough oxygen in the blood. Urgent treatment is needed. Treatment for pulmonary atresia with intact ventricular septum may include a mix of medicines, procedures or surgery to fix the heart. […] Diagnosis […] Tests used to diagnose pulmonary atresia with intact ventricular septum (PA/IVS) include: […] Pulse oximetry. A small sensor attached to a hand or foot measures the amount of oxygen in the blood. Pulse oximetry is simple and painless. […] Electrocardiogram (ECG or EKG). This simple test measures the electrical activity of the heart. It shows how the heart is beating. Sticky patches with sensors, called electrodes, are attached to the chest and sometimes to the arms or legs. The sensors connect to a monitor, which prints or displays results.

• #9

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  Chest X-ray. A chest X-ray shows the shape and size of the heart and lungs. […] Echocardiogram. Sound waves are used to create pictures of the heart in motion. An echocardiogram shows how blood moves through the heart and heart valves, including the pulmonary valve. An echocardiogram can be done during pregnancy is called a fetal echocardiogram. It can diagnose pulmonary atresia in the baby. […] Cardiac catheterization. A doctor places a thin tube called a catheter through a blood vessel, usually in the groin. It’s guided to the heart. Dye flows through the catheter and into the heart arteries. The dye helps the arteries show up more clearly on X-rays. […] Treatment […] Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart problems present at birth.

• #10 Pulmonary Atresia With Intact Ventricular Septum – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/sites/books/NBK546666/?report=reader

  Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. This action is vital for the preoperative survival of these patients, as the ductus arteriosus is the sole source of pulmonary blood flow. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] An interprofessional approach is crucial for achieving optimal outcomes in patients with PA-IVS. The healthcare team typically includes a pediatric cardiologist, a cardiothoracic surgeon, an obstetrician, a maternal-fetal specialist for fetal diagnosis, a neonatal-perinatal specialist, neonatal and intensive care unit nurses, and a cardiac intensivist. […] Clinicians collaborate closely with families to ensure optimal care, offering counseling and answering questions.

• #11 Pulmonary Atresia with Intact Ventricular Septum (IVS) | Texas Children’s

  https://www.texaschildrens.org/content/conditions/pulmonary-atresia-with-intact-ventricular-septum-ivs

  Treatment is required after birth to improve blood flow to the lungs and meet the baby’s oxygen needs. A medication to prevent the closure of the ductus arteriosus, called prostaglandins (PGE), is started as soon as the baby is born. […] Babies born with pulmonary atresia with intact ventricular septum require lifelong monitoring by cardiologists experienced in the treatment of congenital heart defects.

• #12

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  Treatment for pulmonary atresia with intact ventricular septum (PA/IVS) may include medicines and one or more procedures or surgeries. […] Medications […] Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it gives the care team more time to determine the best type of surgery or procedure for the baby. […] Surgeries or other procedures […] A baby with pulmonary atresia with intact ventricular septum (PA/IVS) most often needs one or more surgeries or procedures to improve blood flow and fix the heart. […] Some of these treatments are done in the first days to weeks of a baby’s life. Others are done later. The type of surgery or procedure depends on many things. These include the size of the right lower heart chamber and heart valves, and whether the baby has other heart problems.

• #13 Pulmonary Atresia With Intact Ventricular Septum | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28009

  Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure. Due to the complexity and heterogeneity of this disorder, no single method is effective for all patients. Even though biventricular repair is the ideal and preferred surgical approach, managing this disorder must be highly individualized. […] An interprofessional approach is crucial for achieving optimal outcomes in patients with PA-IVS. The healthcare team typically includes a pediatric cardiologist, a cardiothoracic surgeon, an obstetrician, a maternal-fetal specialist for fetal diagnosis, a neonatal-perinatal specialist, neonatal and intensive care unit nurses, and a cardiac intensivist.

• #14 Pulmonary Atresia and Intact Ventricular Septum – Causes & Treatment

  https://ssmc.ae/doctors-specialities/pulmonary-atresia-and-intact-ventricular-septum/

  Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart defect that occurs in less than 1% of all heart defects. […] Management strategies for PAIVS are highly individualized and depend on various factors, including the size and function of the right ventricle and tricuspid valve, as well as coronary artery anatomy. […] Initial Treatment: Prostaglandin Infusion: Administered to maintain ductal patency, this treatment is critical for survival before surgical intervention. […] Early diagnosis and tailored management strategies are crucial for improving outcomes in affected infants. […] For comprehensive care and management of PAIVS, Sheikh Shakhbout Medical City (SSMC) offers specialized pediatric cardiology services designed to address these unique needs effectively.

• #15

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  Treatments in the first days to weeks of life […] Balloon valvotomy. This treatment is done to fix a pulmonary valve with a narrowed opening. The surgeon inserts a long, thin tube called a catheter into a blood vessel in the baby’s groin and guides it to the heart. A balloon on the tip of the catheter is inflated to widen the valve opening. The balloon is deflated, and the catheter and balloon are removed. […] Balloon atrial septostomy. A balloon is used to widen the natural hole, called the foramen ovale, in the wall between the upper two chambers of the heart. This hole usually closes shortly after birth. Making the hole larger increases the amount of blood available to travel to the lungs. […] Stenting. The doctor places a rigid tube called a stent in the natural connection between the aorta and pulmonary artery. This connection is called the ductus arteriosus. It usually closes soon after birth. Keeping it open allows blood to travel to the lungs.

• #16 Pulmonary Atresia with Intact Ventricular Septum (PA IVS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia-intact-ventricular-septum-pa-ivs

  Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart defect where the pulmonary valve does not form properly, blocking (obstructing) blood flow from the hearts right pumping chamber (ventricle) to the lungs. There is also no opening or connection (intact septum) between the right and left ventricles. This combination results in not enough blood flowing to the lungs, which means not enough oxygenated blood reaches the body. […] Treatment for pulmonary atresia with intact ventricular septum depends on each child’s heart anatomy. Sometimes, PA/IVS can be treated with a cardiac catheterization technique called balloon valvuloplasty. In this procedure, an interventional cardiologist will insert a thin tube (catheter) through a vein in the leg to reach the heart. With the catheter, the doctor can create a hole in the closed-off pulmonary valve. Then, another catheter, which has a balloon on the end of it, is inserted. The interventional cardiologist will briefly inflate the balloon to open up the valve, then deflate the balloon and remove the catheter.

• #17

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  Shunting. This surgery creates a new path from the main blood vessel leading out of the heart, called the aorta, to the pulmonary arteries. The path is called a bypass or shunt. It lets enough blood go to the lungs. An example of this treatment is the Blalock-Taussig shunt procedure. It may be needed in the first few days of life to increase blood flow to the lungs. But babies usually outgrow this shunt within a few months. […] Later surgical procedures […] Glenn procedure. This surgery most often is done between 4 and 6 months of age. The surgeon connects one of the large veins that returns blood to the heart to the pulmonary artery. Another large vein keeps blood flowing to the right side of the heart. The heart then pumps blood through the pulmonary valve that’s been repaired. This can help the lower right heart chamber grow. If this surgery is done without using a heart-lung machine, it’s called an off-pump Glenn procedure.

• #18

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/pulmonary-atresia-with-intact-ventricular-septum

  Fontan procedure. If the lower right lower heart chamber stays too small to do its work, surgeons may make a new path for blood to flow. The pathway lets most, if not all, of the blood coming to the heart to go into the pulmonary artery. The Fontan procedure is most often done when a child is 2 or 3 years old. […] Hybrid procedures. These are surgical and catheter treatments that are done at the same time. They’re sometimes done without a heart-lung machine. […] After treatment, babies with pulmonary atresia should have regular health checkups, ideally with a pediatric heart doctor. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with pulmonary atresia with intact ventricular septum (PA/IVS) live into adulthood. Adults with PA/IVS should be followed by a doctor with specialized training in adult congenital heart disease.

• #19 Pulmonary Atresia with intact ventricular septum | Little Hearts Matter

  https://www.lhm.org.uk/pulmonary-atresia/

  Pulmonary Atresia with intact ventricular septum is a congenital heart condition (a problem that a baby is born with). […] To give the baby a chance to recover from the birth and for the doctors and parents to discuss and plan treatment it is important to maintain the fetal blood flow described earlier. Medical teams will start an infusion of Prostaglandin, a drug that mimics the mothers hormones, after birth to slow the closure of the fetal duct. […] It is vital that parents are fully included in the treatment discussions and decisions taken for their child. Parents are their childs advocates until they reach an age when they can be involved in discussions about their own treatment. […] The aim of surgical treatment is to re-route the blood through the right side of the heart, bypassing the blockages on the left side. This surgery is offered in three stages over three or more years and involves extremely high-risk, open-heart surgery.

• #20 Pulmonary atresia with intact ventricular septum – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia-intact-ventricular-septum/cdc-20396714

  A baby with pulmonary atresia with intact ventricular septum needs one or more surgeries or procedures to improve blood flow and fix the heart. […] After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children. This type of healthcare professional is called a pediatric cardiologist.

• #21 Pulmonary atresia with intact ventricular septum | UM Health-Sparrow

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

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, is a rare heart condition that’s present at birth. Treatment for pulmonary atresia with intact ventricular septum may include medicines and one or more procedures or surgeries to fix the heart. Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart conditions present at birth. Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it keeps the baby stable until a surgery or procedure can be done to permanently fix blood flow. A baby with pulmonary atresia with intact ventricular septum needs one or more surgeries or procedures to improve blood flow and fix the heart. After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children. […] Due to advances in treatment and technology, many people with PA/IVS live into adulthood. Adults with the condition should be followed by a doctor with training in adult congenital heart diseases.

• #22 Pulmonary Atresia with Intact Ventricular Septum (PA IVS) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-atresia-intact-ventricular-septum-pa-ivs

  Children who have had surgery to repair pulmonary atresia with intact ventricular septum need lifelong care by a cardiologist, which will include ongoing diagnostic evaluations, medications to manage symptoms and prevent complications, lifestyle modifications to support cardiovascular health, and emotional and psychosocial support.

• #23 SSA – POMS: DI 23022.585 – Pulmonary Atresia – 08/28/2020

  https://secure.ssa.gov/poms.nsf/lnx/0423022585

  Pulmonary atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. […] Infants born with this condition also tend to have multiple other cardiac conditions, such as poorly developed tricuspid valves, underdeveloped (hypoplastic) right ventricle, and abnormal blood vessels leading into the heart. Surgery is necessary shortly after birth to form a shunt from the systemic circulation to the pulmonary circulation in order to oxygenate the blood. […] Poor feeding during nursing; […] Infants are given medication (i.e. Prostaglandin) to help keep the blood vessel open between the pulmonary artery and the aorta (patent ductus arteriosis or PDA) until the initial shunt surgery can occur. […] Infants surviving into adulthood should be monitored by a cardiologist specializing in the care of adults with congenital heart disease to assess medication needs, surgery, and infection throughout their lifetime.

• #24 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. […] 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. […] Here are some tips for caring for someone with pulmonary atresia after coming home from the hospital: Go to scheduled health checkups. 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. […] Talking with other parents who have a child with a congenital heart defect might give you comfort and support. Ask a member of your child’s care team about local support groups.

• #25 Conditions – Leeds Congenital Hearts

  https://leedscongenitalhearts.com/child/conditions/view/2/50/pulmonary-atresia-with-intact-ventricular-septum

  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 improve the blood supply to the lungs […] The type of operation needed will depend upon the exact abnormality present. […] In these cases the blood supply to the lungs can be helped by a shunt operation. […] 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 or fluid collecting around the lungs. […] All children with pulmonary atresia who have had a shunt operation as a baby will need further treatment when they are older. […] Some children with heart disease cannot manage as much physical exercise as a normal child. […] All patients with pulmonary atresia will be at risk of infection in the heart (called endocarditis), both before and after surgery.

• #26 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 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.

• #27 Pulmonary Atresia with intact ventricular septum | Little Hearts Matter

  https://www.lhm.org.uk/pulmonary-atresia/

  The final stage of surgery will be performed as the child starts to show that they need more blood flow to the lungs. […] The MCT (Medium Chain Triglycerides) diet is a special diet which is sometimes required by children for a short period of time in hospital after heart or chest surgery or for a short period of time at home. […] The outcomes of treatment for right-sided single ventricle conditions are good. Surgery has been available to treat these conditions for over forty years. Over that time we have seen the success of treatment improve. […] Many children require other procedures to improve their heart function as they get older. […] A very important part of the treatment of complex congenital heart disease is the medical support given to the heart and circulation. This support will be altered as the needs of each child and young adult change as they undergo surgery and grow. […] When doctors discuss a new babys diagnosis and the treatment path that they plan for them to travel, they also discuss the risks that go hand in hand with a complex heart problem and the treatments available.

• #28 Pulmonary atresia with intact ventricular septum | PPT

  https://www.slideshare.net/slideshow/pulmonary-atresia-with-intact-ventricular-septum/30908914

  Pulmonary atresia with intact ventricular septum (PAIVS) is a congenital heart defect where the pulmonary valve is blocked, preventing blood flow from the right ventricle to the lungs. It occurs in 1-3% of congenital heart diseases. Surgical interventions for PAIVS have improved, with 5-year survival rates now around 80%. Treatment depends on factors like the size of the tricuspid valve and whether the coronary arteries depend on blood flow from the right ventricle. Options include biventricular repair, univentricular repair, or transplantation. […] PA/IVS is fatal 50 % die within two weeks of birth 85 % by six months PA/IVS is a ductal-dependent lesion, closure of the patent ductus arteriosus (PDA) generally results in rapid clinical deterioration and life-threatening consequences, including severe metabolic acidosis and hypoxemia, seizures, cardiogenic shock, cardiac arrest, and death. Rarely, prolonged survival can occur with pulmonary blood flow maintained by a persistent PDA or systemic artery to pulmonary artery blood flow via one or more collateral blood vessels.

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