How to manage primary pulmonary hypertension: Giving hope to patients with a life-threatening illness

Primary pulmonary hypertension presents a challenge to practicing physicians, in both diagnosis and management. Exposure to anorexigens and complaints of dyspnea and fatigue should prompt careful physical examination and Doppler echocardiography to assess patients for pulmonary hypertension. The burden on office practitioners is heavy, considering how often fatigue and dyspnea are reported, but the key is recognizing when these findings are out of proportion to the patient's well appearance. The discovery of epoprostenol therapy has revolutionized the approach to primary pulmonary hypertension. It has markedly improved quality of life and extended survival in patients with the condition, and it has changed the physician's role from providing emotional support to dying patients to providing management of a chronic disease.