Wenjie Chen, Jingyuan Chen, Min Peng, Jun Luo, Haihua Qiu, Yusi Chen, Jiang Li
Second Xiangya Hospital of Central South University.
China
International Journal of Cardiology
Int J Cardiol 2025;
DOI: 10.1016/j.ijcard.2025.133094
Abstract
Objective: This study aims to refine closure indications and assess long-term outcomes in pulmonary arterial hypertension associated with atrial septal defect (PAH-ASD).
Methods: We enrolled 197 PAH-ASD patients classified as unrepairable (n = 101) or repairable (n = 96), with the latter subdivided into direct repair (DR, n = 74) and treat-and-repair (TR, n = 22) groups. Closure criteria included pulmonary vascular resistance (PVR) 3-6 Wood units and Qp/Qs ≥1.5. The TR group received targeted therapy before closure.
Results: Targeted therapy significantly improved TR group hemodynamics: PVR decreased from 8.0 ± 2.0 to 3.0 ± 1.0 WU (p < 0.001); Qp/Qs increased from 1.7 ± 0.4 to 2.3 ± 0.3 (p < 0.001). Post-closure, pulmonary artery systolic pressure significantly decreased in both TR (67 ± 15 to 38 ± 9 mmHg) and DR groups (66 ± 17 to 40 ± 10 mmHg) (both p < 0.001). Right ventricular remodeling improved significantly. During median follow-up (3.7-4.5 years), mortality was lower in repairable versus unrepairable groups (1 vs. 10 deaths). Univariate COX analysis identified ASD closure as a protective factor (HR 0.093, 95 %CI [0.01,0.98].
Conclusion: ASD closure improves long-term survival in select PAH-ASD patients. Targeted therapy enables closure in previously ineligible patients.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No