Outcomes of staged repair following systemic-to-pulmonary artery shunt in pulmonary atresia with ventricular septal defect.

Abstract

The study objective was to evaluate outcomes of staged repair after systemic-to-pulmonary artery shunt in patients with pulmonary atresia, ventricular septal defect, and ductus-dependent pulmonary circulation, focusing on the morphology and clinical impact of pulmonary artery coarctation. Between January 2014 and July 2024, 87 patients with pulmonary atresia, ventricular septal defect, and ductus-dependent pulmonary circulation who underwent systemic-to-pulmonary artery shunt as initial palliation followed by biventricular repair were retrospectively analyzed. Pulmonary artery anatomy was assessed using serial computed tomography angiography before systemic-to-pulmonary artery shunt and biventricular repair. The pulmonary artery coarctation ratio was defined as the coarctation segment diameter divided by the ipsilateral pulmonary artery diameter. Pulmonary artery coarctation-related adverse outcomes were defined as reintervention or significant hypoplasia of the affected pulmonary artery. The median age and body weight at systemic-to-pulmonary artery shunt were 24 days and 3.4 kg, respectively. There was 1 early (1.1%) and 1 interstage (1.1%) death. Competing risk analysis showed that the probability of biventricular repair at 18 months after systemic-to-pulmonary artery shunt was 97.7%. Overall survival was 94.9% at 10 years. Both branch pulmonary arteries demonstrated significant growth after systemic-to-pulmonary artery shunt (P < .001). The pulmonary artery coarctation ratio decreased from 0.77 to 0.63, whereas the coarctation segment diameter increased from 2.85 to 4.20 mm before biventricular repair (P < .001). Pulmonary artery coarctation-related adverse outcomes occurred in 10 patients (11.8%). On multivariable analysis, a right aortic arch was an independent predictor of pulmonary artery coarctation-related adverse outcomes (odds ratio, 5.95; P = .02). Staged repair after systemic-to-pulmonary artery shunt in pulmonary atresia, ventricular septal defect, and ductus-dependent pulmonary circulation achieves excellent survival. Pulmonary artery growth is preserved despite pulmonary artery coarctation. However, close surveillance is warranted given the potential need for reintervention.