Atrial septal defect repair after a 10-month treatment with bosentan in a patient with severe pulmonary arterial hypertension: A case report

Abstract

Congenital type II atrial septal defect (ASD) is associated with precapillary pulmonary hypertension (PAH) in roughly 10% of cases.1 Principally, closure of the shunt lesion is recommended, and large ASDs must be repaired in early childhood to prevent Eisenmenger's syndrome. Once severe pulmonary hypertension or Eisenmenger's syndrome has developed, ASD closure is problematic due the increased risk of right ventricular failure and pulmonary hypertensive crisis.2 At this stage, heart–lung transplantation is the only surgical option.