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Pulmonary atresia with an intact ventricular septum (PA/IVS) is a rare serious cardiac birth defect. The condition involves complete obstruction of flow across the pulmonary valve (pulmonary atresia) combined with an intact wall between the lower pumping chambers.
Absence of forward flow across the pulmonary valve results in limited blood flow through the right side of the heart and underdevelopment of the right heart structures (tricuspid valve and right ventricle).
In some babies with PA/IVS, there may be abnormalities of the coronary arteries (the arteries which supply the heart muscle with blood).
Babies with PA/IVS can be identified before birth with prenatal ultrasound.
Delivery of a baby with PA/IVS is recommended at a hospital that is prepared for high-risk deliveries with neonatal intensive care, pediatric cardiology and pediatric cardiothoracic surgery services. Babies with PA/IVS require surgical correction in the newborn period.
Cardiology - Pediatric, Pediatrics
Radiology - Pediatric, Radiology