Subpulmonary obstruction in congenitally corrected transposition of the great arteries due to ventricular membranous septal aneurysms

Abstract

The clinical, hemodynamic, and angiographic observations, as well as the surgical approach used for repair in three patients with congenitally corrected transposition of the great arteries and ventricular membranous septal aneurysms, are presented. In two of the three patients the membranous septal aneurysm caused subpulmonary obstruction, with 94 and 125 mm Hg systolic gradients. In each patient the aneurysm was demonstrated by angiocardiography, which also showed differences in size and shape with cardiac systole and diastole. Review of the previously described reports indicates that patients with congenitally corrected transposition often display various forms of pulmonary outflow obstruction and when a ventricular membranous septal aneurysm exists, a significant subpulmonary obstruction is present in most patients. The unique anatomic relationship between the pulmonary artery and a ventricular membranous septal aneurysm in patients with transposition of the great arteries with and without atrioventricular discordance explains why subpulmonary obstruction sometimes develops.