Abstract

Introduction: In single ventricle patients, AV valve regurgitation increases the risk of adverse outcomes, and staged palliation with concomitant AV valve intervention further increases that risk in the post-operative period. Long-term outcomes of valve intervention during stage 2 palliation, however, have not been established. The purpose of this study is to evaluate long-term outcomes after AV valve intervention during stage 2 palliation in patients with RV dominant circulation in a multicenter study utilizing a national public-use database. Methods: The study was performed using the SVR and SVR II public use datasets. From the original cohort of 549 patients, 102 (19%) with moderate or severe tricuspid valve regurgitation as measured by their stage 2 pre-operative core lab echocardiogram were included. Kaplan-Meier curves were performed to describe the association between valve regurgitation and intervention and long-term survival up to 6 years post Fontan. Multivariable Cox proportional-hazards models were used to assess the relationship between valve intervention and survival adjusting for confounding covariates. Results: Patients with moderate or severe valve regurgitation at either stage 1 or 2 had worse long-term survival defined as death or transplant up to 6 years post-Fontan operation ( p < 0.001, p < 0.01). There were 34 patients (33%) of the 102 with significant regurgitation who underwent valve intervention at the time of stage 2 repair. Those who underwent valve intervention at stage 2 surgery were significantly more likely to die or undergo heart transplantation (HR 3.49, CI 1.53-7.94). Of the patients who underwent valve intervention, those who survived were more likely to have none or mild valve regurgitation by the time of stage 3 (78% vs 46%, p = 0.025). Patients with moderate to severe AV valve regurgitation at the time of the Fontan had favorable outcomes regardless of valve intervention with 100% survival. Conclusions: The risks associated with AV valve regurgitation in single ventricle patients are not mitigated by valve intervention at the time of stage 2 palliation. In fact, patients who underwent valve intervention at stage 2 had significantly worse survival as compared to patients with AV valve regurgitation who did not.

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