Key result
Prolonged PAH-targeted therapy enables successful defect closure in severe PAH with superior SVASD.
Why the study?
Pretreatment with targeted PAH therapy to improve hemodynamics and expand closure candidacy in unrepaired ASDs has been reported predominantly in secundum ASDs, with limited data on sinus venosus ASDs.
Does prolonged pre-treatment with PAH-targeted therapy improve pulmonary hemodynamics and expand candidacy for defect closure in adult patients with sinus venosus atrial septal defects and severe PAH?
Case Report (n=2)
Does prolonged pre-treatment with PAH-targeted therapy improve pulmonary hemodynamics and expand candidacy for defect closure in adult patients with sinus venosus atrial septal defects and severe PAH?
Prolonged pre-treatment with PAH-targeted therapy can successfully bridge selected adult patients with sinus venosus atrial septal defects and severe PAH to successful transcatheter or surgical repair.
Pretreatment with targeted pulmonary arterial hypertension (PAH) therapy in adults with unrepaired atrial septal defects (ASDs) has been increasingly reported as a means to improve pulmonary hemodynamics and expand candidacy for defect closure. However, most published reports describe heterogeneous ASD cohorts, predominantly composed of secundum ASDs, with limited data on sinus venosus atrial septal defects (SVASDs). We describe two cases of adult superior SVASD with severe PAH, initially deemed unsuitable for closure, who underwent prolonged pretreatment with PAH‐targeted therapy and subsequently achieved successful repair, one via transcatheter intervention. Both patients demonstrated improved functional status after repair and were able to reduce PAH therapy. These cases underscore the potential of pre‐treatment with PAH‐targeted therapy to bridge to repair in selected SVASD patients and highlight the need for further study in this unique subgroup.
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Sakuma et al. (2026) conducted a case report in Sinus venosus atrial septal defect (SVASD) with severe pulmonary arterial hypertension (PAH) (n=2). Pulmonary arterial hypertension (PAH)-targeted therapy (treat-and-repair strategy) was evaluated on Successful defect repair and reduction in pulmonary vascular resistance. Prolonged pretreatment with PAH-targeted therapy for over 2 years improved pulmonary hemodynamics sufficiently to allow successful defect closure in two adult patients with superior SVASD and severe PAH.
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