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. […] The most prevalent presenting signs and symptoms of PA-IVS include cyanosis and desaturation. Neonates with PS-IVS become symptomatic following the closure of the ductus arteriosus, as their pulmonary circulation is dependent on it. […] The presence of low cardiac output syndrome should raise suspicion for myocardial ischemia, especially in patients with coronary fistulae.

• #2 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.

• #3 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. […] Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include: Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away.

• #4 Pulmonary atresia: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001091.htm

  Symptoms most often occur in the first few hours of life, although it may take up to a few days. […] Symptoms may include: […] Bluish color to the skin (cyanosis) due to low oxygen level in the blood […] Fast breathing […] Fatigue […] Poor eating habits (babies may get tired while nursing or sweat during feedings) […] Shortness of breath.

• #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. […] Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include: Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away.

• #6 Pulmonary atresia – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia/symptoms-causes/syc-20350727

  Symptoms of pulmonary atresia may be seen soon after birth. They can include: […] Blue or gray skin, lips or fingernails due to low oxygen levels. Depending on the skin color, these changes may be harder or easier to see. […] Fast breathing or shortness of breath. […] Tiring easily. […] Not feeding well. […] 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. […] If there’s no VSD, the right lower heart chamber gets little blood flow before birth. The chamber often doesn’t form fully. This is a condition called pulmonary atresia with intact ventricular septum (PA/IVS).

• #7 About Pulmonary Atresia | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/pulmonary-atresia.html

  Babies born with pulmonary atresia will show symptoms at birth or very soon afterwards. They may have a bluish looking skin color, called cyanosis, because their blood doesn’t carry enough oxygen. Infants with pulmonary atresia can have additional symptoms such as: […] Problems breathing […] Ashen or bluish skin color […] Poor feeding […] Extreme sleepiness. […] A baby with pulmonary atresia may need surgery or other procedures soon after birth. Therefore, pulmonary atresia is considered a critical congenital heart defect (critical CHD). […] Most babies with pulmonary atresia will need routine checkups with a heart doctor. A heart doctor will monitor a baby’s progress and check for other health conditions that might develop as they get older. As adults, they may need more surgery or medical care for other possible problems.

• #8 Pulmonary atresia – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-atresia/symptoms-causes/syc-20350727

  Symptoms of pulmonary atresia may be seen soon after birth. They can include: […] Blue or gray skin, lips or fingernails due to low oxygen levels. Depending on the skin color, these changes may be harder or easier to see. […] Fast breathing or shortness of breath. […] Tiring easily. […] Not feeding well. […] 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. […] If there’s no VSD, the right lower heart chamber gets little blood flow before birth. The chamber often doesn’t form fully. This is a condition called pulmonary atresia with intact ventricular septum (PA/IVS).

• #9 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. […] The symptoms of pulmonary atresia with intact ventricular septum may include: Blue or purple tint to lips, skin and nails (cyanosis), Shortness of breath, Difficulty feeding, Fatigue or listlessness, Heart murmur (abnormal heart sounds heard with a stethoscope). […] As single ventricle survivors get older, doctors are recognizing that, while some children do not experience complications, many do, including lung, liver and gastrointestinal complications. […] The outlook can vary depending on how severe the condition is and how well the treatments work. However, many children grow into adulthood, often after having a Fontan procedure and additional treatments over time, and go on to lead fulfilling lives.

• #10 Pulmonary Atresia: Symptoms, Causes and Treatment

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

  Symptoms of pulmonary atresia often appear within the first few hours or days of a baby’s life and may include: […] A bluish tinge to the skin, especially the lips, fingers and toes. […] Breathing problems. […] Fatigue or tiredness. […] Irritability. […] Poor feeding. […] Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation. […] Pulmonary atresia life expectancy varies depending on how severe your child’s 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 don’t live into their 30s without surgery.

• #11 Pulmonary atresia in children – Children’s Health Cardiology

  https://www.childrens.com/specialties-services/conditions/pulmonary-atresia

  Pulmonary atresia is a congenital birth defect where the pulmonary valve that carries blood from the heart to the lungs doesn’t form during development and often requires medical attention soon after birth. […] Symptoms usually occur in the first few hours of life, but can appear within days. […] Cyanosis (bluish discoloration of the skin, fingernails and mouth due to lack of oxygen) […] Fast breathing […] Fatigue (tiredness) […] Poor eating habits […] Shortness of breath […] Heart murmur.

• #12 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. […] Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include: Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. […] If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away.

• #13 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. […] The symptoms of pulmonary atresia with intact ventricular septum may include: Blue or purple tint to lips, skin and nails (cyanosis), Shortness of breath, Difficulty feeding, Fatigue or listlessness, Heart murmur (abnormal heart sounds heard with a stethoscope). […] As single ventricle survivors get older, doctors are recognizing that, while some children do not experience complications, many do, including lung, liver and gastrointestinal complications. […] The outlook can vary depending on how severe the condition is and how well the treatments work. However, many children grow into adulthood, often after having a Fontan procedure and additional treatments over time, and go on to lead fulfilling lives.

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

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

  The presence of low cardiac output syndrome should raise suspicion for myocardial ischemia, especially in patients with coronary fistulae. […] As noted with many other forms of cyanotic congenital heart disease, these patients do not have an improvement in their cyanosis or desaturations with 100% oxygen delivery or failed hyperoxia tests. […] Clinicians also note single first and second heart sounds during cardiac auscultation. A pansystolic murmur is audible at the left lower sternal border if the tricuspid valve regurgitates. […] An additional murmur related to the flow across a patent ductus arteriosus might be audible in affected patients upon examination, especially following the initiation of prostaglandin infusion to maintain ductal patency.

• #15 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. […] 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. Physical examination may reveal single first and second heart sounds, a pansystolic murmur if the tricuspid valve regurgitates, and possibly a murmur from the patent ductus arteriosus. […] The most prevalent presenting signs and symptoms of PA-IVS include cyanosis and desaturation. Neonates with PS-IVS become symptomatic following the closure of the ductus arteriosus, as their pulmonary circulation is dependent on it.

• #16 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. […] In some cases, the condition isn’t diagnosed until after the baby is born. Symptoms in a newborn may include: Bluish tint to the skin, lips and nails (cyanosis) or pale skin, indicating a lack of oxygen in the blood; Difficulty breathing or rapid breathing; Fatigue; Clammy skin; Feeding problems.

• #17 Pulmonary Atresia With Intact Ventricular Septum | SCAI – Seconds Count

  https://www.secondscount.org/condition/pulmonary-atresia-intact-ventricular-septum

  Children with pulmonary atresia with intact ventricular septum (PA/IVS) are born without a functioning pulmonary valve, the valve between the hearts lower right chamber (right ventricle) and the pulmonary artery, which leads to the lungs. […] Lack of blood flow to the right ventricle causes the chamber to develop poorly and become small and ineffective as a pump. […] The resulting mixture of oxygen-poor and oxygen-rich blood is pumped out of the heart through the aorta and to the body. With less oxygen in the blood circulating through the body, the baby may appear blue (cyanotic). […] If the ductus arteriosus closes in a baby with PA/IVS, severe cyanosis will develop because the oxygen-rich blood flowing from the lungs into the circulation is further reduced. Once the ductus arteriosus begins to close, or if the hole in the wall between the two atria becomes smaller, the baby will become quite ill, and immediate medical attention will be required. […] However, after birth, the baby may develop an intense bluish discoloration of the skin. […] The presence of coronary sinusoids may be a risk factor for sudden death.

• #18 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología

  https://www.revespcardiol.org/es-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. […] Pulmonary atresia with intact ventricular septum (PAIVS) is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of any ventricular septal defect. […] The neonate had desaturation and an aortic systolic ejection murmur. […] The neonate suffered low cardiac output which could not be controlled, and died. […] The clinical manifestations in the newborn are cyanosis, hypoxemia, metabolic acidosis, and right and left heart failure. […] The prognosis is usually poor in the absence of treatment, with over 50% mortality at one month. […] Postnatal survival depends on the patency of the ductus arteriosus, which explains the requirement for continuous infusion of prostaglandin-E1; closure of the ductus would lead to death. […] The association of PAIVS with aortic stenosis is rare, with only a few cases reported, either in isolation or as part of HRHS. […] Survival, as in our patient, is unusual, possibly because of left ventricular failure from multifactorial causes.

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

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

  The presence of low cardiac output syndrome should raise suspicion for myocardial ischemia, especially in patients with coronary fistulae. […] As noted with many other forms of cyanotic congenital heart disease, these patients do not have an improvement in their cyanosis or desaturations with 100% oxygen delivery or failed hyperoxia tests. […] Clinicians also note single first and second heart sounds during cardiac auscultation. A pansystolic murmur is audible at the left lower sternal border if the tricuspid valve regurgitates. […] An additional murmur related to the flow across a patent ductus arteriosus might be audible in affected patients upon examination, especially following the initiation of prostaglandin infusion to maintain ductal patency.

• #20 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.

• #21 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología

  https://www.revespcardiol.org/es-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. […] Pulmonary atresia with intact ventricular septum (PAIVS) is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of any ventricular septal defect. […] The neonate had desaturation and an aortic systolic ejection murmur. […] The neonate suffered low cardiac output which could not be controlled, and died. […] The clinical manifestations in the newborn are cyanosis, hypoxemia, metabolic acidosis, and right and left heart failure. […] The prognosis is usually poor in the absence of treatment, with over 50% mortality at one month. […] Postnatal survival depends on the patency of the ductus arteriosus, which explains the requirement for continuous infusion of prostaglandin-E1; closure of the ductus would lead to death. […] The association of PAIVS with aortic stenosis is rare, with only a few cases reported, either in isolation or as part of HRHS. […] Survival, as in our patient, is unusual, possibly because of left ventricular failure from multifactorial causes.

• #22 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 a disease with significant heterogeneity based on multiple anatomic factors including the degree and form of pulmonary atresia, the size of the right ventricle and tricuspid valve (both of which often demonstrate significant degrees of hypoplasia). […] This lesion will typically present in the neonatal period and depending on multiple factors, can be managed with a biventricular repair, single ventricle palliation or, one and a half ventricle pathway or cardiac transplantation (usually reserved for the most severe variants with severe coronary disease and ventricular dysfunction).

• #23 Pulmonary Stenosis, Pulmonary Atresia with Intact Ventricular Septum, and Ductus Arteriosus Constriction | Obgyn Key

  https://obgynkey.com/pulmonary-stenosis-pulmonary-atresia-with-intact-ventricular-septum-and-ductus-arteriosus-constriction/

  PA-IVS is a group of cardiac malformations having in common absent communication between the right ventricle and the pulmonary arterial circulation (pulmonary atresia) in combination with an intact interventricular septum. […] The pulmonary atresia is usually of the membranous type with complete fusion of the valve commissures and a normally developed infundibulum. […] The right ventricular cavity is either hypoplastic with thickened right ventricular myocardium or dilated with significant tricuspid valve regurgitation and a dilated right atrium. […] A hypoplastic right ventricle occurs in the majority of cases. […] One of the major associated findings in hearts with PA-IVS and hypoplastic right ventricles is an anomaly of the coronary circulation, namely, ventriculocoronary arterial communication (VCAC), found in about one-third of cases of PA-IVS.

• #24 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. 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. […] The physical findings for this condition include: Bluish colored skin (cyanosis); Fast breathing; Fatigue; Poor feeding during nursing; Sweating during feeding; and Shortness of breath.

• #25 Pulmonary Stenosis, Pulmonary Atresia with Intact Ventricular Septum, and Ductus Arteriosus Constriction | Obgyn Key

  https://obgynkey.com/pulmonary-stenosis-pulmonary-atresia-with-intact-ventricular-septum-and-ductus-arteriosus-constriction/

  PA-IVS is a group of cardiac malformations having in common absent communication between the right ventricle and the pulmonary arterial circulation (pulmonary atresia) in combination with an intact interventricular septum. […] The pulmonary atresia is usually of the membranous type with complete fusion of the valve commissures and a normally developed infundibulum. […] The right ventricular cavity is either hypoplastic with thickened right ventricular myocardium or dilated with significant tricuspid valve regurgitation and a dilated right atrium. […] A hypoplastic right ventricle occurs in the majority of cases. […] One of the major associated findings in hearts with PA-IVS and hypoplastic right ventricles is an anomaly of the coronary circulation, namely, ventriculocoronary arterial communication (VCAC), found in about one-third of cases of PA-IVS.

• #26 Pulmonary atresia with intact ventricular septum | PPT

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

  Occasionally, infants with PAIVS have shown signs of both right and left ventricular ischemia, likely related to the coronary artery fistulae. Association with atretic, hypoplastic, or obstructed central coronary arteries, called right ventricular dependent coronary circulation (RVDCC), carries a higher risk of morbidity and mortality.

• #27 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. Pulmonary flow is dependent on the ductus arteriosus. Other morphological anomalies are also present. […] The neonate had low oxygen saturation and a systolic murmur originating in the aorta. An echocardiogram showed pulmonary atresia with intact ventricular septum and a bicuspid, thickened, stenotic aortic valve. Low cardiac output ensued and could not be controlled, and the infant died. […] The neonate had desaturation and an aortic systolic ejection murmur. Echocardiogram showed PAIVS and a thickened and stenotic bicuspid aortic valve. The neonate suffered low cardiac output which could not be controlled, and died.

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

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

  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.

• #29 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

• #30 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

• #31 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

• #32 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. […] The symptoms of pulmonary atresia with intact ventricular septum may include: Blue or purple tint to lips, skin and nails (cyanosis), Shortness of breath, Difficulty feeding, Fatigue or listlessness, Heart murmur (abnormal heart sounds heard with a stethoscope). […] As single ventricle survivors get older, doctors are recognizing that, while some children do not experience complications, many do, including lung, liver and gastrointestinal complications. […] The outlook can vary depending on how severe the condition is and how well the treatments work. However, many children grow into adulthood, often after having a Fontan procedure and additional treatments over time, and go on to lead fulfilling lives.

• #33 Brugada Syndrome and Pulmonary Atresia with Intact Interventricular Septum: Fortuitous Finding or New Genetic Connection?

  https://www.mdpi.com/2073-4425/15/5/638

  Brugada syndrome is a rare arrhythmogenic syndrome associated mainly with pathogenic variants in the SCN5A gene. […] Pulmonary atresia with an intact ventricular septum is characterized by the lack of a functional pulmonary valve, due to the underdevelopment of the right ventricle outflow tract. […] Long-term follow-up of PA-IVS patients has shown a high incidence of arrhythmic malignant events, with SCD being one of the causes of late mortality. […] The fact that both pathologies share a preferential pathophysiological location in the RVOT raises questions regarding a more than coincidental connection. […] SCD of arrhythmic etiology is an issue in long-term follow-up of PA-IVS patients, suggesting that some of these patients could have overlapping phenotypes, mainly due to deleterious variants in the SCN5A gene.

• #34 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

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

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

  Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include: […] Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color. […] Fast breathing or shortness of breath. […] Tiredness. […] Poor feeding. […] Pulmonary atresia with intact ventricular septum, also called PA/IVS, is often found during pregnancy or soon after birth. If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away.

• #36 Pulmonary Atresia | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/pulmonary-atresia/

  Pulmonary atresia is a congenital heart malformation in which the valve from the right side of the heart that normally allows the movement of blood from the heart to the lungs does not form during pregnancy. One of every 10,000 babies is born with pulmonary atresia. […] After birth, the signs of the condition include fast breathing or shortness of breath, and ashen or bluish color to the skin, poor feeding, and extreme sleepiness. […] A cardiac catheterization can be done to finalize the diagnosis.

• #37 NeoCardio Lab – Pulmonary Atresia Intact Ventricular Septum

  https://www.neocardiolab.com/congenital-heart-defects/pulmonary-atresia-intact-ventricular-septum

  Pulmonary atresia with intact ventricular septum (PAIVS) is a complex congenital heart defect characterized by pulmonary valvular atresia. […] Patients may have various presentations, with myocardial sinusoids complicating the course of their management. […] Pulmonary atresia with intact ventricular septum is a complex condition where there is dependent ductal pulmonary circulation. […] They will be cyanotic, considering that the right to left shunt is obligatory at the atrial level, introducing deoxygenated blood in the systemic circulation. […] This condition is of particular interest, considering that there is a risk in a subcategory of infants with PAIVS of persistent sinusoids, possibly stealing from the coronary circulation. […] Because of this, these remnant embryological channels (sinusoids) may stay patent and connect the RV cavity to the coronary system.

• #38 Pulmonary Valve Atresia | NCBDDD | CDC

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

  Pulmonary valve atresia is a structural heart anomaly characterized clinically by cyanosis and anatomically by an imperforate pulmonary valve that blocks completely the flow of blood through the right ventricular outflow tract. […] Infants with pulmonary atresia (with or without ventricular septal defect) typically present early in the neonatal period with low oxygen saturation and cyanosis, which worsens over time as the ductus closes. Some infants might also have massive cardiomegaly. […] Because pulmonary atresia causes low blood oxygen saturation, newborn screening with pulse oximetry can help with early detection of these cases.

• #39 Pulmonary atresia with intact ventricular septum | Altru Health System

  https://www.altru.org/health-library/conditions/pulmonary-atresia-with-intact-ventricular-septum

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, is a rare heart condition that’s present at birth. Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include: Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. Pulmonary atresia with intact ventricular septum, also called PA/IVS, is often found during pregnancy or soon after birth. If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away. 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. 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.

• #40 Pulmonary atresia with intact ventricular septum – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-intact-ventricular-septum/

  Pulmonary atresia (uh-TREE-zhuh) is a rare heart problem present at birth (congenital heart defect). […] In pulmonary atresia with intact ventricular septum (PA/IVS), there isn’t a hole between the two pumping chambers of the heart. […] 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 combination of medication, procedures or surgery to correct the congenital heart defect. […] Babies with pulmonary atresia need immediate treatment, preferably at a medical center with surgeons and other health care providers who have experience with complex congenital heart disease. […] Treatment for pulmonary atresia with intact ventricular septum (PA/IVS) may include medications and one or more procedures or surgeries.

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

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

  Clinicians also note single first and second heart sounds during cardiac auscultation. A pansystolic murmur is audible at the left lower sternal border if the tricuspid valve regurgitates. […] An additional murmur related to the flow across a patent ductus arteriosus might be audible in affected patients upon examination, especially following the initiation of prostaglandin infusion to maintain ductal patency. […] Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. […] 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. […] 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. […] RVDCC, especially atresia of the coronary ostia, is an independent risk factor for poor outcomes.

• #42 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. This medication enables blood to continue to flow to the lungs for oxygen until a more permanent treatment strategy is determined. […] Babies born with pulmonary atresia with intact ventricular septum require lifelong monitoring by cardiologists experienced in the treatment of congenital heart defects.

• #43 Pulmonary atresia with intact ventricular septum | Altru Health System

  https://www.altru.org/health-library/conditions/pulmonary-atresia-with-intact-ventricular-septum

  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. A baby with pulmonary atresia with intact ventricular septum 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 conditions.

• #44 The double shunt technique as a bridge to heart transplantation in a patient with pulmonary atresia with intact septum and right ventricular-dependent coronary circulation – JTCVS Techniques

  https://www.jtcvstechniques.org/article/S2666-2507(21)00084-5/fulltext

  Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart defect with a broad spectrum that is associated with varying degrees of tricuspid valve (TV) and right ventricular (RV) hypoplasia and a wide range of coronary abnormalities. The most extreme form is right ventricular-dependent coronary circulation (RVDCC), which is estimated to occur in 3% to 34% of these cases. […] Significant ST changes with continued elevation in her cardiac biomarkers were concerning while she was maintained on prostaglandin therapy. […] The main concern is to stabilize the patient until transplant. […] Despite intensive care unit admission, holding all enteric nutrition, initiation of total parenteral nutrition, inotropic and pressor support (ie, epinephrine and vasopressin), and delivery of increasing sedation, signs of myocardial ischemia increased.

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

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

  The presence of low cardiac output syndrome should raise suspicion for myocardial ischemia, especially in patients with coronary fistulae. […] As noted with many other forms of cyanotic congenital heart disease, these patients do not have an improvement in their cyanosis or desaturations with 100% oxygen delivery or failed hyperoxia tests. […] Clinicians also note single first and second heart sounds during cardiac auscultation. A pansystolic murmur is audible at the left lower sternal border if the tricuspid valve regurgitates. […] An additional murmur related to the flow across a patent ductus arteriosus might be audible in affected patients upon examination, especially following the initiation of prostaglandin infusion to maintain ductal patency.

• #46 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 a disease with significant heterogeneity based on multiple anatomic factors including the degree and form of pulmonary atresia, the size of the right ventricle and tricuspid valve (both of which often demonstrate significant degrees of hypoplasia). […] This lesion will typically present in the neonatal period and depending on multiple factors, can be managed with a biventricular repair, single ventricle palliation or, one and a half ventricle pathway or cardiac transplantation (usually reserved for the most severe variants with severe coronary disease and ventricular dysfunction).

• #47

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. […] The aim of this retrospective study was to compare the long-term morbidity of patients treated with catheter-based therapy with those who underwent heart surgery as the initial intervention for right ventricular decompression. […] The inclusion criterion was decompression of the right ventricle for initial intervention. […] All included patients had a membranous atresia and a bi- or tripartite right ventricle. […] There was no mortality in either group during the entire study period. […] In group A, all attempted perforations with radiofrequency of the pulmonary valve were successful.

• #48

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  16 out of 18 patients reached biventricular circulation as the end result while 2 patients had a one-and-a half ventricle (1,5-ventrice) repair (open pulmonary valve and bidirectional Glenn anastomosis) at the end of the study. […] In group B, 15 out of 16 patients reached biventricular circulation while one patient required univentricular palliation. […] The complication rate differed between the included studies in frequency and in severity.

• #49 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

• #50 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 a disease with significant heterogeneity based on multiple anatomic factors including the degree and form of pulmonary atresia, the size of the right ventricle and tricuspid valve (both of which often demonstrate significant degrees of hypoplasia). […] This lesion will typically present in the neonatal period and depending on multiple factors, can be managed with a biventricular repair, single ventricle palliation or, one and a half ventricle pathway or cardiac transplantation (usually reserved for the most severe variants with severe coronary disease and ventricular dysfunction).

• #51 Pulmonary atresia with intact ventricular septum | Altru Health System

  https://www.altru.org/health-library/conditions/pulmonary-atresia-with-intact-ventricular-septum

  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. A baby with pulmonary atresia with intact ventricular septum 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 conditions.

• #52

  https://link.springer.com/article/10.1007/s00246-024-03566-x

  This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. […] The aim of this retrospective study was to compare the long-term morbidity of patients treated with catheter-based therapy with those who underwent heart surgery as the initial intervention for right ventricular decompression. […] The inclusion criterion was decompression of the right ventricle for initial intervention. […] All included patients had a membranous atresia and a bi- or tripartite right ventricle. […] There was no mortality in either group during the entire study period. […] In group A, all attempted perforations with radiofrequency of the pulmonary valve were successful.

• #53 Single Ventricle Defects | American Heart Association

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

  Pulmonary Atresia/Intact Ventricular Septum – The pulmonary valve does not exist, and the only blood receiving oxygen is the blood that is diverted to the lungs through openings that normally close during development. […] 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.

• #54 Single Ventricle Defects | American Heart Association

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

  Pulmonary Atresia/Intact Ventricular Septum – The pulmonary valve does not exist, and the only blood receiving oxygen is the blood that is diverted to the lungs through openings that normally close during development. […] 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.

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

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

  Clinicians also note single first and second heart sounds during cardiac auscultation. A pansystolic murmur is audible at the left lower sternal border if the tricuspid valve regurgitates. […] An additional murmur related to the flow across a patent ductus arteriosus might be audible in affected patients upon examination, especially following the initiation of prostaglandin infusion to maintain ductal patency. […] Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. […] 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. […] 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. […] RVDCC, especially atresia of the coronary ostia, is an independent risk factor for poor outcomes.

• #56 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.

• #57 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.

• #58 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

• #59 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

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

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

  Clinicians also note single first and second heart sounds during cardiac auscultation. A pansystolic murmur is audible at the left lower sternal border if the tricuspid valve regurgitates. […] An additional murmur related to the flow across a patent ductus arteriosus might be audible in affected patients upon examination, especially following the initiation of prostaglandin infusion to maintain ductal patency. […] Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. […] 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. […] 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. […] RVDCC, especially atresia of the coronary ostia, is an independent risk factor for poor outcomes.

• #61 Pulmonary atresia with intact ventricular septum | PPT

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

  Occasionally, infants with PAIVS have shown signs of both right and left ventricular ischemia, likely related to the coronary artery fistulae. Association with atretic, hypoplastic, or obstructed central coronary arteries, called right ventricular dependent coronary circulation (RVDCC), carries a higher risk of morbidity and mortality.

• #62

  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). […] Chitayat et al. (1992) reported 2 sisters with hypoplastic right heart and pulmonary atresia. The first sib was found in the newborn period to have this abnormality. An attempt at surgical repair was unsuccessful. In the next pregnancy, fetal echocardiography at 22 weeks of gestation demonstrated the same cardiac abnormalities, which were confirmed at autopsy in the fetus. No other malformations were found in either case and no other relatives were affected. […] Grossfeld et al. (1997) reported 2 first cousins with pulmonary atresia and intact ventricular septum. The first had a severely hypoplastic right ventricle and tricuspid valve and a restrictive atrial septal defect. She died at age 12 days. The patient’s maternal aunt delivered a son with congenital heart disease 23 years earlier. At that time, pulmonary atresia with intact ventricular septum, hypoplastic tricuspid valve and right ventricle, and patent ductus arteriosus were noted. That patient died at age 5 months. Grossfeld et al. (1997) suggested autosomal dominant inheritance with incomplete penetrance.

• #63 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  The clinical manifestations in the newborn are cyanosis, hypoxemia, metabolic acidosis, and right and left heart failure. […] The prognosis is usually poor in the absence of treatment, with over 50% mortality at one month. […] The association of PAIVS with aortic stenosis is rare, with only a few cases reported, either in isolation or as part of HRHS. Survival, as in our patient, is unusual, possibly because of left ventricular failure from multifactorial causes.

• #64 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. This medication enables blood to continue to flow to the lungs for oxygen until a more permanent treatment strategy is determined. […] Babies born with pulmonary atresia with intact ventricular septum require lifelong monitoring by cardiologists experienced in the treatment of congenital heart defects.

• #65 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic

  https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/

  Treatment for pulmonary atresia depends on the severity of the condition and any associated heart defects. It typically involves surgery to improve blood flow to the lungs: […] Children who have been treated for pulmonary atresia need lifelong follow-up care with a cardiologist. […] Pulmonary atresia may be associated with other heart defects, such as atrial septal defects (ASD) or patent ductus arteriosus (PDA), which can further complicate the condition. […] Children with pulmonary atresia may face challenges related to physical development, endurance, and overall health. […] With early diagnosis and treatment, many children with pulmonary atresia can lead healthy lives.

• #66 About Pulmonary Atresia | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/pulmonary-atresia.html

  Babies born with pulmonary atresia will show symptoms at birth or very soon afterwards. They may have a bluish looking skin color, called cyanosis, because their blood doesn’t carry enough oxygen. Infants with pulmonary atresia can have additional symptoms such as: […] Problems breathing […] Ashen or bluish skin color […] Poor feeding […] Extreme sleepiness. […] A baby with pulmonary atresia may need surgery or other procedures soon after birth. Therefore, pulmonary atresia is considered a critical congenital heart defect (critical CHD). […] Most babies with pulmonary atresia will need routine checkups with a heart doctor. A heart doctor will monitor a baby’s progress and check for other health conditions that might develop as they get older. As adults, they may need more surgery or medical care for other possible problems.

• #67 Pulmonary atresia with intact ventricular septum – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/pulmonary-atresia-with-intact-ventricular-septum/

  A baby with pulmonary atresia with intact ventricular septum (PA/IVS) most likely will require one or more procedures to improve heart function and blood flow. Some surgeries and procedures are done in the first days to weeks of a baby’s life. Others are done later. The type of surgery done depends on many things including the size of the right lower heart chamber (right ventricle) and other heart valves, and if the baby has other heart problems. […] After treatment, babies with pulmonary atresia should have regular checkups, ideally with a pediatric heart doctor (pediatric cardiologist). Due to advances in treatment and technology, many people with pulmonary atresia with intact ventricular septum (PA/IVS) survive into adulthood.

• #68 Outcomes of Patients with Pulmonary Atresia with Intact Ventricular Septum Reaching Adulthood

  https://www.techscience.com/chd/v15n1/39373/html

  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect characterized by an imperforate right ventricular outflow tract, intact ventricular septum, and varying degrees of right ventricular and tricuspid valve hypoplasia. […] In adult survivors with PA-IVS, the overall survival rates at 5 years and 10 years were 96.2% (95% confidence interval [CI], 77.299.4) and 96.2% (95% CI, 77.299.4), respectively. […] Heart failure occurred in 4 patients who underwent BVR, in 1 patient who underwent 1.5VR, and in 2 patients who underwent UVR. […] The cumulative heart failure-free survival rates at 5 years and 10 years were 81.4% (95% CI, 62.192.1) and 74.6% (95% CI, 52.388.7), respectively. […] Atrial tachyarrhythmias were observed in 5 (16%) of the 32 patients in our study. […] Adult survivors with PA-IVS are at risk for heart failure, arrhythmic events, and surgical re-intervention.

• #69 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. […] The symptoms of pulmonary atresia with intact ventricular septum may include: Blue or purple tint to lips, skin and nails (cyanosis), Shortness of breath, Difficulty feeding, Fatigue or listlessness, Heart murmur (abnormal heart sounds heard with a stethoscope). […] As single ventricle survivors get older, doctors are recognizing that, while some children do not experience complications, many do, including lung, liver and gastrointestinal complications. […] The outlook can vary depending on how severe the condition is and how well the treatments work. However, many children grow into adulthood, often after having a Fontan procedure and additional treatments over time, and go on to lead fulfilling lives.

• #70 Pulmonary atresia – Wikipedia

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

  The symptoms/signs of pulmonary atresia that will occur in babies are consistent with cyanosis, some fatigue and some shortness of breath (eating may be a problem as well). […] The diagnosis of pulmonary atresia can usually be diagnosed within hours or minutes after birth. […] If uncorrected, babies with this type of congenital heart disease may only survive for the first few days of life. Many children with pulmonary atresia will go on to lead normal lives, though complications such as endocarditis, stroke and seizures are possible.

• #71 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. […] The symptoms of pulmonary atresia with intact ventricular septum may include: Blue or purple tint to lips, skin and nails (cyanosis), Shortness of breath, Difficulty feeding, Fatigue or listlessness, Heart murmur (abnormal heart sounds heard with a stethoscope). […] As single ventricle survivors get older, doctors are recognizing that, while some children do not experience complications, many do, including lung, liver and gastrointestinal complications. […] The outlook can vary depending on how severe the condition is and how well the treatments work. However, many children grow into adulthood, often after having a Fontan procedure and additional treatments over time, and go on to lead fulfilling lives.

• #72 Pulmonary atresia with intact ventricular septum | Altru Health System

  https://www.altru.org/health-library/conditions/pulmonary-atresia-with-intact-ventricular-septum

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, is a rare heart condition that’s present at birth. Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include: Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color. Fast breathing or shortness of breath. Tiredness. Poor feeding. Pulmonary atresia with intact ventricular septum, also called PA/IVS, is often found during pregnancy or soon after birth. If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away. 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. 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.

• #73 NeoCardio Lab – Pulmonary Atresia Intact Ventricular Septum

  https://www.neocardiolab.com/congenital-heart-defects/pulmonary-atresia-intact-ventricular-septum

  Pulmonary atresia with intact ventricular septum (PAIVS) is a complex congenital heart defect characterized by pulmonary valvular atresia. […] Patients may have various presentations, with myocardial sinusoids complicating the course of their management. […] Pulmonary atresia with intact ventricular septum is a complex condition where there is dependent ductal pulmonary circulation. […] They will be cyanotic, considering that the right to left shunt is obligatory at the atrial level, introducing deoxygenated blood in the systemic circulation. […] This condition is of particular interest, considering that there is a risk in a subcategory of infants with PAIVS of persistent sinusoids, possibly stealing from the coronary circulation. […] Because of this, these remnant embryological channels (sinusoids) may stay patent and connect the RV cavity to the coronary system.

• #74 NeoCardio Lab – Pulmonary Atresia Intact Ventricular Septum

  https://www.neocardiolab.com/congenital-heart-defects/pulmonary-atresia-intact-ventricular-septum

  Pulmonary atresia with intact ventricular septum (PAIVS) is a complex congenital heart defect characterized by pulmonary valvular atresia. […] Patients may have various presentations, with myocardial sinusoids complicating the course of their management. […] Pulmonary atresia with intact ventricular septum is a complex condition where there is dependent ductal pulmonary circulation. […] They will be cyanotic, considering that the right to left shunt is obligatory at the atrial level, introducing deoxygenated blood in the systemic circulation. […] This condition is of particular interest, considering that there is a risk in a subcategory of infants with PAIVS of persistent sinusoids, possibly stealing from the coronary circulation. […] Because of this, these remnant embryological channels (sinusoids) may stay patent and connect the RV cavity to the coronary system.

• #75 NeoCardio Lab – Pulmonary Atresia Intact Ventricular Septum

  https://www.neocardiolab.com/congenital-heart-defects/pulmonary-atresia-intact-ventricular-septum

  This may lead to severe coronary steal upon decompression of the RV. […] As such, these patients are at risk of severe myocardial ischemia and there is a requirement to go to the cath lab before any surgery in order to delineate if there is indeed sinusoids connecting to the coronary circulation, before attempting any intervention. […] Depending on the RV size, the RVOT configuration and the presence or absence of sinusoids, these patients will have different approaches for their interventions. […] They may require single ventricular repair because of the above considerations. […] Severe tricuspid valve annular hypoplasia. […] Severe right ventricular hypoplasia. […] No flow coming into the MPA from the RVOT. Pulmonary atresia.

• #76 NeoCardio Lab – Pulmonary Atresia Intact Ventricular Septum

  https://www.neocardiolab.com/congenital-heart-defects/pulmonary-atresia-intact-ventricular-septum

  This may lead to severe coronary steal upon decompression of the RV. […] As such, these patients are at risk of severe myocardial ischemia and there is a requirement to go to the cath lab before any surgery in order to delineate if there is indeed sinusoids connecting to the coronary circulation, before attempting any intervention. […] Depending on the RV size, the RVOT configuration and the presence or absence of sinusoids, these patients will have different approaches for their interventions. […] They may require single ventricular repair because of the above considerations. […] Severe tricuspid valve annular hypoplasia. […] Severe right ventricular hypoplasia. […] No flow coming into the MPA from the RVOT. Pulmonary atresia.

• #77 The double shunt technique as a bridge to heart transplantation in a patient with pulmonary atresia with intact septum and right ventricular-dependent coronary circulation – JTCVS Techniques

  https://www.jtcvstechniques.org/article/S2666-2507(21)00084-5/fulltext

  The unknown length of time to wait for a donor organ and ongoing progressive signs of ischemia resulted in changing the strategy to provide reliable blood supply to both pulmonary circulation and (more importantly) the myocardium. […] The patient continued to do well and remained hemodynamically stable until 1 month postoperatively when she started experiencing recurrent episodes of desaturation. […] This rare subset of patients with PA is better served with transplantation, and no survival has been demonstrated without transplantation in the presence of bilateral COA. […] We propose this double shunt strategy in PA/IVS and RVDCC as an initial stage I palliation for patients with single-ventricle pathway or as a bridge to transplantation.

• #78 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. Pulmonary flow is dependent on the ductus arteriosus. Other morphological anomalies are also present. […] The neonate had low oxygen saturation and a systolic murmur originating in the aorta. An echocardiogram showed pulmonary atresia with intact ventricular septum and a bicuspid, thickened, stenotic aortic valve. Low cardiac output ensued and could not be controlled, and the infant died. […] The neonate had desaturation and an aortic systolic ejection murmur. Echocardiogram showed PAIVS and a thickened and stenotic bicuspid aortic valve. The neonate suffered low cardiac output which could not be controlled, and died.

• #79 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología

  https://www.revespcardiol.org/es-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  Pulmonary atresia with intact ventricular septum is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of ventricular septal defect. […] Pulmonary atresia with intact ventricular septum (PAIVS) is the complete obstruction of the right ventricular outflow tract due to pulmonary valve atresia in the absence of any ventricular septal defect. […] The neonate had desaturation and an aortic systolic ejection murmur. […] The neonate suffered low cardiac output which could not be controlled, and died. […] The clinical manifestations in the newborn are cyanosis, hypoxemia, metabolic acidosis, and right and left heart failure. […] The prognosis is usually poor in the absence of treatment, with over 50% mortality at one month. […] Postnatal survival depends on the patency of the ductus arteriosus, which explains the requirement for continuous infusion of prostaglandin-E1; closure of the ductus would lead to death. […] The association of PAIVS with aortic stenosis is rare, with only a few cases reported, either in isolation or as part of HRHS. […] Survival, as in our patient, is unusual, possibly because of left ventricular failure from multifactorial causes.

• #80 Pulmonary Atresia With Intact Ventricular Septum Associated With Severe Aortic Stenosis – Revista Española de Cardiología (English Edition)

  https://www.revespcardiol.org/en-pulmonary-atresia-with-intact-ventricular-articulo-13055369

  The clinical manifestations in the newborn are cyanosis, hypoxemia, metabolic acidosis, and right and left heart failure. […] The prognosis is usually poor in the absence of treatment, with over 50% mortality at one month. […] The association of PAIVS with aortic stenosis is rare, with only a few cases reported, either in isolation or as part of HRHS. Survival, as in our patient, is unusual, possibly because of left ventricular failure from multifactorial causes.

• #81

  https://omim.org/entry/265150

  De Stefano et al. (2008) reported monozygotic twin sisters with pulmonary valve atresia and intact ventricular septum. The girls died of respiratory failure at age 3 and 7 days, respectively. Echocardiography and postmortem examination of both patients showed pulmonary valve atresia with 3 fused valves and no identifiable lumen. There was generalized cardiomegaly, right atrial dilatation, right ventricular hypertrophy, and small right ventricular chambers. The tricuspid valves were dysplastic with 2 thick leaflets attached to the right ventricular endocardium. One twin also had aortic stenosis with a bicuspid aortic valve and thick, dysplastic leaflets. Molecular analysis identified a biallelic 55-kb deletion of chromosome 20q13.12 in both girls. The unaffected nonconsanguineous parents were each heterozygous for 1 of the deletions.

• #82 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 an anomaly in which the pathway from the right ventricle (RV) to the pulmonary artery is completely sealed off (PA) and there is no ventricular septal defect. […] At birth, the pulmonary valve leaflets may be fused and appear clinically indistinguishable from congenital forms of PA. […] In cases of severe tricuspid regurgitation with a normal TV annulus, the right atrium and RV become dilated, placing the fetus at risk for arrhythmia and hydrops fetalis. Marked cardiomegaly with cardiothoracic area ratios greater than 0.60 may compromise pulmonary vascular and lung development. […] 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. […] Severe dilation of the right heart secondary to severe tricuspid regurgitation and massive cardiomegaly compromises lung development and may lead to hydrops fetalis.

• #83 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 an anomaly in which the pathway from the right ventricle (RV) to the pulmonary artery is completely sealed off (PA) and there is no ventricular septal defect. […] At birth, the pulmonary valve leaflets may be fused and appear clinically indistinguishable from congenital forms of PA. […] In cases of severe tricuspid regurgitation with a normal TV annulus, the right atrium and RV become dilated, placing the fetus at risk for arrhythmia and hydrops fetalis. Marked cardiomegaly with cardiothoracic area ratios greater than 0.60 may compromise pulmonary vascular and lung development. […] 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. […] Severe dilation of the right heart secondary to severe tricuspid regurgitation and massive cardiomegaly compromises lung development and may lead to hydrops fetalis.

• #84 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 an anomaly in which the pathway from the right ventricle (RV) to the pulmonary artery is completely sealed off (PA) and there is no ventricular septal defect. […] At birth, the pulmonary valve leaflets may be fused and appear clinically indistinguishable from congenital forms of PA. […] In cases of severe tricuspid regurgitation with a normal TV annulus, the right atrium and RV become dilated, placing the fetus at risk for arrhythmia and hydrops fetalis. Marked cardiomegaly with cardiothoracic area ratios greater than 0.60 may compromise pulmonary vascular and lung development. […] 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. […] Severe dilation of the right heart secondary to severe tricuspid regurgitation and massive cardiomegaly compromises lung development and may lead to hydrops fetalis.

• #85 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 an anomaly in which the pathway from the right ventricle (RV) to the pulmonary artery is completely sealed off (PA) and there is no ventricular septal defect. […] At birth, the pulmonary valve leaflets may be fused and appear clinically indistinguishable from congenital forms of PA. […] In cases of severe tricuspid regurgitation with a normal TV annulus, the right atrium and RV become dilated, placing the fetus at risk for arrhythmia and hydrops fetalis. Marked cardiomegaly with cardiothoracic area ratios greater than 0.60 may compromise pulmonary vascular and lung development. […] 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. […] Severe dilation of the right heart secondary to severe tricuspid regurgitation and massive cardiomegaly compromises lung development and may lead to hydrops fetalis.

• #86 Pulmonary Atresia: Causes, Symptoms, and Treatment – Longmore Clinic

  https://longmoreclinic.org/pulmonary-atresia-causes-symptoms-and-treatment/

  Treatment for pulmonary atresia depends on the severity of the condition and any associated heart defects. It typically involves surgery to improve blood flow to the lungs: […] Children who have been treated for pulmonary atresia need lifelong follow-up care with a cardiologist. […] Pulmonary atresia may be associated with other heart defects, such as atrial septal defects (ASD) or patent ductus arteriosus (PDA), which can further complicate the condition. […] Children with pulmonary atresia may face challenges related to physical development, endurance, and overall health. […] With early diagnosis and treatment, many children with pulmonary atresia can lead healthy lives.

• #87 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. This condition is characterized by the absence of a properly formed pulmonary valve, which prevents blood flow from the right ventricle to the lungs. As a result, the right ventricle and tricuspid valve often remain underdeveloped, leading to significant challenges in oxygenating blood effectively. PAIVS typically presents at or shortly after birth, necessitating immediate medical attention due to the critical nature of the defect. […] Newborns with PAIVS often exhibit symptoms such as cyanosis (a bluish tint to the skin) and low oxygen saturation levels, particularly after the closure of the patent ductus arteriosus (PDA), which is crucial for maintaining pulmonary blood flow. Physical examinations may reveal distinct heart sounds and murmurs indicative of tricuspid regurgitation or PDA.

• #88 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.

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

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

  The prognosis for children with PAIVS varies significantly based on individual anatomical considerations. Regular follow-up care from a pediatric cardiology team is essential to monitor heart function and address any complications that may arise over time. […] Early diagnosis and tailored management strategies are crucial for improving outcomes in affected infants.

• #90 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.

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