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J Thorac Cardiovasc Surg 1998;116:905-913
© 1998 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

PRIMARY REPAIR IS SUPERIOR TO INITIAL PALLIATION IN CHILDREN WITH ATRIOVENTRICULAR SEPTAL DEFECT AND TETRALOGY OF FALLOT

From the Division of Cardiovascular Surgery, Department of Surgery,^a and the Division of Paediatric Cardiology, Department of Paediatrics,^b The Hospital for Sick Children and the Faculty of Medicine, University of Toronto,^a,b Toronto, Ontario, Canada.

Read at the Seventy-eighth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass, May 3-6, 1998.

Received for publication May 20, 1998. Revisions requested July 8, 1998; revisions received July 29, 1998. Accepted for publication Aug 10, 1998. Address for reprints: Glen Van Arsdell, MD, Cardiovascular Surgery, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada.

Objective:The objective was to explore the best management algorithm for atrioventricular septal defect in conjunction with tetralogy of Fallot.
Methods: We reviewed the cases of 38 children referred to our division (March 1981–August 1997) who had atrioventricular septal defect associated with tetralogy of Fallot; 32 (84%) had Down syndrome. Twenty-one received initial palliation with a systemic-to-pulmonary artery shunt; of these, 2 (9.5%) died 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 transannular patch in 22 (71%); 14 (64% of 22) had a monocuspid valve inserted. Four 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 ventilatory support (8 vs 4 days) and inotropic support (8 vs 4 days), and had longer intensive care stays (11 vs 6 days) and hospital stays (24 vs 15 days). Eleven children (35%) underwent reoperation, 7 (58%) for right ventricular outflow reconstruction and pulmonary arterioplasty. Reoperation was more frequent in the palliation group than in the primary operation group (64% vs 12%). The single late death was related to a reoperation in the palliation group.
Conclusions: Atrioventricular septal defect with tetralogy of Fallot can be repaired with a low mortality rate. Initial palliation with a shunt resulted in a more complex postoperative course and a higher reoperative rate. Primary repair is superior to initial palliation with later repair.

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