Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation

BACKGROUND Total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC's impact on staged palliation outcomes.METHODS In a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (inter -quartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model.RESULTS Primary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syn-dromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was signifi-cantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P < .001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an in-dependent risk factor for mortality in patients with TAPVC (P < .001).CONCLUSIONS Overall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients. (Ann Thorac Surg 2023;115:1213-22) (c) 2023 by The Society of Thoracic Surgeons

Automated summary

This study aimed to evaluate the impact of total anomalous pulmonary venous connection (TAPVC) on the outcomes of staged palliation. The results showed that overall survival is lower in TAPVC single-ventricle patients compared to non-TAPVC patients, but TAPVC has less impact on survival after the second stage operation. Pulmonary venous obstruction (PVO) is identified as an independent risk factor for mortality in TAPVC patients.