pulmonary atresia
Pronunciation: PUL-mo-nar-ee uh-TREE-zhuh
Congenital heart defect where the pulmonary valve fails to develop properly, blocking blood flow from the right ventricle to the lungs.
Full Definition
Pulmonary atresia is a congenital heart defect characterized by the absence or severe underdevelopment of the pulmonary valve, preventing normal blood flow from the right ventricle to the pulmonary arteries. This condition can occur with an intact ventricular septum (PA-IVS) or with a ventricular septal defect (PA-VSD). Infants with pulmonary atresia are typically cyanotic and depend on alternative pathways for pulmonary blood flow, such as a patent ductus arteriosus or collateral circulation. The condition requires immediate cardiac intervention and often involves a series of staged surgical procedures to establish adequate pulmonary circulation.
Usage
Usage note: Specify whether associated with intact ventricular septum (IVS) or ventricular septal defect (VSD).
In Context
- "The newborn was diagnosed with pulmonary atresia with intact ventricular septum requiring emergency cardiac catheterization." — Cardiology consultation
- "Pulmonary atresia with VSD typically requires a different surgical approach than the intact septum variant." — Surgical planning note