Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot (see this term).
The presence of an abnormality of cardiac function that is responsible for the failure of the heart to pump blood at a rate that is commensurate with the needs of the tissues or a state in which abnormally elevated filling pressures are required for the heart to do so. Heart failure is frequently related to a defect in myocardial contraction.
Survival into sixth decade of untreated pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals: a magnetic resonance imaging study.
Ratti C, Grassi L, Ligabue G, Della Casa G, Barbieri A, Gavioli M, Bompani B. J Cardiovasc Med (Hagerstown). 2009;10(7):570-1.
A 56-year-old male with a history of untreated pulmonary atresia with ventricular septal defect diagnosed during childhood was referred to our institution for congestive heart failure.
