Longest Survivor of Pulmonary Atresia With Ventricular Septal Defect

A 59-year-old woman was admitted because of cyanosis and dyspnea on exertion and at rest. In her childhood, she was suspected of having ventricular septal defect (VSD), but she refused to undergo cardiac catheterization and operation. Dyspnea on exertion gradually developed after adolescence. On admission, chest roentgenography demonstrated enlarged cardiac silhouette with elevated cardiac apex, a right aortic arch, and enlargement of the main pulmonary arteries and their major branches with increased pulmonary arterial vascularity (Figure 1). Echocardiography revealed a large VSD which lay beneath the dilated aorta that overrides the interventricular septum, hypertrophied right ventricle, and the blind outflow tract of the right ventricle …