Pulmonary atresia is a congenital heart defect in which the pulmonary valve fails to form properly, resulting in complete obstruction of blood flow from the right ventricle to the pulmonary artery. Without a functioning pulmonary valve, blood cannot reach the lungs through the normal route to pick up oxygen.
The condition varies in severity depending on how much of the right-sided structures are affected. In pulmonary atresia with intact ventricular septum, the right ventricle is often small and underdeveloped. In pulmonary atresia with ventricular septal defect, the anatomy overlaps with tetralogy of Fallot and may be associated with abnormal pulmonary arteries. In both forms, survival after birth depends on alternative pathways for blood to reach the lungs, typically a patent ductus arteriosus, which normally closes shortly after birth.
Pulmonary atresia is a critical congenital heart defect requiring intervention in the neonatal period. Treatment typically begins with a prostaglandin infusion to keep the ductus arteriosus open, followed by surgical or catheter-based procedures. The specific surgical approach depends on the anatomy and may involve staged palliative surgery or, in suitable cases, complete repair. Many patients require further procedures as they grow.
Adults who have had surgical treatment for pulmonary atresia in childhood are cared for by Adult Congenital Heart Disease (ACHD) specialist services. They may be at elevated risk of arrhythmia and require lifelong cardiac monitoring. See also: Congenital Heart Disease.
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