Pulmonary atresia and intact ventricular septum complicating corrected transposition of the great vessels

Abstract

A three and one-half month old infant with corrected transposition of the great vessels, pulmonary atresia, and intact ventricular septum is presented. Cardiac catheterization and angiocardiography disclosed a small blind right-sided pulmonary (anatomic left) ventricle with systolic pressure exceeding systemic levels. The chamber communicates with the coronary arterial system via transmyocardial sinusoids. To our knowledge, this is the first report of this syndrome complex diagnosed during life.