Volume 71 Number 2 March 2004	back to contents

¹Instructor and ²Assistant Professor of Medicine, Mount Sinai Pulmonary Hypertension Program, Mount Sinai School of Medicine, New York, NY.

Address all correspondence to Michael Poon, M.D., Director, Mount Sinai Pulmonary Hypertension Program, Cardiology Division, Box 1033, Mount Sinai School of Medicine, 1 East 100th Street, New York, NY 10029; E-mail: michael.poon@msnyuhealth.org

Adapted from a Grand Rounds presentation to The Zena and Michael A. Wiener Cardiovascular Institute, Department of Medicine, Mount Sinai School of Medicine, New York, NY, on December 3, 2001, and updated as of July 2003.

ABSTRACT

Primary pulmonary hypertension is a rare disease of the pulmonary vasculature manifested by dyspnea on exertion, syncope, and signs and symptoms of right heart failure. In the absence of adequate treatment, primary pulmonary hypertension has a grave prognosis, with a median survival of 2.8 years. Pulmonary arterial hypertension develops in association with known risk factors and predisposing clinical conditions, and shares many clinical, pathological and therapeutic characteristics with primary pulmonary hypertension. Therapeutic choices in pulmonary arterial hypertension depend on the etiology of the disease, severity of functional impairment and hemodynamic response following acute vasodilator administration during right heart catheterization. Agents currently approved for the specific treatment of pulmonary arterial hypertension are continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan. A small group of patients who demonstrate true acute vasoreactivity at right heart catheterization may be chronically treated with oral calcium channel blockers. In addition, most patients with pulmonary hypertension receive conventional treatment, represented by anticoagulants, diuretics, inotropic medication or oxygen supplementation. Treatment of pulmonary arterial hypertension has significantly altered the natural course of the disease, with pronounced symptomatic, functional and survival benefit. Current clinical research focuses on the discovery of new targets of therapy and the use of a combination treatment approach, which will offer hope and valuable insight into the pathogenetic basis of this devastating illness.

KEYWORDS

Primary pulmonary hypertension, pulmonary arterial hypertension, treatment, prostacyclin, endothelin receptor antagonist, epoprostenol, treprostinil, bosentan.

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