Total anomalous pulmonary venous connection repair; risk factors and outcome

BackgroundRepair of total anomalous pulmonary venous connection (TAPVC) is associated with high mortality and morbidity. Age of presentation, low birth weight, type of TAPVC, pre-operative infection, clinical situation, pulmonary venous obstruction, pulmonary hypertension and associated lesions are very important factors affect the outcome of TAPVC repair.MethodsSeventy patients underwent TAPVC repair from 2000 to 2012 with a median age of 33 days and mean weight of 4.6kg. Twenty-six patients (48.6%) had supracardiac type, 23 patients (32.8%) had intracardiac type, and nine patients (8.6%) had infracardiac type, while seven patients (10%) had mixed TAPVC.ResultsOperative mortality was 5.7% (4 patients), while late deaths was 4.3% (3 patients). Four patients out of these 7 deaths were due to pulmonary hypertensive crises (4/7, 57.1%). Obstruction and single-ventricle physiology were associated with a higher mortality rate (3/7, 42.8%).Mean CPB time was 104.3min (range, 70–190min) and mean cross-clamp time was 42.4min (range, 29–70min). Three patients had recurrent pulmonary venous obstruction. (4.3%) which required early re-intervention.ConclusionMortality rate in TAPVC repair is higher in those patients who had single-ventricle physiology or pulmonary venous obstruction at time of repair. Pulmonary hypertension is another risk factor affecting the post-operative course of infants with TAPVC, particularly patients with obstructed, infracardiac TAPVC