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. […] 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 diagnosis of this anomaly is predominantly made through echocardiography, which can detect PA-IVS prenatally in about 86% of cases. […] However, echocardiography alone cannot determine coronary circulation details, necessitating cardiac catheterization with angiography to assess coronary connections and RVDCC fully.

• #2 Prenatal Diagnosis of Pulmonary Atresia with Intact Ventricular Septum

  https://www.contemporaryobgyn.net/view/prenatal-diagnosis-pulmonary-atresia-intact-ventricular-septum

  Hypoplasia of the right ventricle is uncommon as an isolated entity. It may result from tricuspid atresia, but is most commonly secondary to pulmonary atresia with intact ventricular septum. […] Pulmonary atresia with intact ventricular septum is rare, accounting for 1-3% of cases of congenital heart disease. It occurs in 0.1 to 0.4 in 10,000 live births. Pulmonary atresia with intact ventricular septum has been reported to occur slightly more frequently in males. […] Because a normal caliber main pulmonary artery does not exclude the diagnosis of pulmonary atresia, demonstrating reversal of blood flow through the ductus is essential. Retrograde blood flow through the ductus is virtually always present in pulmonary atresia with intact ventricular septum. […] The outcome for infants born with pulmonary atresia and intact ventricular septum is poor. They are dependent on a patent ductus arteriosus for survival in the neonatal period. Administration of prostaglandin E1 at birth is essential to maintain patency until surgery can be performed. […] The long-term prognosis may depend, in part, on the size of the right ventricle because mortality rates are higher among those neonates with a severely hypoplastic right ventricle and a small tricuspid valve orifice.

• #3 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.

• #4 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. […] 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.

• #5 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. […] The diagnosis of this anomaly is predominantly made through echocardiography, which can detect PA-IVS prenatally in about 86% of cases. […] However, echocardiography alone cannot determine coronary circulation details, necessitating cardiac catheterization with angiography to assess coronary connections and RVDCC fully.

• #6 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. […] The diagnosis of this anomaly is predominantly made through echocardiography, which can detect PA-IVS prenatally in about 86% of cases. […] However, echocardiography alone cannot determine coronary circulation details, necessitating cardiac catheterization with angiography to assess coronary connections and RVDCC fully.

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

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

  Pulmonary atresia with intact ventricular septum (PA-IVS) is one of the rare forms of congenital heart disease, accounting for <1% of total heart defects. 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. [...] The diagnosis of this anomaly is predominantly made through echocardiography, which can detect PA-IVS prenatally in about 86% of cases. This imaging assesses the interatrial septum, tricuspid valve, right ventricle, and branch pulmonary arteries. [...] However, echocardiography alone cannot determine coronary circulation details, necessitating cardiac catheterization with angiography to assess coronary connections and RVDCC fully.

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

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

  Pulmonary atresia may be diagnosed during pregnancy or soon after the baby is born. […] An ultrasound, a tool that creates pictures of the baby, may detect pulmonary atresia during pregnancy. If the health care provider suspects pulmonary atresia, they can request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby’s heart. This test can show problems with the structure of the heart and how the heart is working. […] During a physical examination, a doctor can see the symptoms, such as bluish skin or problems breathing. Using a stethoscope, a doctor will check for a heart murmur (an abnormal „whooshing” sound caused by blood not flowing properly). However, it is not uncommon for a heart murmur to be absent right at birth. […] If a doctor suspects a problem, the health care provider might request an echocardiogram, which is an ultrasound of the heart. Cardiac catheterization can also confirm the diagnosis by looking inside the heart and measuring the blood pressure and oxygen levels. An electrocardiogram (EKG) may also be conducted to confirm the diagnosis. […] Pulmonary atresia can also be detected with newborn pulse oximetry screening. Low levels of oxygen in the blood can be a sign of a critical CHD like pulmonary atresia. Newborn screening using pulse oximetry can identify some infants with pulmonary atresia before they show any symptoms.

• #9 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

  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. […] Once the child is born, the baby does not get enough oxygen in the blood. The main sign of oxygen deficiency is the presence of a bluish-purple discolouration of the skin and mucous membranes. Occasionally, there is also rapid and shallow breathing. After birth, it is necessary to treat the newborn immediately. Treatment of PA-IVS may include a combination of medications, procedures, or surgery to correct the congenital heart defect.

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

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

  In the fetus with PA/IVS, there is no antegrade flow across the pulmonary valve. […] 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 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. […] Fetal intervention for PA/IVS remains controversial, although proponents argue that fetal pulmonary valvuloplasty is indicated in cases of impending hydrops.

• #11 Pulmonary atresia with intact interventricular septum | PPT

  https://www.slideshare.net/ramachandrabarik/pulmonary-atresia-with-intact-interventricular-septum

  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, and an intact ventricular septum that allows no connection between the right and left ventricles […] Diagnosis of PA IVS should include: 1) Adequacy of PDA to sustain adequate pulmonary blood flow; adequacy of ASD for decompression of systemic venous return to LA 2) Size of RV; presence or absence of inlet, trabecular and/or infundibular portions 3) Evaluation of RVOT, anatomic nature of pulmonary valve (muscular/ membranous) 4) Any evidence of opening in a membranous pulmonary valve, esp. if presence of PR 5) Evaluation of TV annulus diameter, any sign of Ebsteins anomaly, related chordal attachment abnormalities 6) Identification of coronary artery abnormalities and/or RVDCC […] Fetal echo can predict biventricular circulation (at 26 weeks gestation) with 92% specificity.

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

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

  Key Echocardiographic Features […] 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. […] The morphology of the RV may be abnormal and is an important component of the anomaly. […] Coronary abnormalities are common in PA/IVS, especially in the setting of a hypertrophied, hypertensive RV with little or no tricuspid regurgitation. […] The prevalence of coronary arterial abnormalities in PA/IVS is as follows. […] Finally, left heart abnormalities may be seen in PA/IVS. […] PA/IVS is a rare congenital heart defect with no known genetic etiology.

• #13 Pulmonary atresia with intact interventricular septum | PPT

  https://www.slideshare.net/ramachandrabarik/pulmonary-atresia-with-intact-interventricular-septum

  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, and an intact ventricular septum that allows no connection between the right and left ventricles […] Diagnosis of PA IVS should include: 1) Adequacy of PDA to sustain adequate pulmonary blood flow; adequacy of ASD for decompression of systemic venous return to LA 2) Size of RV; presence or absence of inlet, trabecular and/or infundibular portions 3) Evaluation of RVOT, anatomic nature of pulmonary valve (muscular/ membranous) 4) Any evidence of opening in a membranous pulmonary valve, esp. if presence of PR 5) Evaluation of TV annulus diameter, any sign of Ebsteins anomaly, related chordal attachment abnormalities 6) Identification of coronary artery abnormalities and/or RVDCC […] Fetal echo can predict biventricular circulation (at 26 weeks gestation) with 92% specificity.

• #14 Prenatal Diagnosis of Pulmonary Atresia with Intact Ventricular Septum

  https://www.contemporaryobgyn.net/view/prenatal-diagnosis-pulmonary-atresia-intact-ventricular-septum

  Hypoplasia of the right ventricle is uncommon as an isolated entity. It may result from tricuspid atresia, but is most commonly secondary to pulmonary atresia with intact ventricular septum. […] Pulmonary atresia with intact ventricular septum is rare, accounting for 1-3% of cases of congenital heart disease. It occurs in 0.1 to 0.4 in 10,000 live births. Pulmonary atresia with intact ventricular septum has been reported to occur slightly more frequently in males. […] Because a normal caliber main pulmonary artery does not exclude the diagnosis of pulmonary atresia, demonstrating reversal of blood flow through the ductus is essential. Retrograde blood flow through the ductus is virtually always present in pulmonary atresia with intact ventricular septum. […] The outcome for infants born with pulmonary atresia and intact ventricular septum is poor. They are dependent on a patent ductus arteriosus for survival in the neonatal period. Administration of prostaglandin E1 at birth is essential to maintain patency until surgery can be performed. […] The long-term prognosis may depend, in part, on the size of the right ventricle because mortality rates are higher among those neonates with a severely hypoplastic right ventricle and a small tricuspid valve orifice.

• #15

  https://journals.lww.com/pjog/fulltext/2024/07000/prenatally_diagnosed_pulmonary_atresia_with_intact.11.aspx

  Congenital heart disease is the most common birth defect, affecting 1%1.2% of live born infants. Pulmonary atresia with intact ventricular septum (PA-IVS) accounts for 1% of all total heart defects. The cause of PA-IVS has been unclear. Thus, experience for prenatal diagnosis of PA-IVS is limited in any single institution. […] Prenatal diagnosis of PA-IVS allows options for the termination of pregnancy, fetal cardiac interventional therapy, early postnatal initiation of prostaglandin E1, and planned early neonatal interventional surgeries for palliation and repair. Early assessment of fetal cardiac features is useful for a better outcome. […] Prenatal diagnosis of PA-IVS aids in a patient-specific counseling including specific fetal characteristics that determine morbidity and mortality. Prenatal diagnosis also allows for options of termination of pregnancy in countries where it is legal and FCI (pulmonary valvuloplasty). Early assessment of fetal cardiac features through detailed fetal cardiac evaluation would be useful for the proper timing of FCI.

• #16

  https://journals.lww.com/pjog/fulltext/2024/07000/prenatally_diagnosed_pulmonary_atresia_with_intact.11.aspx

  Although the benefit of prenatal intervention versus postnatal intervention is still contentious, there have been increasing evidence that it can alter the natural history of fetuses and provide an opportunity for a better outcome postnatally. […] Prenatal diagnosis helps in the risk stratification of the most ideal postnatal palliative and corrective approach. This is determined by the initial TV morphology, RV morphology, form of the PV atresia (membranous atresia imperforate valve versus muscular atresia atresia of the entire outflow tract), and the presence or absence of VCAC. […] Several prognostic models have been described to predict biventricular outcome versus a single ventricle outcome in the setting of PA-IVS. […] Maternal psychological state is in general positively affected by prenatal diagnosis of CHD in women who elect to continue a pregnancy to term because they are spared the guilt associated with decision-making as they are given time to prepare for the arrival or death of the neonate. […] Prenatal diagnosis of CHD ultimately seems to produce more hemodynamically stable neonates with better outcomes than those undetected before birth, as it provides opportunity for detailed cardiac malformation analysis and fetal intervention.

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

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

  In the fetus with PA/IVS, there is no antegrade flow across the pulmonary valve. […] 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 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. […] Fetal intervention for PA/IVS remains controversial, although proponents argue that fetal pulmonary valvuloplasty is indicated in cases of impending hydrops.

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

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

  To evaluate the efficacy of prenatal ultrasound in diagnosing pulmonary atresia with intact ventricular septum (PA/IVS). […] This retrospective study analyzed 48 cases of PA/IVS at the Fujian Maternity and Child Health Hospital between January 2013 and December 2023. Prenatal ultrasound was used to characterize and classify the features of PA/IVS. […] Fetal echocardiography is an effective tool for diagnosing PA/IVS. While PA/IVS can be diagnosed in early gestation, it remains diagnostical challenging. […] Given the progressive nature of PA/IVS in utero, sequential ultrasound examinations during the second and third trimesters are essential for monitoring disease progression and hemodynamic changes. Additionally, a comprehensive evaluation for associated intracardiac and extracardiac anomalies should be systematically conducted throughout the pregnancy.

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

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

  The early pregnancy detection rate of 8.3% in our cohort aligns with emerging evidence supporting first-trimester cardiac screening. […] The association of NT thickening and abnormal DV flow with subsequent PA/IVS diagnosis suggests these markers warrant thorough fetal echocardiography. […] The 14.6% twin pregnancy incidence exceeds general population expectations, with particularly notable progression dynamics in TTTS cases. […] The 86.4% termination rate for mid-late diagnoses reflects both disease severity and parental counseling realities.

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

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

  The early pregnancy detection rate of 8.3% in our cohort aligns with emerging evidence supporting first-trimester cardiac screening. […] The association of NT thickening and abnormal DV flow with subsequent PA/IVS diagnosis suggests these markers warrant thorough fetal echocardiography. […] The 14.6% twin pregnancy incidence exceeds general population expectations, with particularly notable progression dynamics in TTTS cases. […] The 86.4% termination rate for mid-late diagnoses reflects both disease severity and parental counseling realities.

• #21 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 may be diagnosed before birth with a fetal ultrasound, called an echocardiogram. Sometimes, it’s diagnosed a few hours or days after birth when the ductus arteriosus (the extra blood vessel that connects the pulmonary artery to the aorta while a baby is in the womb) begins to close. Because the baby will then have no blood flow to the lungs, they will become very blue (cyanotic).

• #22 Pulmonary atresia – Wikipedia

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

  Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The diagnosis of pulmonary atresia can be done via the following exams/methods: an echocardiogram, chest x-ray, EKG and an exam to measure the amount of O2 in the body. […] In regards to the diagnosis of pulmonary atresia the body requires oxygenated blood for survival. pulmonary atresia is not threatening to a developing fetus however, because the mother’s placenta provides the needed oxygen since the baby’s lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with pulmonary atresia there is no opening on the pulmonary valve for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth.

• #23 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. […] 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. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] The primary goal of cardiac catheterization is to assess for RVDCC. […] Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure.

• #24 Pulmonary Valve Atresia | NCBDDD | CDC

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

  Postnatal. 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. Echocardiography is the key diagnostic procedure, although other imaging techniques, including catheterization, might be necessary to fully guide management and care. […] 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.

• #25 Pulmonary atresia: Types, causes, symptoms, and more

  https://www.medicalnewstoday.com/articles/pulmonary-atresia

  If a doctor notices signs of pulmonary atresia once a baby is born, they may request an echocardiogram or other tests. […] Doctors treat most cases of pulmonary atresia with medication to keep the ductus arteriosus open. […] A baby with pulmonary atresia may need surgery soon after birth. […] The condition is usually noticeable at birth or soon after, and in some cases a doctor may be able to diagnose it during pregnancy. […] Signs and symptoms of pulmonary atresia can include cyanosis, heart murmur, breathing difficulties, feeding difficulties, tiredness, and failure to thrive. […] While the exact cause of pulmonary atresia is unclear, a person can take steps to reduce the likelihood that the condition will develop.

• #26 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. […] 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. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] The primary goal of cardiac catheterization is to assess for RVDCC. […] Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure.

• #27 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, can be diagnosed before birth with an ultrasound during a routine pregnancy screening. Or it may be diagnosed soon after the baby is born. […] Tests used to diagnose pulmonary atresia with intact ventricular septum, also called PA/IVS, include: […] A small sensor clipped to a hand or foot measures how much oxygen is in the blood. […] Also called an ECG or EKG, this simple test measures the electrical activity of the heart. It shows how the heart is beating. […] A chest X-ray shows the shape and size of the heart and lungs. […] Sound waves are used to make pictures of the beating heart. An echocardiogram shows how blood moves through the heart and heart valves, including the pulmonary valve. […] This test shows the heart’s arteries. A doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the groin. It’s guided to the heart.

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

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

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, can be diagnosed before birth with an ultrasound during a routine pregnancy screening. Or it may be diagnosed soon after the baby is born. […] Tests used to diagnose pulmonary atresia with intact ventricular septum, also called PA/IVS, include: […] A small sensor clipped to a hand or foot measures how much oxygen is in the blood. […] Also called an ECG or EKG, this simple test measures the electrical activity of the heart. It shows how the heart is beating. […] A chest X-ray shows the shape and size of the heart and lungs. […] Sound waves are used to make pictures of the beating heart. An echocardiogram shows how blood moves through the heart and heart valves, including the pulmonary valve. […] This test shows the heart’s arteries. A doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the groin. It’s guided to the heart. Dye flows through the catheter and into the heart arteries. The dye helps the arteries show up more clearly on X-rays.

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

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

  Pulmonary atresia may be diagnosed during pregnancy or soon after the baby is born. […] An ultrasound, a tool that creates pictures of the baby, may detect pulmonary atresia during pregnancy. If the health care provider suspects pulmonary atresia, they can request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby’s heart. This test can show problems with the structure of the heart and how the heart is working. […] During a physical examination, a doctor can see the symptoms, such as bluish skin or problems breathing. Using a stethoscope, a doctor will check for a heart murmur (an abnormal „whooshing” sound caused by blood not flowing properly). However, it is not uncommon for a heart murmur to be absent right at birth. […] If a doctor suspects a problem, the health care provider might request an echocardiogram, which is an ultrasound of the heart. Cardiac catheterization can also confirm the diagnosis by looking inside the heart and measuring the blood pressure and oxygen levels. An electrocardiogram (EKG) may also be conducted to confirm the diagnosis. […] Pulmonary atresia can also be detected with newborn pulse oximetry screening. Low levels of oxygen in the blood can be a sign of a critical CHD like pulmonary atresia. Newborn screening using pulse oximetry can identify some infants with pulmonary atresia before they show any symptoms.

• #30 Pulmonary atresia with intact ventricular septum

  https://www.mymlc.com/health-information/diseases-and-conditions/p/pulmonary-atresia-with-intact-ventricular-septum/?section=Diagnosis

  Cardiac catheterization. In catheterization, your baby’s doctor inserts a thin, flexible tube (catheter) into a blood vessel in your baby’s groin and guides it to your baby’s heart. This test shows your baby’s heart structure and the blood pressure and oxygen levels in your baby’s heart, pulmonary artery and aorta. The doctor may inject a dye into the catheter to make your baby’s arteries visible under X-ray. […] Pulse oximetry. This test indicates how much oxygen your baby is getting through his or her blood. […] X-ray. An X-ray shows your baby’s doctor the size of your baby’s pulmonary atresia, as well as bones and other tissues.

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

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

  Other tests are needed to help with the diagnosis. They may include: Chest X-ray – a diagnostic test that uses invisible electromagnetic energy beams to create images of internal tissues, bones, and organs onto film. Electrocardiogram (ECG or EKG) – a test that records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias or dysrhythmias), and can see heart muscle stress. This test may look different than normal for a newborn with pulmonary atresia. Echocardiogram (echo) – a procedure that looks at the structure and function of the heart by using sound waves recorded and shown on a screen. The sounds waves make a moving picture of the heart and heart valves. Cardiac catheterization – an invasive procedure that gives detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is put into a blood vessel in the groin and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart and in the pulmonary artery and aorta. Contrast dye is injected to see the heart structures more clearly. […] This heart defect may also be diagnosed on fetal echocardiograms. It is one of the cardiac defects that may be found on screening ultrasounds. Early diagnosis of the defect allows for prompt intervention at the time of birth.

• #32 Pulmonary atresia with intact ventricular septum

  https://www.mymlc.com/health-information/diseases-and-conditions/p/pulmonary-atresia-with-intact-ventricular-septum/?section=Diagnosis

  Pulmonary atresia (uh-TREE-zhuh) is one of several heart valve defects that is found at birth or shortly afterward. In pulmonary atresia, the valve that allows blood to flow from the heart to the lungs (pulmonary valve) doesn’t work correctly. Instead of opening and closing to allow blood to travel from the heart to the lungs, a sheet of tissue or muscle blocks blood flow. […] Several tests may be used to diagnose pulmonary atresia, including: […] Echocardiogram. In an echocardiogram, sound wave movement shows the features of your child’s heart. Your doctor might also use an echocardiogram of your abdomen before you deliver your baby (fetal echocardiogram) to diagnose pulmonary atresia. […] Electrocardiogram (ECG). In this test, sensor patches with wires attached (electrodes) measure the electrical impulses given off by your baby’s heart. An ECG reveals abnormal heart rhythms (arrhythmias or dysrhythmias) and may show other heart problems.

• #33 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, can be diagnosed before birth with an ultrasound during a routine pregnancy screening. Or it may be diagnosed soon after the baby is born. […] Tests used to diagnose pulmonary atresia with intact ventricular septum, also called PA/IVS, include: […] A small sensor clipped to a hand or foot measures how much oxygen is in the blood. […] Also called an ECG or EKG, this simple test measures the electrical activity of the heart. It shows how the heart is beating. […] A chest X-ray shows the shape and size of the heart and lungs. […] Sound waves are used to make pictures of the beating heart. An echocardiogram shows how blood moves through the heart and heart valves, including the pulmonary valve. […] This test shows the heart’s arteries. A doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the groin. It’s guided to the heart.

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

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

  Pulmonary atresia with intact ventricular septum, also called PA/IVS, can be diagnosed before birth with an ultrasound during a routine pregnancy screening. Or it may be diagnosed soon after the baby is born. […] Tests used to diagnose pulmonary atresia with intact ventricular septum, also called PA/IVS, include: […] A small sensor clipped to a hand or foot measures how much oxygen is in the blood. […] Also called an ECG or EKG, this simple test measures the electrical activity of the heart. It shows how the heart is beating. […] A chest X-ray shows the shape and size of the heart and lungs. […] Sound waves are used to make pictures of the beating heart. An echocardiogram shows how blood moves through the heart and heart valves, including the pulmonary valve. […] This test shows the heart’s arteries. A doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the groin. It’s guided to the heart. Dye flows through the catheter and into the heart arteries. The dye helps the arteries show up more clearly on X-rays.

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

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

  Pulmonary atresia with intact ventricular septum (PA-IVS) is one of the rare forms of congenital heart disease, accounting for <1% of total heart defects. 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. [...] The diagnosis of this anomaly is predominantly made through echocardiography, which can detect PA-IVS prenatally in about 86% of cases. This imaging assesses the interatrial septum, tricuspid valve, right ventricle, and branch pulmonary arteries. [...] However, echocardiography alone cannot determine coronary circulation details, necessitating cardiac catheterization with angiography to assess coronary connections and RVDCC fully.

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

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

  A readily available and accessible modality for cardiac evaluation is 2-dimensional echocardiography, which is frequently used in the diagnosis of PA-IVS. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] Cardiac catheterization with angiocardiography is often used to diagnose patients with PA-IVS, as it provides additional information regarding coronary circulation; this procedure is critical in patients for whom decompression of the right ventricle is a consideration. […] The primary goal of cardiac catheterization is to assess for RVDCC. The diagnosis of RVDCC is made when a significant portion of the ventricular myocardium depends on the right ventricle for the blood supply.

• #37 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, can be diagnosed before birth with an ultrasound during a routine pregnancy screening. Or it may be diagnosed soon after the baby is born. […] Tests used to diagnose pulmonary atresia with intact ventricular septum, also called PA/IVS, include: […] A small sensor clipped to a hand or foot measures how much oxygen is in the blood. […] Also called an ECG or EKG, this simple test measures the electrical activity of the heart. It shows how the heart is beating. […] A chest X-ray shows the shape and size of the heart and lungs. […] Sound waves are used to make pictures of the beating heart. An echocardiogram shows how blood moves through the heart and heart valves, including the pulmonary valve. […] This test shows the heart’s arteries. A doctor places a thin, flexible tube called a catheter into a blood vessel, usually in the groin. It’s guided to the heart.

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

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

  A readily available and accessible modality for cardiac evaluation is 2-dimensional echocardiography, which is frequently used in the diagnosis of PA-IVS. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] Cardiac catheterization with angiocardiography is often used to diagnose patients with PA-IVS, as it provides additional information regarding coronary circulation; this procedure is critical in patients for whom decompression of the right ventricle is a consideration. […] The primary goal of cardiac catheterization is to assess for RVDCC. The diagnosis of RVDCC is made when a significant portion of the ventricular myocardium depends on the right ventricle for the blood supply.

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

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

  Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] The primary goal of cardiac catheterization is to assess for RVDCC. […] The diagnosis of RVDCC is made when a significant portion of the ventricular myocardium depends on the right ventricle for the blood supply.

• #40 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. […] 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. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] The primary goal of cardiac catheterization is to assess for RVDCC. […] Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. […] Neonates with PA-IVS require interventional treatment, either through a catheter-based intervention or a surgical procedure.

• #41 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. […] 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.

• #42 Pulmonary Atresia: Overview, Causes, Treatment

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

  MRI or CT scan: An MRI scan or CT scan provides detailed heart and blood vessel images. […] Pulmonary atresia can be a serious condition with a risk of death, especially without appropriate treatment. But with advances in medical and surgical care, the outlook for individuals with pulmonary atresia has improved significantly, and many can lead relatively normal lives with appropriate management. […] In a study of 171 children with pulmonary atresia and intact ventricular septum, a rarer form, showed improved 1-year survival from 76% to 92% between 1980-1998 and 1999-2016, respectively. […] The exact survival rate varies, but overall, there has been a significant improvement in survival rates in recent years.

• #43 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: […] 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. […] In some cases, the condition isn’t diagnosed until after the baby is born. Symptoms in a newborn may include: […] 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. […] 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).

• #44 Pulmonary Atresia | Boston Children’s Hospital

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

  Pulmonary atresia is type of heart defect that a baby is born with. It occurs when the pulmonary valve — normally located between the right ventricle and the pulmonary artery — doesn’t form properly. This means that blood can’t flow from the heart to the lungs to get oxygen to the body. In some cases, babies with pulmonary atresia may also have a small, or missing, right ventricle that can’t properly pump blood to the lungs. […] Pulmonary atresia is a life-threatening condition, affecting one out of every 10,000 newborns. Babies born with pulmonary atresia need medication and surgery to correct the heart defect and improve blood flow to the lungs. […] Most babies with pulmonary atresia are diagnosed shortly after birth. In some cases, it is diagnosed before birth by a prenatal ultrasound.

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

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

  Immediately following the diagnosis of PA-IVS, an attempt should be made to initiate prostaglandin infusion to maintain ductus arteriosus patency. […] Clinicians should also recall that patients in categories 2, 3, and 4 require a systemic-to-pulmonary artery shunt or stenting of the PDA in the immediate newborn period. […] The echocardiogram plays a vital role in diagnosing PA-IVS. […] Cardiac catheterization provides additional information regarding the status of the coronary circulation.

• #46 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.

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

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

  Managing PA-IVS begins with prostaglandin infusion to maintain ductal patency, which is crucial for survival preoperatively. […] The preferred surgical or transcatheter intervention is influenced by several variables, which include the size and function of the tricuspid valve, the anatomy of the right ventricle and coronary arteries, and the type of pulmonary atresia. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] The primary goal of cardiac catheterization is to assess for RVDCC. […] The diagnosis of RVDCC is made when a significant portion of the ventricular myocardium depends on the right ventricle for the blood supply.

• #48

  https://journals.lww.com/pjog/fulltext/2024/07000/prenatally_diagnosed_pulmonary_atresia_with_intact.11.aspx

  Congenital heart disease is the most common birth defect, affecting 1%1.2% of live born infants. Pulmonary atresia with intact ventricular septum (PA-IVS) accounts for 1% of all total heart defects. The cause of PA-IVS has been unclear. Thus, experience for prenatal diagnosis of PA-IVS is limited in any single institution. […] Prenatal diagnosis of PA-IVS allows options for the termination of pregnancy, fetal cardiac interventional therapy, early postnatal initiation of prostaglandin E1, and planned early neonatal interventional surgeries for palliation and repair. Early assessment of fetal cardiac features is useful for a better outcome. […] Prenatal diagnosis of PA-IVS aids in a patient-specific counseling including specific fetal characteristics that determine morbidity and mortality. Prenatal diagnosis also allows for options of termination of pregnancy in countries where it is legal and FCI (pulmonary valvuloplasty). Early assessment of fetal cardiac features through detailed fetal cardiac evaluation would be useful for the proper timing of FCI.

• #49

  https://journals.lww.com/pjog/fulltext/2024/07000/prenatally_diagnosed_pulmonary_atresia_with_intact.11.aspx

  Although the benefit of prenatal intervention versus postnatal intervention is still contentious, there have been increasing evidence that it can alter the natural history of fetuses and provide an opportunity for a better outcome postnatally. […] Prenatal diagnosis helps in the risk stratification of the most ideal postnatal palliative and corrective approach. This is determined by the initial TV morphology, RV morphology, form of the PV atresia (membranous atresia imperforate valve versus muscular atresia atresia of the entire outflow tract), and the presence or absence of VCAC. […] Several prognostic models have been described to predict biventricular outcome versus a single ventricle outcome in the setting of PA-IVS. […] Maternal psychological state is in general positively affected by prenatal diagnosis of CHD in women who elect to continue a pregnancy to term because they are spared the guilt associated with decision-making as they are given time to prepare for the arrival or death of the neonate. […] Prenatal diagnosis of CHD ultimately seems to produce more hemodynamically stable neonates with better outcomes than those undetected before birth, as it provides opportunity for detailed cardiac malformation analysis and fetal intervention.

• #50

  https://journals.lww.com/pjog/fulltext/2024/07000/prenatally_diagnosed_pulmonary_atresia_with_intact.11.aspx

  Although the benefit of prenatal intervention versus postnatal intervention is still contentious, there have been increasing evidence that it can alter the natural history of fetuses and provide an opportunity for a better outcome postnatally. […] Prenatal diagnosis helps in the risk stratification of the most ideal postnatal palliative and corrective approach. This is determined by the initial TV morphology, RV morphology, form of the PV atresia (membranous atresia imperforate valve versus muscular atresia atresia of the entire outflow tract), and the presence or absence of VCAC. […] Several prognostic models have been described to predict biventricular outcome versus a single ventricle outcome in the setting of PA-IVS. […] Maternal psychological state is in general positively affected by prenatal diagnosis of CHD in women who elect to continue a pregnancy to term because they are spared the guilt associated with decision-making as they are given time to prepare for the arrival or death of the neonate. […] Prenatal diagnosis of CHD ultimately seems to produce more hemodynamically stable neonates with better outcomes than those undetected before birth, as it provides opportunity for detailed cardiac malformation analysis and fetal intervention.

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

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

  To evaluate the efficacy of prenatal ultrasound in diagnosing pulmonary atresia with intact ventricular septum (PA/IVS). […] This retrospective study analyzed 48 cases of PA/IVS at the Fujian Maternity and Child Health Hospital between January 2013 and December 2023. Prenatal ultrasound was used to characterize and classify the features of PA/IVS. […] Fetal echocardiography is an effective tool for diagnosing PA/IVS. While PA/IVS can be diagnosed in early gestation, it remains diagnostical challenging. […] Given the progressive nature of PA/IVS in utero, sequential ultrasound examinations during the second and third trimesters are essential for monitoring disease progression and hemodynamic changes. Additionally, a comprehensive evaluation for associated intracardiac and extracardiac anomalies should be systematically conducted throughout the pregnancy.

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

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

  The early pregnancy detection rate of 8.3% in our cohort aligns with emerging evidence supporting first-trimester cardiac screening. […] The association of NT thickening and abnormal DV flow with subsequent PA/IVS diagnosis suggests these markers warrant thorough fetal echocardiography. […] The 14.6% twin pregnancy incidence exceeds general population expectations, with particularly notable progression dynamics in TTTS cases. […] The 86.4% termination rate for mid-late diagnoses reflects both disease severity and parental counseling realities.

• #53

  https://journals.lww.com/pjog/fulltext/2024/07000/prenatally_diagnosed_pulmonary_atresia_with_intact.11.aspx

  Although the benefit of prenatal intervention versus postnatal intervention is still contentious, there have been increasing evidence that it can alter the natural history of fetuses and provide an opportunity for a better outcome postnatally. […] Prenatal diagnosis helps in the risk stratification of the most ideal postnatal palliative and corrective approach. This is determined by the initial TV morphology, RV morphology, form of the PV atresia (membranous atresia imperforate valve versus muscular atresia atresia of the entire outflow tract), and the presence or absence of VCAC. […] Several prognostic models have been described to predict biventricular outcome versus a single ventricle outcome in the setting of PA-IVS. […] Maternal psychological state is in general positively affected by prenatal diagnosis of CHD in women who elect to continue a pregnancy to term because they are spared the guilt associated with decision-making as they are given time to prepare for the arrival or death of the neonate. […] Prenatal diagnosis of CHD ultimately seems to produce more hemodynamically stable neonates with better outcomes than those undetected before birth, as it provides opportunity for detailed cardiac malformation analysis and fetal intervention.

• #54 Pulmonary Atresia | Boston Children’s Hospital

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

  If your baby is born with a bluish tint to the skin or other symptoms of pulmonary atresia, he or she will likely see a cardiologist (heart doctor). […] To diagnose pulmonary atresia, the cardiologist will examine the baby and measure the oxygen level in his or her blood. The cardiologist will also listen for a heart murmur — a noise heard through a stethoscope that’s caused by the turbulence of blood flow. This will give the cardiologist an initial idea of the kind of heart problem your baby may have. […] The cardiologist may order one or more of the following tests to help diagnose pulmonary atresia: Electrocardiogram (ECG or EKG), Echocardiogram (cardiac ultrasound), Cardiac MRI, Cardiac catheterization, Chest X-ray. […] A cardiac catheterization is often performed to determine how blood is supplied to the heart in patients with pulmonary atresia with intact ventricular septum.

• #55 Pulmonary atresia: MedlinePlus Medical EncyclopediaLock

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

  Although both conditions are called pulmonary atresia, they are, in fact, different defects. This article discusses pulmonary atresia without a VSD. […] The following tests may be ordered: Chest x-ray, Echocardiogram, Electrocardiogram (ECG), Heart catheterization, Pulse oximetry — shows the amount of oxygen in the blood. […] A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. […] Most cases can be helped with surgery. […] Outcome varies because of the different forms of this defect. A baby may need only a single procedure or could need three or more surgeries and have only a single working ventricle. […] There is no known way to prevent this condition. […] If the defect is found before birth, medical specialists (such as a pediatric cardiologist, cardiothoracic surgeon, and neonatologist) can be present at the birth, and ready to help as needed. This preparation can mean the difference between life and death for some babies.

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

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

  A readily available and accessible modality for cardiac evaluation is 2-dimensional echocardiography, which is frequently used in the diagnosis of PA-IVS. […] Echocardiogram alone can diagnose PA-IVS, but additional information regarding coronary circulation, a significant predictor of outcomes, and the type of repair cannot be discerned from this modality alone. […] Cardiac catheterization with angiocardiography is often used to diagnose patients with PA-IVS, as it provides additional information regarding coronary circulation; this procedure is critical in patients for whom decompression of the right ventricle is a consideration. […] The primary goal of cardiac catheterization is to assess for RVDCC. The diagnosis of RVDCC is made when a significant portion of the ventricular myocardium depends on the right ventricle for the blood supply.

• #57

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

  The diagnostic echocardiograms were compared between the two groups along with outcomes related to final circulatory status, complications, number and type of reinterventions, and mortality. […] In our study we found that decompression of the right ventricle could be performed safe, with no associated mortality, using either catheter-based intervention or heart surgery. […] The findings of this retrospective population-based multicenter study demonstrate that both catheter-based intervention and heart surgery are safe procedures with a low risk of complications.

• #58 Pulmonary Atresia: Symptoms, Causes and Treatment

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

  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. […] Medical procedures and surgeries can improve your childs condition, but they arent cures. […] Your child will have regular follow-up appointments with their pediatric cardiologist. This starts two to four weeks after leaving the hospital. […] People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. They may have appointments at least every six months.

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