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J Thorac Cardiovasc Surg 1999;117:1190-1211
© 1999 Mosby, Inc.

SURGERY FOR CONGENITAL HEART DISEASE

ATRIOVENTRICULAR DISCORDANCE: RESULTS OF REPAIR IN 127 PATIENTS

From the Division of Cardiovascular Surgery,^a Division of Cardiology,^b Hospital for Sick Children, Toronto Congenital Cardiac Centre for Adults,^c University of Toronto, Toronto, Ontario, Canada, and Division of Cardiothoracic Surgery, Kosair Children's Hospital,^d Department of Mathematics,^e University of Louisville, Jewish Hospital Heart & Lung Institute, Louisville, Ky.

Received for publication June 23, 1998. Revisions requested Dec 10, 1998. Revisions received Feb 16, 1999. Accepted for publication Feb 26, 1999. Address for reprints: Thomas Yeh, Jr, MD, PhD, University of Louisville, Jewish Hospital Heart & Lung Institute, 201 Abraham Flexner Way, Suite 1200, Louisville, KY 40202.

Objective: The conventional management of patients with atrioventricular discordance is directed at associated lesions, taking advantage of physiologic "correction"; however, the morphologic right ventricle and tricuspid valve support the systemic circulation. Questions surrounding survival using this approach led us to analyze our institutional results.
Methods: All patients with atrioventricular discordance undergoing biventricular repair were analyzed (n = 127, 1959-1997), excluding those with functionally univentricular hearts. The ventriculoarterial connection associated with atrioventricular discordance varied and was most commonly discordant (87%), but occasionally concordant (6%), double-outlet right ventricle (6%), or double-outlet left ventricle (1%). At initial presentation, the most common lesions associated with atrioventricular discordance were ventricular septal defect (86%), pulmonary stenosis (64%), tricuspid regurgitation (28%), and atrioventricular block (12%). Nine patients underwent a double switch procedure to create ventriculoarterial concordance and the remainder were managed conventionally without correcting discordant connections.
Results: Operative mortality was 6% and did not vary by associated lesion. Twenty years after repair, survival was 48%. Within 20 years, 56% of patients required reoperation, usually for atrioventricular valve incompetence (n = 16), pulmonary stenosis (n = 16), or both (n = 3). Pacemakers were required in 50 patients, 4 before repair, 40 within 2 months of repair, and 6 remotely after repair. In early follow-up, the double switch procedure (n = 9) had equivalent mortality and a high pacemaker requirement for atrioventricular block.
Conclusions: Analysis of conventional management of atrioventricular discordance revealed cumulative increases in mortality, systemic atrioventricular valve (tricuspid) replacement, complete atrioventricular block, and incidence of reoperation. Alternative management should be examined.

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