Percutaneous treatment of pulmonary atresia with intact ventricular septum: Single-center experience

INTRODUCTION: Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare cyanotic congenital heart lesion that is managed with multiple interventions for palliation. This study aimed to evaluate the cases of neonatal PA/IVS followed in our clinic.
METHODS: This retrospective study was conducted at a pediatric cardiac center between January 2020 and December 2024. All patients with PA/IVS who were managed solely with catheterization-based interventions (valve perforation, pulmonary valvuloplasty, balloon atrial septostomy, or ductal stenting) as neonates were analyzed. Demographic, procedural, and clinical data were collected. The results were statistically evaluated.
RESULTS: During the study period, 22 neonates diagnosed with PA/IVS (n=11 male, median age 5 days [IQR 3–7 days], median weight 3.1 kg [2.9–3.3]) underwent heart catheterization. Four cases had tripartite, nine cases had bipartite, and nine cases had monopartite morphology. Three patients had coronary-dependent circulation, and nine cases had coronary artery fistula. Three patients underwent valve perforation and pulmonary balloon valvuloplasty; ten patients underwent valve perforation and pulmonary balloon valvuloplasty with a stent in the ductus arteriosus; four patients underwent ductus stenting + balloon atrial septostomy; and five patients underwent ductus arteriosus stent procedure. During the procedure, arrhythmia was observed in two cases; one case had pericardial tamponade and atrial fibrillation; one case had stent embolization; one case had mild pericardial effusion; and one case had temporary pulse loss. Three cases (13.6%) were lost within the first 30 days.
DISCUSSION AND CONCLUSION: The management of PA/IVS patients undergoing catheter-based neonatal interventions exhibits unique characteristics. Individualized management of these patients in the neonatal period may have a positive impact on outcomes.