Surgical management of total anomalous pulmonary venous connection: Thirty-year trends

Background: Reports of surgical correction of total anomalous pulmonary venous connection (TAPVC) over the past 30 years indicate a general improvement in operative survival. However, prevention of late pulmonary venous obstruction continues to be a cornerstone of successful repair. The purpose of the study was to identify factors associated with improvement in perioperative mortality and to determine risk factors for death and reoperation due to pulmonary vein stenosis after repair of TAPVC. Methods and Results: Using univariate and multiple regression analysis, we analyzed risk of early and late mortality and need for reoperation in 105 patients operated on between April 1966 and June 1995. Despite increased frequency of neonatal repair in the most recent time period (29% in 1966 through 1985; 55% in 1991 through 1995, P<.05), operative mortality declined (13% in 1966 through; 1985; 0% in 1991 through 1995). The incidence of postoperative pulmonary hypertensive episodes and death related to pulmonary hypertension decreased significantly over the study period (P<.001). Aggressive preoperative elective medical stabilization and prophylaxis of postoperative pulmonary hypertensive episodes may have contributed to this improvement. By univariate analysis, preoperative pulmonary hypertension (P<.02) and preoperative pulmonary vein obstruction (P<.01) correlated with early mortality up to 1990 but not in the past 5 years. Multiple logistic regression analysis showed that only a small pulmonary confluence associated with diffuse pulmonary vein stenosis was an independent risk factor for early (P<.001) and late (P=.01) death as well as need for reoperation (P=.007). Type of TAPVC was not a significant risk factor throughout the three decades of our experience. At a median follow-up of 87 months, late survival was 98% (93 of 95 operative survivors), and all are NYHA class I. Conclusions: Improvement in surgical technique as well as preoperative and postoperative management account for the reduction in mortality and need for reoperation for most types of TAPVC. However, the presence of a small venous confluence and diffuse pulmonary vein stenosis remains a risk factor for adverse outcome.