Effect of Atrioventricular Valve Repair on Multistage Palliation Results of Single-Ventricle Defects

Background: The presence of significant atrioventricular valve (AVV) regurgitation results in unfavorable conditions that affect the success of single-ventricle (SV) multistage palliation. We report our institution's AVV repair experience.

Methods: We examined incidence of AVV repair in 603 infants who underwent initial SV palliative surgery from 2002 to 2012. We explored patients' characteristics and anatomic and operative details associated with death, transplantation, and AVV reoperation.

Results: AVV repair was performed in 60 patients during first-stage (n = 10), Glenn (n = 27), and Fontan (n = 23). Median age at AVV repair was 6.9 months (interquartile range, 4.2-24.1 months). Underlying SV anomaly was hypoplastic left heart syndrome (n = 30), heterotaxy (n = 15), and other (n = 15). The AVV was tricuspid (n = 34), mitral (n = 6), or common (n = 20). Preoperatively, all patients had AVV regurgitation moderate or higher, and 7 (12%) had ventricular dysfunction moderate or higher. After the repair, AVV regurgitation was none/trivial in 21 (35%), mild in 21 (35%), and moderate or higher in 17 (30%). Competing risks analysis showed that 10 years after AVV repair, 18% of patients had undergone AVV reoperation, 26% had died or undergone transplantation, and 56% were alive without subsequent reoperation. Transplant-free survival was 38%, 65%, and 100% for AVV repair at first-stage, Glenn, or Fontan (P = .0011), respectively, and was 74%, 83%, and 56% for tricuspid, mitral, and common AVV repair (P = .344), respectively. Factors associated with transplant-free survival were timing of AVV repair, underlying SV anomaly, and systemic ventricle function.

Conclusions: AVV repair at the first-stage operation and reduced systemic ventricle function are associated with poor outcomes. In those high-risk patients, different approaches that involve initial palliation mode, timing of AVV repair, or listing for transplantation might be warranted.