Transient use of oral bosentan can be an additional option to reduce pulmonary arterial hypertension in a patient with severe pulmonary arterial hypertension associated with atrial septal defect.

Atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) is thought to preclude shunt closure. However, there are several reports that vasodilator treatment is associated with good clinical outcome in these patients, recently. We report a case of good clinical outcome in a patient with ASD and severe PAH successfully treated with operative closure of ASD and subsequent use of oral bosentan medication. This case supports that the corrective repair of ASD and an oral bosentan treatment can be one of the treatment options in the selected patients with severe PAH associated with ASD.