Subpulmonary Obstruction from Aneurysmal Ventricular Septum in a Child with Dextrocardia and Congenitally Corrected Transposition of the Great Arteries

Abstract

A 3-year-old boy underwent evaluation for dextrocardia. Echocardiograms showed features of corrected transposition physiology, a perimembranous ventricular septal defect (VSD) (Fig. 1), and aneurysmal tissue beneath the pulmonary valve that caused severe subpulmonary stenosis (Figs. 2–4). Eighteen months after VSD closure and resection of the aneurysmal tissue, the patient was asymptomatic with only mild residual pulmonary outflow tract obstruction. Fig. 1. Two-dimensional echocardiograms A) in apical 4-chamber view from the right chest and B) in color-flow Doppler mode show a moderate-to-large ventricular septal defect (VSD). Fig. 2. Echocardiograms (subcostal 4-chamber views). A) In the morphologic left ventricle (MLV), aneurysmal tissue (arrow) protrudes into the pulmonary outflow tract. B) Color-flow Doppler mode reveals turbulent flow (arrow) in the pulmonary outflow tract. Fig. 3. A) Continuous-wave Doppler echocardiographic recording across the pulmonary outflow tract shows a peak velocity >5 m/s, suggesting severe obstruction. B) Angiogram (60° left anterior oblique view) from the morphologic left ventricle (MLV) ... Fig. 4. Cineangiographic image from the A) left lateral view reveals the morphologic left ventricle (MLV) and the pulmonary outflow tract obstruction from aneurysmal tissue (arrows). B) The 60° left anterior oblique view reveals a severely narrowed right ...