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Atrial Septal Defect and Pulmonary Arterial Hypertension
Quick Takes
- Patients with severe PAH and secundum ASD should be evaluated in a multidisciplinary fashion.
- Secundum ASD may be treated with a fenestrated device even in the presence of severe PAH in select patients.
Study Questions:
What are the outcomes with combining pulmonary arterial hypertension (PAH) medical therapy with atrial septal defect (ASD) closure using a fenestrated atrial septal occluder (F-ASO) device in patients with severe PAH and a secundum ASD?
Methods:
Fifty-six consecutive patients with severe PAH and secundum ASD were included (median age, 50.5 years; ASD size: 26.9 ± 4.6 mm). PAH medical therapy was given for 3 months before ASD closure was performed using an F-ASO device in patients with (Qp/Qs) ≥1.5 on medical therapy. Medical therapy was continued post-device closure and dual antiplatelet therapy was added for 6 months post-procedure.
Results:
After 3 months of PAH medical therapy, systolic pulmonary arterial pressure (sPAP) and pulmonary vascular resistance (PVR) decreased (sPAP: -14.5 mm Hg and PVR: -3.9 WU), and exercise capacity (+72.0 m) improved. Qp/Qs increased by 0.9, but with increased RV dilatation by 3.5 mm (all p < 0.001). Subsequent ASD closure was associated with a further drop in sPAP (-6.0 mm Hg). Exercise capacity (+60.5 m) and RV dilatation (-9.9 mm) improved as well, with over a median follow-up of 10 months’ post-device closure. One year later, PAP was normalized in 8 of 19 patients, and PAH recurred in five patients after discontinuation of targeted medical therapy.
Conclusions:
In patients with secundum ASD and severe PAH, PAH-specific medical therapy followed by percutaneous device closure using an F-ASO device is safe and effective. This combined treatment approach improves exercise capacity, with favorable RV remodeling.
Perspective:
Pretreatment with medical therapy followed by partial ASD closure using a fenestrated device in patients who have a Qp/Qs ≥1.5 after medical treatment seems a promising approach. Historically severe PAH has been an absolute contraindication for secundum ASD closure, but this report offers hope in such patients moving forward; these findings need to be validated in larger cohorts, and future work should focus on further refining the patient selection process in such cases. One can easily foresee a need for multidisciplinary teams and close collaboration between PAH experts and structural heart interventional teams.
Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Prevention, Pulmonary Hypertension, Hypertension
Keywords: Atrial Fibrillation, Blood Pressure, Cardiology Interventions, Dilatation, Exercise Tolerance, Heart Defects, Congenital, Heart Septal Defects, Atrial, Hypertension, Pulmonary, Platelet Aggregation Inhibitors, Septal Occluder Device, Systole, Vascular Resistance
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