Outcomes of different rehabilitative procedures in patients with pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries

Abstract

Objectives: The objective of this study was to compare our clinical outcomes of the central shunt and the right ventricle-pulmonary artery (RV-PA) connection in patients with pulmonary atresia, ventricular septal defect and the major aortopulmonary collateral arteries.

Methods: From November 2009 to October 2017, a total of 157 consecutive patients with pulmonary atresia, ventricular septal defect, the major aortopulmonary collateral arteries and the hypoplastic PAs who underwent palliative surgery were included. Seventy patients underwent the central shunt (the central shunt group) and 87 patients underwent the RV-PA connection (the RV-PA group). Propensity score matching was used to create 2 cohorts with similar baseline characteristics: 56 central shunt patients were one-to-one-matched with 56 RV-PA connection patients. The early and late outcomes were compared.

Results: The median duration of follow-up was 18 months in the central shunt group and 22 months in the RV-PA group (P = 0.10). The probability of complete repair was significantly lower in the central shunt group as compared with the RV-PA group (P = 0.048). The Kaplan-Meier estimates of complete repair rates were 47.2 ± 10.0% after 3 years and 56.0 ± 11.6% after 5 years in the central shunt group, which were lower as compared with 62.3 ± 7.6% after 3 years and 74.5 ± 7.2% after 5 years in the RV-PA group. The increase in the mean McGoon ratio and the mean Nakata index were significantly lower in the central shunt group than those in the RV-PA group (0.57 ± 0.52 vs 1.02 ± 0.44, P = 0.036; 98.2 ± 35.1 mm2/m2 vs 176.9 ± 85.4 mm2/m2, P = 0.025, respectively). The in-hospital morbidity and mortality after complete repair were similar between 2 groups.

Conclusions: Compared with the central shunt, the RV-PA connection appears to be a more effective palliative procedure to improve the probability of complete repair and PA growth in patients with pulmonary atresia, ventricular septal defect and the major aortopulmonary collateral arteries, in whom primary repair is not feasible.

Keywords: Central shunt; Major aortopulmonary collateral artery; Pulmonary atresia; Rehabilitation; Right ventricle to pulmonary artery connection.