Outcome of Membranous Pulmonary Valve Atresia with Intact Ventricular Septum Treated by Cardiac Catheterization in a Developing Country

Abstract

Background: Pulmonary atresia with intact ventricular septum is a cyanotic heart disease with variable morphological features depending on the degree of right ventricular and tricuspid valve hypoplasia. Currently, the standard management for plate-like, membranous pulmonary atresia includes pulmonary valve perforation using radiofrequency wire and pulmonary valve dilation, with or without ductus arteriosus stenting. Radiofrequency wires are expensive and not available in many developing countries. We report our experience with perforation of the pulmonary valve using coronary wires followed by balloon dilation, with or without ductus arteriosus stenting.Methods: This is a retrospective review of patients with pulmonary atresia and intact ventricular septum who underwent initial percutaneous treatment at our institution between 2012 and 2020. Patients were followed till March 2022. We describe the baseline anatomy, procedure success and complications, and outcome.Results: Fifteen patients were treated during the study period. Pulmonary valve perforation using soft end of a hydrophilic-tip coronary wire was successful in 14 patients (93%). Ductus arteriosus stenting was performed in 10 patients. There was one post-procedure mortality due to neonatal sepsis. By the last follow up at median age of 34 months (21-100), twelve patients had 2-ventricular circulation, One with 1.5-ventricle circulation, and one with univentricular circulation. Conclusions: Perforation of atretic pulmonary valve using soft end of hydrophilic-tip coronary wire for patients with membranous pulmonary atresia and intact ventricular septum is highly successful. Most patients will end up with 2-ventricular circulation.