JACC Case Rep. 2026 Sep 1:110220. doi: 10.1016/j.jaccas.2026.110220. Online ahead of print.
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.
PMID:42709004 | DOI:10.1016/j.jaccas.2026.110220
