Atrioventricular discordance: Results of repair in 127 patients

Objective: The conventional management of patients with atrioventricular di scordance is directed at associated lesions, taking advantage of physiologi c "correction"; however, the morphologic right ventricle and tricuspid valv e support the systemic circulation. Questions surrounding survival using th is approach led us to analyze our institutional results. Methods: All patie nts with atrioventricular discordance undergoing biventricular repair were analyzed (n = 127, 1959-1997), excluding those with functionally univentric ular hearts. The ventriculoarterial connection associated with atrioventric ular discordance varied and was most commonly discordant (87%), but occasio nally concordant (6%), double-outlet right ventricle (6%), or double-outlet left ventricle (1%), At initial presentation, the most common lesions asso ciated with atrioventricular discordance were ventricular septal defect (86 %), pulmonary stenosis (64%), tricuspid regurgitation (28%), and atrioventr icular block (12%), Nine patients underwent a double switch procedure to cr eate ventriculoarterial concordance and the remainder were managed conventi onally without correcting discordant connections, Results: Operative mortal ity 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 stenosi s (n = 16), or both (n = 3), Pacemakers were required in 50 patients, 4 bef ore repair, 40 within 2 months of repair, and 6 remotely after repair. In e arly follow-up, the double switch procedure (n = 9) had equivalent mortalit y and a high pacemaker requirement for atrioventricular block, Conclusions: Analysis of conventional management of atrioventricular discordance reveal ed cumulative increases in mortality, systemic atrioventricular valve (tric uspid) replacement, complete atrioventricular block, and incidence of reope ration. Alternative management should be examined.