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

Abstract 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 palliation surgery from 2002-12. We explored patients' characteristics, anatomic and operative details associated with death, transplantation and AVV reoperation. Results Sixty patients received AVV repair during first-stage (n=10), Glenn (n=27), Fontan (n=23). Median age at AVV repair was 6.9 months (IQR 4.2-24.1). Underlying SV anomaly was HLHS (n=30), heterotaxy (n=15), other (n=15). The AVV was tricuspid (n=34), mitral (n=6), common (n=20). Pre-operatively, all patients had AVV regurgitation ≥ moderate and 7 (12%) had ventricular dysfunction ≥ moderate. Post-repair, AVV regurgitation was none/trivial (n=21, 35%), mild (n=21, 35%), ≥ moderate (n=17, 30%). Competing risks analysis showed that 10-years following 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=0.0011) and was 74%, 83% and 56% for tricuspid, mitral and common AVV repair (p=0.344). Factors associated with transplant-free survival were timing of AVV repair, underlying SV anomaly, and systemic ventricle function. Conclusions AVV repair at first-stage surgery 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.