Combination therapy with prostacyclin and tadalafil for severe pulmonary arterial hypertension: A pilot study

4 patients with severe pulmonary arterial hypertension (PAH) that deteriorated despite prostacyclin therapy (2 with Eisenmenger syndrome, 1 with scleroderma-associated PAH, 1 with histiocytosis X).

Tadalafil 10-20 mg once daily added to prostacyclin therapy for 3 months.

Clinical improvement including 6-minute walk distance (6MWD), New York Heart Association (NYHA) functional class, and echocardiographic pulmonary arterial pressure at 3 months.surrogate