Materiały źródłowe

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

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  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. […] PA-IVS is a rare and varied cardiac anomaly, occurring in 4 to 5 out of every 100,000 live births. […] Although outcomes have improved over time, they remain cautious, with reported survival rates of 70% to 75% at 1 year and 63% to 67% at 5 years, depending on the type of postnatal circulation. […] A collaborative study in the United Kingdom and Eire published in 1998 reported an incidence of approximately 4 per 100,000 live births for this disorder. […] In another study published in the United States in 1984, the incidence of PA-IVS was approximately 8 per 100,000 live births.

• #2 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. […] PA-IVS is 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. […] PA-IVS is a rare and varied cardiac anomaly, occurring in 4 to 5 out of every 100,000 live births. […] A collaborative study in the United Kingdom and Eire published in 1998 reported an incidence of approximately 4 per 100,000 live births for this disorder.

• #3 Pulmonary Atresia with Intact Ventricular Septum | Radiology Key

  https://radiologykey.com/pulmonary-atresia-with-intact-ventricular-septum-2/

  Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart defect with no known genetic etiology. The prevalence of PA ranges from 4.1 to 8.3 per 100,000 live births and accounts for approximately 3% of serious congenital heart defects. Males and females are affected equally. Multifactorial inheritance suggests a risk of recurrence of 3% to 5%. […] At initial encounter of a fetus with PA/IVS, prognosis and counseling hinge on assessment of the anatomy as being most favorable for a two-ventricle (biventricular) repair strategy or a single-ventricle palliative strategy. […] Once the diagnosis is established, serial fetal echocardiography should be performed with focus on the features (see Key Features) as well as those in the previously discussed, published criteria in order to survey for findings that may change the prognosis from a two-ventricle to a single-ventricle strategy. […] Most fetuses with PA/IVS have no significant problems in utero. However, the presence of extensive fistulous communications places the fetus at risk for ischemia, arrhythmia, and sudden death in utero.

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

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

  In another study published in the United States in 1984, the incidence of PA-IVS was approximately 8 per 100,000 live births. […] With the widespread availability and use of fetal echocardiography, the number of patients with complex forms of congenital heart disease, including PA-IVS, being diagnosed during pregnancy across the world is increasing. […] PA-IVS has no preference for either sex. […] The genetic evaluation of twins in the report was notable for a 55 kb deletion at WFDC8 and WFDC9; however, the clinical significance of this gene deletion is unknown. […] Several studies have demonstrated a gradual improvement in the survival rates of patients with this disorder, attributed to advancements in pediatric cardiology and cardiothoracic surgery. […] A population-based study from the United Kingdom and Ireland, published in 2005, reported 1-year survival rates of approximately 71% and 5-year survival rates of about 64%.

• #5 Type I and II pulmonary atresia with intact ventricular septum in infants: a 10-year experience in initial surgery at one center | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-022-02549-1

  Pulmonary atresia with intact ventricular septum (PA/IVS) is a type of complex cyanotic congenital heart disease with a low incidence, accounting for only 1% to 3% of congenital heart disease (CHD); however, it is a critically ill condition with high mortality. […] The reasonable choice of the initial surgery method is the key to improving the survival rate, which is also an important factor affecting the prognosis. […] Early active surgical treatment of PA/IVS is the key to improving the survival rate, and staged decompression is relatively stable, even if this may increase the rate of postoperative reintervention. The reasonable choice of the initial surgical strategy is an important factor affecting the prognosis. Taken together, BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve, and BT shunt combined with PDA ligation, RVOT incision, transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation, RVOT incision and transannular patch.

• #6 Pulmonary Atresia with Intact Ventricular Septum (PA-IVS): Definition, Diagnosis, and Treatment

  https://angolodeldottorino.it/en/Medicine/Cardiology/Congenital_Heart_Diseases/Pulmonary_Atresia_with_Intact_Ventricular_Septum_(PA-IVS).php

  Pulmonary atresia with an intact ventricular septum has an estimated incidence of about 4-5 cases per 100,000 live births, accounting for approximately 1-3% of all congenital heart defects. […] There is no significant sex predilection. […] However, it is frequently associated with coronary anomalies, such as coronary artery fistulae connecting to the right ventricle or coronary circulation dependent on a hypoplastic right ventricle, which influence prognosis and treatment.

• #7 Pulmonary Atresia and Intact Ventricular Septum | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-and-intact-ventricular-septum-3/

  Pulmonary atresia and intact ventricular septum was first described in 1783 by Hunter and then revisited 86 years later in 1869 by Peacock. Epidemiology Data obtained from the New England Regional Infant Cardiac Program identified 75 patients with this disorder, accounting for 3.1% of all infants enrolled in the study. The Baltimore-Washington Infant Study defined the prevalence for this disorder as 0.083/1,000 live births. Despite being uncommon, when integrated into the overall statistics of congenital heart disease, pulmonary atresia and intact ventricular septum is one of the more common types of cyanotic congenital heart disease in the neonate, along with transposition of the great arteries and pulmonary atresia with ventricular septal defect. A published study from the United Kingdom and Eire defined the incidence of pulmonary atresia and intact ventricular septum to be 4.1/100,000 live births. Overall, by the best estimates pulmonary atresia and intact ventricular septum occurs at a rate of 0.6/10,000 live births. However, if one includes pregnancies that are spontaneously or electively aborted after 20 weeks gestational age with live births the rate has been reported as much as 10 times higher at 0.6/1,000. Fetal echocardiography provides a unique window to study the later phases of the fetal cardiac development recognizing that cardiac organogenesis is complete by about 8 weeks of gestation. There is increasing evidence that fetuses with severe tricuspid regurgitation may not fare well. Such fetuses are known to develop right-sided heart failure with pleural and pericardial effusions, ascites, pulmonary hypoplasia, and fetal death. Thus, fetal loss might be anticipated in a specific subset of patients with pulmonary atresia, intact ventricular septum, extremely severe tricuspid regurgitation, and a low-pressure right ventricle. The data collected from the United Kingdom and Eire also showed that termination of pregnancy once this diagnosis was established led to an important reduction in live-born incidence in mainland Britain. Prenatal echocardiography is becoming an important predictor of postnatal management of this patient population and will likely grow to include strategic application of in utero intervention.

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

  https://www.ncbi.nlm.nih.gov/books/NBK546666/

  With the widespread availability and use of fetal echocardiography, the number of patients with complex forms of congenital heart disease, including PA-IVS, being diagnosed during pregnancy across the world is increasing. […] PA-IVS has no preference for either sex. […] In addition, no association with genetic disorders has been identified, although De Stefano et al reported PA-IVS in monozygotic twins.

• #9 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 rare congenital heart defect. It occurs at a rate of less than one per 10,000 live births. […] Pulmonary atresia with intact ventricular septum can be detected during pregnancy through a routine fetal echocardiogram (ultrasound of the fetal heart). A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and cardiac expertise your baby will need at birth, optimizing outcomes. […] 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). […] Babies born with pulmonary atresia with intact ventricular septum require lifelong monitoring by cardiologists experienced in the treatment of congenital heart defects.

• #10

  https://link.springer.com/article/10.1007/s00404-020-05929-0

  Pulmonary atresia with intact ventricular septum (PAIVS) is a rare, heterogenous cardiac anomaly with an incidence of 45/100,000 live births. Outcomes have improved over time, but are still guarded with reported one- and 5-year survival rates of 70-75% and 63-67%, depending on the type of postnatal circulation. While in Germany prenatal detection rate for congenital heart defect (CHD) is low with 20% for all CHD and about 42% for severe CHD, in UK two-thirds of cases with PAIVS/critical pulmonary stenosis (CPS) are detected prenatally. The rate of termination of pregnancy (TOP) in PAIVS/CPS is up to 60%, especially when univentricular (UV) outcome is supposed. Therefore, prediction of circulation outcome is an important aspect for parental counselling. […] The aim of our study was to describe outcomes in prenatally diagnosed PAIVS/CPS, to analyse foetal echocardiographic parameters for predicting BV outcome and growth rate of these during gestation in the largest cohort to date.

• #11 Pulmonary Atresia With Intact Ventricular Septum | Thoracic Key

  https://thoracickey.com/pulmonary-atresia-with-intact-ventricular-septum-5/

  Fetal loss may account for an underestimated incidence and prevalence of the defect, which is estimated at 0.6/10,000 live births. […] Consequently, the real incidence of this defect is speculated as being up to ten times higher. […] Because of this wide spectrum and variability in disease severity, mainly related to tricuspid valve size, RV chamber anatomy, and coronary artery anomalies, complex algorithms for treatment are required to ensure optimum outcomes for these often fragile patients. […] These algorithms have resulted in improved survival in patients diagnosed with PA/IVS, particularly in the more severe forms of the spectrum. […] PA/IVS can be diagnosed in utero and this can help with educating the family and preparing the medical and surgical team for eventual treatment options, including having the baby delivered at a center where prostaglandins can be started and transfer to a cardiac center initiated.

• #12 Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) | Pediatric Echocardiography

  https://pedecho.org/library/chd/pa-ivs

  Pulmonary atresia with intact ventricular septum (PA/IVS) is characterized by atresia of the pulmonary valve. The pulmonary valve is imperforate by way of a membranous or thicker muscular atresia. The ventricular septum is intact and pulmonary blood flow is dependent on a patent ductus arteriosus. PA/IVS is found in about 6 to 8.3 in 100,000 live births. This number is thought to be an underestimation due to the frequency of fetal or in utero termination. […] There is no sex predilection or identifiable genetic cause known at this time. […] The hemodynamics can differ greatly depending on the specific constellation of anatomic variance. There will be progressive cyanosis in the early neonatal period. They will typically be a murmur related to the degree of tricuspid valve regurgitation as well as a ductal murmur. These patients will need prostaglandin infusion in the neonatal period to ensure patency of the patent ductus arteriosus in order to maintain adequate pulmonary blood flow.

• #13 Pulmonary Valve Atresia | NCBDDD | CDC

  https://archive.cdc.gov/www_cdc_gov/ncbddd/birthdefects/surveillancemanual/quick-reference-handbook/pulmonary-valve-atresia.html

  Pulmonary atresia with intact ventricular septum is often isolated but can be associated with unrelated anomalies and syndromes as well as with other intracardiac anomalies, especially those that involve the right side of the heart. […] Pulmonary atresia with ventricular septal defect can be associated with deletion 22q11, unlike the form with intact ventricular septum. Because of this association, look for other birth defects, including cleft palate and internal anomalies.

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

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

  PA-IVS has no preference for either sex. […] In addition, no association with genetic disorders has been identified, although De Stefano et al reported PA-IVS in monozygotic twins. […] The genetic evaluation of twins in the report was notable for a 55 kb deletion at WFDC8 and WFDC9; however, the clinical significance of this gene deletion is unknown. […] Similarly, Chitayat et al reported the incidence of PA-IVS in 2 siblings with no other associated cardiac anomalies. […] Regarding the timing of occurrence, Kusche and Van Mierop suggested that the insult leading to PA-IVS occurs later in gestation compared to pulmonary atresia with ventricular septal defect (PA-VSD). […] They indicated that the insult leading to PA-VSD occurs before the complete formation of the ventricular septum, whereas PA-IVS occurs following the completion of ventricular septal formation.

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

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

  PA-IVS is a rare and varied cardiac anomaly, occurring in 4 to 5 out of every 100,000 live births. […] Although outcomes have improved over time, they remain cautious, with reported survival rates of 70% to 75% at 1 year and 63% to 67% at 5 years, depending on the type of postnatal circulation. […] A collaborative study in the United Kingdom and Eire published in 1998 reported an incidence of approximately 4 per 100,000 live births for this disorder. […] In another study published in the United States in 1984, the incidence of PA-IVS was approximately 8 per 100,000 live births. […] With the widespread availability and use of fetal echocardiography, the number of patients with complex forms of congenital heart disease, including PA-IVS, being diagnosed during pregnancy across the world is increasing.

• #15

  https://omim.org/entry/265150

  Pulmonary atresia with intact ventricular septum accounts for less than 3% of all congenital heart defects (Grossfeld et al., 1997). […] Grossfeld et al. (1997) suggested autosomal dominant inheritance with incomplete penetrance. […] De Stefano et al. (2008) reported monozygotic twin sisters with pulmonary valve atresia and intact ventricular septum.

• #16 Pulmonary atresia with intact ventricular septum epidemiology and outcome in children born in Sweden 1980-1999

  https://gupea.ub.gu.se/handle/2077/10019

  Pulmonary atresia with intact ventricular septum epidemiology and outcome in children born in Sweden 1980-1999 […] Aims: To describe children born with pulmonary atresia with intact ventricular septum (PA-IVS) in Sweden between 1980 and 1999, the incidence and outcome of PA-IVS, to examine cardio-pulmonary outcomes in survivors and to evaluate their quality of life. […] Results: The incidence was 4.2/100, 000 live births. […] The survival rate was 68% ten years after initial surgery. […] Overall quality of life was similar to that of a healthy control group, but subjects with PA-IVS reported more psychosomatic symptoms. […] Summary: PA-IVS is an unusual and heterogeneous congenital heart defect associated with high mortality during the first years of life. Membranous pulmonary atresia was associated with a better outcome than muscular pulmonary atresia with respect to survival, myocardial perfusion defects and exercise capacity. The majority of the survivors had biventricular repair. Overall quality of life was good.

• #17 Pulmonary atresia with intact ventricular septum | PPT

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

  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. […] Surgical interventions are improving with a 5-year survival rate of approximately 80%.

• #18 Pulmonary atresia with intact ventricular septum (PA/IVS) – UpToDate

  https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs

  Pulmonary atresia with intact ventricular septum (PA/IVS) is characterized by complete obstruction to right ventricular (RV) outflow with varying degrees of RV and tricuspid valve (TV) hypoplasia. Blood is thus unable to flow from the RV to the pulmonary artery and lungs, and an alternative source of pulmonary blood flow is required for survival. If untreated, PA/IVS is almost always fatal. Outcomes of surgical interventions are improving, with a five-year survival rate of approximately 80 percent. […] PA/IVS is a rare congenital cardiac defect that consists of atresia of the pulmonary valve resulting in an absent connection between the right ventricular outflow tract (RVOT) and pulmonary arteries as well as an IVS that allows no connection between the right and left ventricles. […] PA/IVS features developmental abnormalities of the RV and TV that are „upstream” of pulmonary outflow. The pulmonary arteries may be small, but their architecture and branching patterns are usually otherwise normal.

• #19 18: Pulmonary Atresia with Intact Ventricular Septum | Veterian Key

  https://veteriankey.com/18-pulmonary-atresia-with-intact-ventricular-septum/

  Pulmonary atresia with intact ventricular septum (PA/IVS) is a relatively uncommon congenital heart defect characterized by luminal discontinuity between the right ventricular outflow tract and the main pulmonary artery in the absence of a ventricular septal defect. […] In the New England Regional Infant Cardiac Program, 75 of 2251 infants (3.3%) had PA/IVS, and 85% of these were diagnosed in the first week of life. […] Estimates of the incidence of PA/IVS vary from one in 8000 live births to one in 144,000 live births. […] Without treatment, 50% of infants die within 2 weeks, and 85% die by 6 months of age. […] Death is typically due to complications of cyanosis secondary to insufficient pulmonary blood flow.

• #20 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

  Treatment for pulmonary atresia with intact ventricular septum depends on each child’s heart anatomy. […] 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. […] With advancements in medical and surgical treatments for congenital heart disease, the prognosis for children with PA/IVS has greatly improved.

• #20 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. […] PA/IVS can also impact how the coronary arteries (the blood vessels that supply oxygen-rich blood to the heart muscle) form before birth. […] Pediatricians from other hospitals often refer newborns to the Cardiac Center at Childrens Hospital of Philadelphia (CHOP) when they notice these symptoms. […] Pulmonary atresia with intact ventricular septum may be diagnosed before birth with a fetal ultrasound, called an echocardiogram. […] Diagnosing pulmonary atresia with intact ventricular septum may require some or all of these tests: Echocardiogram (also called echo or ultrasound), which uses sound waves to create an image of the heart; Electrocardiogram (ECG), which takes a record of the electrical activity of the heart; Pulse oximetry, a noninvasive way to monitor the oxygen content of the blood; Chest X-ray; Cardiac CT or cardiac MRI, which allows doctors to see a 3D image of the hearts structures in detail; Cardiac catheterization, which is when a thin tube (catheter) is inserted into the heart through the large veins or arteries in the leg to take measurements throughout the heart.

• #21 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. […] Pulmonary atresia with intact ventricular septum, also called PA/IVS, is often found during pregnancy or soon after birth. […] The cause of pulmonary atresia with intact ventricular septum, also called PA/IVS, is not clear. […] What increases the risk of pulmonary atresia with intact ventricular septum, also called PA/IVS, is not well known. […] Pulmonary atresia with intact ventricular septum, also called PA/IVS, can be diagnosed before birth with an ultrasound during a routine pregnancy screening. Or it may be diagnosed soon after the baby is born. […] Treatment for pulmonary atresia with intact ventricular septum, also called PA/IVS, may include medicines and one or more procedures or surgeries. […] 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.

• #22 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

  Pulmonary atresia with intact ventricular septum encompasses a group of cardiac anomalies that have in common a complete obstruction of the valve that connects the right ventricle (RV) and the pulmonary artery that carries blood to the lungs, in the presence of an intact interventricular septum. […] 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.

• #23 Pulmonary Atresia with Intact Ventricular Septum | SpringerLink

  https://link.springer.com/10.1007/978-3-030-42937-9_57-1

  Pulmonary atresia with intact ventricular septum is a cyanotic congenital heart disease characterized by complete obstruction of the right ventricular outflow tract, right ventricular hypoplasia, myocardial hypertrophy, and deranged coronary arteries. […] Advances in fetal imaging have led to improvements in our ability to prognosticate which patients are likely to have functionally single versus biventricular therapeutic pathways. […] In addition, early data on fetal intervention reveals a potential to promote the growth of the right-sided structures. […] Postnatally, our understanding of the anatomical and physiological factors has resulted in a tailored approach to patient care, with improved survival. […] Angiographic assessment of the coronary arteries has long been a key diagnostic tool, and transcatheter interventions are becoming more refined, with resulting improvement in outcomes.

• #24 Pulmonary Atresia | Boston Children’s Hospital

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

  Pulmonary atresia is a life-threatening condition, affecting one out of every 10,000 newborns. […] Babies with pulmonary atresia need some type of treatment soon after birth. The specific type of treatment your baby needs will depend on the severity of his or her condition. […] A cardiac catheterization is often performed to determine how blood is supplied to the heart in patients with pulmonary atresia with intact ventricular septum. Using this test, doctors can tell if the blood supply to the heart is dependent upon flow directly from the right ventricle through abnormal coronary connections, called fistulae. If the blood supply is dependent upon the right ventricle, surgery is recommended. […] Many children with pulmonary atresia need one or more surgeries to fix the problem.

• #25 Pulmonary Atresia: Symptoms, Causes and Treatment

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

  Pulmonary atresia life expectancy varies depending on how severe your childs condition is and other individual factors. Survival rates are better today than they were in previous decades. […] Without having surgery to fix pulmonary atresia with a ventricular septal defect, the survival rate is 50% at age 1 and 8% at 10 years of age. Most people dont live into their 30s without surgery. […] People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. They may have appointments at least every six months.

• #25 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 occurs in about one out of 10,000 live births. […] Genetic factors, such as an abnormal gene or chromosomal defect, may increase the chances of heart defects in certain families. Some children with genetic disorders like DiGeorges syndrome or velocardiofacial syndrome may be at greater risk for pulmonary atresia. […] Complications of pulmonary atresia may include: Delays in growth, Abnormal heart rhythms, Heart failure, Liver disease. […] It is also very important to monitor for complications after surgical repair. Fontan circulation is not normal, so your child will need close lifelong follow up. […] 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 babys birth, they can treat your newborn to improve their oxygen circulation.

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

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022585

  Pulmonary atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. […] Pulmonary atresia may occur with or without a hole in the wall (septum) that separates the right ventricle and the left ventricle. If the infant does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA-IVS). […] 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. […] A diagnosis of pulmonary atresia is usually made shortly after birth. Disease progression is variable based on the severity of the congenital heart defect and the response to medication and surgical interventions. […] Infants surviving into childhood and adults may develop problems with their heart functioning later in life due to congestive heart failure, angina, arrhythmias, cyanosis, and sudden death.

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