Continuous epoprostenol therapy and septal defect closure in a patient with severe pulmonary hypertension.

A 31-year-old woman with exertional dyspnea diagnosed as having atrial septal defect (ASD) with severe pulmonary hypertension (PH). Intravenous epoprostenol therapy was started to improve PH. Although pulmonary arterial pressure decreased, her symptoms remained in class III of WHO functional class, probably because of exacerbation of the left-to-right shunt caused by the reduction of pulmonary vascular resistance (PVR). Transcatheter atrial septal closure was therefore performed. Soon after the procedure, additional reduction in pulmonary arterial pressure was achieved. Her symptoms improved and oxygen inhalation was discontinued. One year after the procedure, although intravenous epoprostenol was still required, her symptoms had improved to class I of WHO functional class without exacerbation of PH. Transcatheter atrial septal closure after lowering PVR by intravenous epoprostenol would be a novel therapy for patients with ASD accompanied by PH.