What to expect after repair of total anomalous pulmonary venous connection: data from 193 patients and 2902 patient years

Total anomalous pulmonary venous connection (TAPVC) occurs as isolated cases, in combination with single ventricle physiology, and may be complicated by pulmonary venous obstruction. We sought to identify potential risk factors for long-term mortality and reoperations. Data from 193 consecutive patients who had undergone repair of TAPVC between 1974 and 2011 were analysed using multivariate Cox regression. Mean follow-up time was 15.0 +/- 11.0 years, 95% complete. Survival was 82.7 +/- 2.9% at 20 years. Single ventricle physiology (5.9% of the patients, P < 0.001) emerged as the only significant risk factor for mortality in multivariate analyses. Freedom from cardiac reoperation was 82.2 +/- 3.3% at 20 years. Single ventricle physiology (P < 0.001) was the only risk factor for cardiac reoperations in multivariate analyses. Freedom from reoperations for pulmonary venous obstruction was 90.4 +/- 2.5% at 20 years. An age at operation of < 30 days (52.8% of the patients, P = 0.007) was the only risk factor for reoperations for pulmonary venous obstruction in univariate analyses. In patients with isolated TAPVC (n = 177), preoperative pulmonary venous obstruction (53.7% of the patients, P = 0.030) and deep hypothermic circulatory arrest (78.5% of the patients, P = 0.017) emerged as risk factors for mortality in univariate analyses. An age at operation of < 30 days (53.7% of the patients, P = 0.022) was the only risk factor for reoperations for pulmonary venous obstruction in univariate analyses. Survival into the third decade without reoperations is excellent in patients with isolated TAPVC without preoperative pulmonary venous obstruction, irrespective of the type of anomalous connection. In contrast, survival of patients with TAPVC and single ventricle physiology is among the poorest of all congenital heart defects. Reoperations for pulmonary venous obstruction are rare and are predominantly required in patients who were operated on as neonates. Survival may be improved by using a strategy of low-flow cardiopulmonary bypass.