Outcomes of total anomalous pulmonary venous drainage and predictors of mortality—Tertiary center experience

Abstract

BackgroundTotal anomalous pulmonary venous drainage (TAPVD) is a rare congenital anomaly associated with a high mortality rate, especially when associated with complex congenital heart disease (CHD). ObjectivesTo present the experience in managing TAPVD patients, along with mid to long-term outcomes. MethodsThis is a retrospective study, conducted between 2011 to 2020, aiming to document outcomes and identify predictors for mortality. ResultsA total of forty-nine patients were included. The mean follow-up time, median gestational age, weight and age at operation were 4.6 years (± 2.3 years), 38 weeks (32-42 weeks), 3.2 kilograms (1.8-50 kg) and 17 days (0-8395 days), respectively. Thirty-four patients had isolated disease while 15 patients had complex TAPVD (10 univentricular). Pre- and postoperative pulmonary venous obstruction (PVO) were documented at 24.5% and 21%, respectively. The mortality rate was at 16% and highly associated with the complex group (p = 0.000). Multivariate analysis revealed univentricular physiology (SV), cardiopulmonary bypass time, rectal temperature and open chest as the only independent risk factors for early mortality. ConclusionOutcomes were excellent in patients with the isolated type, but mortality remained high within the complex group, except for a subset of patients with unobstructed, cardiac-type TAPVD associated with Heterotaxy and SV physiology. Death continues to be associated with previously identified risk factors along with operative rectal temperatures and a postoperative open chest. PVO was not identified as a risk factor for death, except in univariate analysis, with good outcomes following secondary repair to relieve the obstruction. Overall survival beyond hospital discharge offers excellent outcomse.