Perventricular Closure of a Large Ventricular Septal Defect in Congenitally Corrected Transposition of the Great Arteries

We report the case of a 30 year-old male with congenitally corrected transposition of the great arteries, atrial, and ventricular septal defects (VSD), and pulmonary stenosis. He previously underwent three palliative surgical procedures before undergoing intracardiac repair at age 20 with a left ventricular to pulmonary artery (LV-PA) conduit, VSD closure, and replacement of the systemic atrioventricular valve. A residual VSD was noted postoperatively. He did well for approximately 10 years when he started becoming more breathless with daily activities and was noted to have a resting room air oxygen saturation of 85%. Despite increased diuretic therapy he continued to deteriorate and was ultimately admitted to the hospital in florid right and left heart failure with recurrent atrial fibrillation. Catheterization revealed pulmonary hypertension (pulmonary artery pressure = 80/17 mm Hg), moderate conduit stenosis, severe pulmonic regurgitation, and oxygen saturation of 75%. Calculated shunt fraction (Qp : Qs) was 1.3:1. He was referred for surgical intervention, specifically, LV-PA conduit replacement, oversewing of the pulmonic valve, VSD closure, and pacemaker placement. Intraoperatively, the VSD could not be closed despite multiple attempts through various approaches. Therefore, perventricular VSD closure using two Amplatzer septal occluders (AGA Medical, Golden Valley, MN) was performed in the operating room with the chest open off cardiopulmonary bypass. Following deployment, the residual shunt was small and the inferior vena cava-to-pulmonary artery saturation step-up was only 4%. The left ventricular systolic pressure decreased to one half systemic. This case highlights the utility and efficacy of a hybrid approach in the treatment of complex congenital heart disease.