Mid and Long-Term Surgical Outcomes for Functional Single Ventricles with Total Anomalous Pulmonary Venous Connection
Damien LaPar, Kyle Mitchell, Antonio Corno, Christopher Greenleaf, Peter Chen, Jon Meliones, Jorge Salazar
University of Texas McGovern Medical School, Houston, Texas, United States
Objectives: Patients with functional single ventricle anatomy with total anomalous pulmonary venous connection (SV/TAPVC) represent a rare and challenging surgical cohort. Limited series have evaluated operative outcomes for SV/TAPVC palliation. The purpose of this study was to better characterize mid- to long-term surgical outcomes in this high-risk patient population.
Methods: A total of 29 SV/TAPVC neonates (age 9 days [5, 21], weight 3.1 kg [2.9, 3.4]) underwent surgical repair and/or palliation over a 10-year study period (2011-2021). Univariate and Kaplan-Meier analyses evaluated operative and long-term outcomes.
Results: Median follow-up was 3.7 (0.2, 7.2) years. Nineteen (66%) patients presented with heterotaxy syndrome. The distribution of TAPVC anatomy included: supracardiac (55%, n=16), mixed (17%, n=5), infracardiac (14%, n=4), and cardiac (14%, n=4). Seven (24%) patients presented with obstructed TAPVC. Primary TAPVC repair occurred in 15 (52%) patients. Those without primary TAPVC repair underwent isolated systemic-PA shunting (n=8), Norwood (n=3), PA banding (n=2), or bidirectional Glenn (BDG, n=1) palliation. The overall mortality rate was 31% (n=9), and a total of 16 (55%) patients achieved Stage II BDG, 8 (28%) Stage III Fontan completion, and 2 (7%) underwent biventricular conversion. Patients presenting with obstructed TAPVC (71% vs 29%, P=0.01) and those requiring perioperative ECMO (67% vs. 33%, P=0.03) had a higher likelihood of death. The incidence of postoperative pulmonary vein stenosis (PPVS) among all patients was 17% (n=5), occurring at a median of 2.7 (2.5, 2.9) years. Kaplan-Meier estimates for overall survival was 72%, 68%, 68%, and 68% at 1-, 3-, 5-, and 10-years and was significantly higher for those with obstructed TAPVC (P=0.02, Figure). Moreover, the overall freedom from PPVS repair was 96%, 80%, 74%, and 74% at 1-, 3-, 5-, and 10 years (Figure).
Conclusions: Surgical outcomes for functional single ventricles with total anomalous pulmonary venous connection can be performed with good mid- to long- term survival in the modern surgical era. Obstructed TAPVC for these patients remains a critical “high-risk” feature. After initial palliation and TAPVC repair, select patients may be candidates for biventricular conversion.
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