Outcomes of Surgery for Mixed Total Anomalous Pulmonary Venous Drainage in Children

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average follow-up after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair.