985-55 Surgical Repair of Double Outlet Right Ventricle with Non-committed Ventricular Septal Defect

Abstract

Between January 1978 and June 1993, 32 patients presented for management of double outlet RV with non-committed VSD. 29 patients had definitive palliation or repair with 3 early and 1 late deaths (hospital mortality: 10.4%). The VSD type was muscular in 4, inlet in 12 and perimembranous but distant from both great arteries in 16 patients. Complicating anomalies with a major influence on management were present in 23 children (72%). These included aortic arch interruption (3), coarctation of the aorta (4), pulmonary stenosis (11), pulmonary atresia (2). multiple VSOs (3), AV discordance (2), left atrial isomerism (3), unbalanced ventricles (8) and criss-cross AV connection (2) with 11 children having more than one of these lesions. Initial palliation was performed in 20 patients and consisted of pulmonary artery banding in 8 (with repair of an interrupted aortic arch in 2), systemic-pulmonary artery shunt in 9 and repair of coarctation of the aorta in 3. Of these, 17 subsequently had definitive surgery: Fontan procedure in 9 and biventricular repair in 8 (arterial switch in one, intraventricular baffle in 7). 3 patients died following attempted biventricular repair. 12 patients underwent repair as the initial procedure: Fontan procedure in 2 and biventricular repair in 10 (arterial switch in 3 and intraventricular baffle in 7) with no deaths. Thus, of the 29 patients who had definitive surgery, 11 had Fontan procedures and 18 biventricular repair with no death in the Fontan group and 3 early deaths in the biventricular repair group (not statistically significant by Fischer's exact test, p = 0.27). Only 2 patients have required reoperations, both for residual VSDs. 9 of the Fontan procedures were undertaken in patients who had complex anatomy (e.g. AV discordance, unbalanced ventricles, multiple VSDs, atrial isomerism or pulmonary atresia), whereas 14 children of the biventricular repair group had non-complex anatomy (p < 0.005). Surgery for double outlet RV with non-committed VSD needs to be tailored to individual patients, but biventricular repair was achieved in a substantial majority of patients with non-complex anatomy with relatively low risk and a low incidence of reoperations.