ArticleInternational journal of cardiology. Congenital heart disease2026
Pulmonary vasomodulators in adults with repaired pulmonary atresia with ventricular septal defect: Single-center experience and review of literature.
Article in International journal of cardiology. Congenital heart disease, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: 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. Methods: Retrospective analysis of adults with repaired PA-VSD and PH treated with pulmonary vasomodulator therapies from 2003 to 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. Results: 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 Conclusions: This study suggests relative safety and potential benefit of pulmonary vasomodulator therapies in adults with PH and PA-VSD.
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