Pulmonary Atresia and Ventricular Septal Defect in a Thirty-Six Year Old Woman

Abstract

Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare form of congenital heart disease. The blood supply to the pulmonary arteries is provided by a patent arterial duct or by major aorto-pulmonary collateral arteries (MAPCAs). Here we present a thirty six years old Syrian war victim who was referred to our clinic with complaints of dyspnea on exertion and syncope. When questioned she admitted that she was having these complaints since her childhood and had a disease that she was offered cardiac catheterization and operation which she refused. Echocardiographic and computed tomography angiography findings revelaed pulmonary atresia with ventricular septal defect and aortopulmonary collateral arteries provided the blood flow to pulmonary arteries. Most patients without surgery do not live more than three decades. The presented case is one of those surviving more than three decades without surgery.