Heterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown.

From July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Children’s Hospital Japan with significant (3–4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome.

Patients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0–2) and those with a poor outcome (3–4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage.

Atrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.