Repair of major intracardiac anomalies associated with atrioventricular discordance.

Thirty-five patients with atrioventricular (AV) discordance underwent repair of major intracardiac defects. Ventricular septal defect (VSD) was the most frequently encountered lesion, present alone or in combination with other lesions in 86% of these patients. Pulmonary stenosis (51%) and tricuspid insufficiency (37%) were the other lesions encountered. The initial operative mortality of 8.6% is approaching that for these same defects when repaired in the patient with normal atrioventricular connections. However, the late mortality of 19% with an average follow-up of 4.4 years is higher than expected in the absence of AV discordance. Reoperation for residual or recurrent defects was required in 9 of the 32 survivors and was associated with a mortality of 33%. Seven of the 9 reoperations were required for tricuspid valve dysfunction. Elective repair of major anomalies in association with AV discordance can be accomplished safely, but these patients require careful long-term follow-up in anticipation of late problems.