Transcatheter Sinus Venosus ASD Closure in Severe Pulmonary Hypertension
- 1Division of Pediatric Cardiology, Washington University School of Medicine, St Louis Children's Hospital, St. Louis, Missouri, USA
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.
Related
- Complete transcatheter closure of residual aortopulmonary window in an elderly patient allowing optimization of refractory pulmonary arterial hypertension therapy.
- A Large Retrospective Study to Evaluate Utility of Zephyr Covered Stent in Transcatheter Closure of Sinus Venosus Defects
- Residual Pulmonary Hypertension After Transcatheter Edge-to-Edge Repair in Patients With Secondary Mitral Regurgitation
- Atrial Flow Regulator Bridging to Heart Transplantation in Congenital Heart Disease With Severe Combined Pulmonary Hypertension.
- Pulmonary arterial hypertension before closure of a persistent arterial duct (PDA) in children <12 months: clinical implications and outcome
BibTeX
@article{allam2026transcatheter,
title = {{Transcatheter Sinus Venosus ASD Closure in Severe Pulmonary Hypertension}},
author = {Hadeel Allam and Eli Fredman and Daisuke Kobayashi and Manish Aggarwal and Ronald Grady and David Balzer},
journal = {JACC Case Reports},
year = {2026},
month = sep,
doi = {10.1016/j.jaccas.2026.110220},
url = {https://doi.org/10.1016/j.jaccas.2026.110220},
note = {CC BY-NC-ND 4.0},
}
RIS
TY - JOUR
TI - Transcatheter Sinus Venosus ASD Closure in Severe Pulmonary Hypertension
AU - Hadeel Allam
AU - Eli Fredman
AU - Daisuke Kobayashi
AU - Manish Aggarwal
AU - Ronald Grady
AU - David Balzer
PY - 2026
DA - 2026/09/01
JO - JACC Case Reports
DO - 10.1016/j.jaccas.2026.110220
UR - https://doi.org/10.1016/j.jaccas.2026.110220
AB - 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.
SN - 2666-0849
ER -