Palliation of systemic sclerosis-associated pulmonary hypertension by atrial septostomy

The onset of pulmonary hypertension in patients with systemic sclerosis carries a poor prognosis. Atrial septostomy has been used successfully to palliate endstage primary pulmonary hypertension but has not been attempted in other forms of pulmonary vascular disease. We report substantial clinical improvement following atrial septostomy in a patient with systemic sclerosis complicated by severe, isolated pulmonary hypertension. After the procedure, exercise capacity was improved and exertional syncope abolished. We suggest that this procedure should be considered for other patients with this diagnosis.