Prostacyclin and Primary Pulmonary Hypertension

Fortunately, primary pulmonary hypertension is uncommon. Most physicians only know of it from reading in medical school that it is a fatal disease that typically affects young women in the prime of their life and for which no therapy exists. Those who have had to care for affected patients can testify to how it erodes the quality of a patient's life by making the most menial task limited by dyspnea, syncope, and fatigue and how it progresses unabated until the patient succumbs. In the past, experts would feel a profound sense of ambivalence when a patient with the disorder was referred. Although they could provide a correct diagnosis and expert care, they knew that they could do little to alter the clinical course.

In 1982, heart and lung transplantation was reported as a definitive therapy [1]. Although it did provide a glimmer of hope, the few centers experienced in heart and lung transplantation, the long waiting time to receive donor organs, and the high postoperative morbidity and mortality rates painted …