Sildenafil in the management of primary pulmonary hypertension

Primary pulmonary hypertension is a rare disorder of unknown etiology with a poor prognosis. There is no cure, and drug therapy is effective in only a few patients. Calcium-channel antagonists and anticoagulants are the mainstay of therapy. Prostacyclin therapy leads to significant clinical improvement but its use is restricted due to high cost and complex drug delivery systems. Sildenafil is a selective vasodilator and has been shown to be effective in decreasing pulmonary vascular resistance in animal models of pulmonary hypertension. We report the use of sildenafil in two patients of primary pulmonary hypertension who were refractory to conventional drug therapy.