Work

Pulmonary vasomodulators in adults with repaired pulmonary atresia with ventricular septal defect: Single-center experience and review of literature

Year: 2025

Type: article

Abstract: Despite repair, many patients with pulmonary atresia with ventricular septal defect (PA-VSD) develop pulmonary hypertension (PH). We aimed to evaluate the effectiveness and safety of pulmonary vasomodulators in patients with PH and PA-VSD. Retrospective analysis of adults with repaired PA-VSD and PH treated with pulmonary vasomodulator therapies from 2003-2022. Clinical improvement was defined as a decrease in New York Heart Association Functional Class by at least one point. Adverse effects were defined by therapy discontinuation. In total, 12 adults (47 [29-67] years; 75% female), with pre-treatment functional class of 3 (2-4) were included. Cardiac anatomy and surgical history was heterogeneous though most patients had non-confluent pulmonary arteries at birth and residual lesions post repair. Pre-treatment mean values for pulmonary artery pressure, pulmonary artery wedge pressure, and transpulmonary gradient were 41 (29-838), 17 (6-34), and 25 (16-55) mmHg, respectively. Pre-treatment cardiac index was 1.9 (1.1-3.1) L/min/m 2 . Phosphodiesterase-5 inhibitors were the most frequently prescribed therapy, and 50% of patients were treated with at least 2 classes of medications. During the 3 (0.2-15.5) years of follow-up, 4 patients died, 1 was status post heart/lung transplant, and 7 survived. Adverse effects occurred in 5 patients, with 2 experiencing pulmonary edema potentially related to therapy. For patients who survived without transplant, post-treatment functional class was 2 (2-3), and 5 experienced clinical improvement. Patients who survived without transplant were treated for 7.5 (3-15.5) years. This study suggests relative safety and potential benefit of pulmonary vasomodulator therapies in adults with PH and PA-VSD. • Pulmonary atresia with VSD is associated with segmental and post-capillary PH. • Patients with PA-VSD and PH have high morbidity and mortality. • Pulmonary vasomodulators can reduce symptoms from PH in PA-VSD. • Pulmonary vasomodulators are safe in PH due to PA-VSD.

Source: International Journal of Cardiology Congenital Heart Disease

DOI: 10.1016/j.ijcchd.2025.100643