To describe the early and midterm outcome after atrio-ventricular valve (AVV) procedure in patients with univentricular hearts (UVH) and to identify risk factors for AVV reoperation and death.

Retrospective review of patients undergoing procedure for AVV regurgitation at any phase of univentricular palliation from 1998-2013. Patient and procedure related variables were analyzed.

28 consecutive patients underwent 34 procedures for moderate+ AVV regurgitation at a median age of 3.7 years. 29% of patients had a common, 25% had dominant left, 14% had dominant right AVV and 32% two AVV. All patients benefited from valve repair at first procedure without early mortality. At hospital discharge patients preserved their ventricular function (FS <30%: preop 17% vs. postop 21%, NS) and only 14% had moderate+ residual regurgitation. Dominant left AVV and postoperative moderate+ regurgitation were univariate risk factors for death and transplantation. Younger age, need for repair before superior cavo-pulmonary shunt and significant residual regurgitation were univariate risk factors for AVV reoperation. Freedom from death and transplantation was 84% (CI 95%±0.14) at 5 and 10 years. Survival free from AVV reoperation was 77% (CI 95%±0.18) at 5 years and 66% at 10 years (CI 95±0.25). At last visit, 91% of survivors were in class NYHA 1-2 without ventricular dysfunction and with mild or less AVV regurgitation.

Patients with UVH and moderate+ atrio-ventricular valve regurgitation can profit from AVV repair without deterioration of their ventricular function but remain at increased risk for death and AVV reoperation.