Impact of obstruction in total anomalous pulmonary venous connection: surgery morbidity, mortality, and survival

Introduction and objectives: Total anomalous pulmonary venous connection is a rare condition with a wide anatomical spectrum and a favorable prognosis, except in the presence of pulmonary venous obstruction. The objective was to identify factors associated with operative mortality in patients undergoing surgical correction. Methods: A retrospective analysis was conducted on patients operated on between 2013 and 2022. The primary outcome was operative mortality, while the secondary outcome was the presence of major complications. Survival at 1 and 5 years after discharge was also evaluated. Logistic regression was used to identify preoperative and intraoperative risk factors for operative mortality, and Kaplan-Meier survival curves and a Cox model were employed to assess factors associated with 1-year survival. Results: A total of 74 patients were included (56.8% male, 59.5% with supracardiac drainage). Operative mortality was 16.2% (95%CI, 8.7-26.6), higher in patients with obstruction at diagnosis (36.6% vs 4.5%). Patients with obstruction more frequently had infracardiac drainage, lower age and weight, increased need for mechanical ventilation and vasoactive support, and longer extracorporeal circulation times. The reintervention rate was 21.6%. Overall survival was 88.7% at one year (95%CI, 77.7-94.4) and 85.4% at 5 years (95%CI, 73.9-92.1). Survival was lower in patients with residual obstruction (37.5% at 1 year, 95%CI, 8.7-67.4; 25% at 5 years, 95%CI, 3.7-55.8; HRa, 47.4, 95%CI, 3.77-594.9). Conclusions: Survival in patients with surgically corrected total anomalous pulmonary venous connection at our institution is comparable to international data, but outcomes are worse in those with obstruction at diagnosis.