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.
Yeh T., J., Connelly, M. S., Coles, J. G., Webb, G. D., McLaughlin, P. R., Freedom, R. M., … Williams, W. G. (1999). Atrioventricular discordance: Results of repair in 127 patients. Journal of Thoracic and Cardiovascular Surgery, 117(6), 1190–1203. https://doi.org/10.1016/S0022-5223(99)70259-X