Tricuspid valve repair in hypoplastic left heart syndrome

Currently, the survival for the Norwood procedure for hypoplastic left heart syndrome is approximately 90% in selected centers. However, the development of tricuspid regurgitation remains a significant obstacle to successful staged repair in a subset of these patients. The results of tricuspid valve repair in this challenging patient population remain largely unknown.

Twenty-eight patients with significant (3-4+) tricuspid regurgitation after the Norwood procedure required tricuspid valve repair from August 1995 through December 2002. The clinical and Doppler-echocardiographic data were reviewed to determine the efficacy of repair and patient outcome.

Follow-up was 96% complete (27/28). Patients were divided into 2 groups on the basis of tricuspid regurgitation at late follow-up: those with a successful late outcome (0-2+) and those with a poor outcome (3-4+). There were 17 (63%) patients with a successful result and 10 (37%) with an adverse outcome. Age, weight, follow-up duration, valve anatomy, and stage of palliation were not significantly different between groups. Early postoperative 0 to 2+ regurgitation was associated with a durable result (P =.012) and preserved ventricular function (P =.04). Need for repair other than a partial annuloplasty was predictive of a poor outcome (P =.04). Overall survival was 67% (18/27). Survival was 94% (16/17) for patients with a successful late result versus 20% (2/10) for those with a poor outcome (P =.0002).

Tricuspid valve repair can be accomplished in this challenging patient population with excellent results. Successful tricuspid valve repair is predictive of continued good valve function and preserved right ventricular function. Successful valve repair at late follow-up predicts excellent late survival.