Ryo Sakuma, Gareth Morgan, Elizabeth Yeung, Benjamin S. Frank, Charles Simpkin, Dunbar Ivy
University of Colorado School of Medicine.
United States
Pulmonary Circulation
Pulm Circ 2026; 16:
DOI: 10.1002/pul2.70285
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes