Hadeel Allam, Eli Fredman, Daisuke Kobayashi, Manish Aggarwal, R. Mark Grady, David Balzer
Washington University School of Medicine and St Louis Children’s Hospital.
United States
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2026;
DOI: 10.1016/j.jaccas.2026.110220
Abstract
Background: Data on sinus venosus atrial septal defect (SVASD) closure in patients with coexistent pulmonary arterial hypertension (PAH) are scarce because current guidelines restrict closure to a pulmonary vascular resistance (PVR) of <5 WU.
Case summary: A 20-year-old woman with severe PAH and a PVR of 12.6 WU underwent successful transcatheter SVASD and partial anomalous pulmonary venous return correction. A small atrial communication was intentionally created as a temporary safety “pop-off,” enabling aggressive postprocedural escalation of PAH therapy. WHY BEYOND THE GUIDELINES?: Patients with severe PAH may still benefit from transcatheter SVASD closure despite guidelines. These patients require highly individualized, nonguideline-directed interventions.
Discussion: Current guidelines restrict ASD closure to PVR <5 WU, and prior data on closure at higher resistances are largely limited to secundum defects. This successful fenestrated SVASD closure at a baseline PVR of 12.6 WU challenges the utility of relying on a single hemodynamic threshold. Creating a temporary, controlled fenestration provides an essential safety margin, expanding interventional options for high-risk patients who would otherwise be precluded from care.
Take-home message: Transcatheter closure of SVASD in patients with severe PAH can effectively reduce the left-to-right shunt and facilitate escalation of PAH-targeted therapies.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: 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: Yes