Pretreatment with PAH-targeted therapy enabled successful defect repair and improved functional status in two adults with sinus venosus atrial septal defects and severe PAH.
Does pretreatment with PAH-targeted therapy enable successful defect repair and improve functional status in adults with sinus venosus atrial septal defects and severe PAH?
Pretreatment with PAH-targeted therapy can successfully bridge adult patients with severe PAH and sinus venosus atrial septal defects to surgical or transcatheter repair.
Absolute Event Rate: 0% vs 0%
ABSTRACT 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.
Sakuma et al. (Thu,) reported a other. Pretreatment with PAH-targeted therapy enabled successful defect repair and improved functional status in two adults with sinus venosus atrial septal defects and severe PAH.