BACKGROUND: Pulmonary atresia with ventricular septal defect is a rare and complex congenital heart disease. In cases where pulmonary blood flow is supplied exclusively by major aortopulmonary collateral arteries, traditional surgical interventions may be challenging or delayed, especially in resource-limited settings. This study evaluated the feasibility, safety, and outcomes of the right ventricular outflow tract perforation through the retrograde trans-collateral approach in patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries dependent pulmonary circulation. METHODS: The study cohort comprised 10 patients with pulmonary atresia and ventricular septal defect who underwent attempted retrograde trans-collateral right ventricular outflow tract perforation via major aortopulmonary collateral arteries from October 2021 to February 2025, including 1 unsuccessful procedure. RESULTS: The median age at intervention was 4.1 years, and the median weight was 17 kg. Post-procedure, systemic oxygen saturation increased significantly ( P <0.01). Follow-up imaging demonstrated substantial growth of the pulmonary arteries following retrograde trans-collateral right ventricular outflow tract recanalization, with significant improvements in both right and left pulmonary artery Z scores ( P <0.01) and a significant increase in the Nakata index from a median of 49 to 111.7 mm 2 /m 2 ( P <0.01). CONCLUSIONS: Retrograde trans-collateral right ventricular outflow tract perforation is a feasible and safe catheter-based strategy for selected patients with pulmonary atresia with ventricular septal defect, promoting central pulmonary artery growth and serving as a bridge to future surgical repair.
AbdelSalam et al. (Thu,) studied this question.