Repair of Sinus Venosus Atrial Septal Defect in 2 Patients With Severe Pulmonary Arterial Hypertension

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.