Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot

Objective: The objective was to explore the best management algorithm for a trioventricular septal defect in conjunction with tetralogy of Fallot. Meth ods: We reviewed the cases of 38 children referred to our division (March 1 981-August 1997) who had atrioventricular septal defect associated with tet ralogy of Fallot; 32 (84%) had Down syndrome. Twenty-one received initial p alliation with a systemic-to-pulmonary artery shunt; of these, 2 (9.5%) die d before complete repair. Thirty-one children underwent complete repair; 14 of these (45%) had undergone initial palliation (mean age at shunt 20 +/- 24 months), Right ventricular outflow obstruction was relieved by a transan nular patch in 22 (71%); 14 (64% of 22) had a monocuspid valve inserted. Fo ur required an infundibular patch. Results: Two children (6.4%) died early after repair; 1 had undergone previous palliation, Patients with palliation underwent repair at an older age (78 vs 36 months), required longer ventil atory support (8 vs 4 days) and inotropic support (8 vs 4 days), and had lo nger intensive care stays (II vs 6 days) and hospital stays (24 vs 15 days) , Eleven children (35%) underwent reoperation, 7 (58%) for right ventricula r outflow reconstruction and pulmonary arterioplasty. Reoperation was more frequent in the palliation group than in the primary operation group (64% v s 12%), The single Late death was related to a reoperation in the palliatio n group. Conclusions: Atrioventricular septal defect with tetralogy of Fall ot can be repaired with a low mortality rate. Initial palliation with a shu nt resulted in a more complex postoperative course and a higher reoperative rate. Primary repair is superior to initial palliation with later repair.