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Epoprostenol (Prostacyclin) and Pulmonary Hypertension
- University of Pennsylvania School of Medicine; Philadelphia, PA 19104 (Fishman)
Pulmonary hypertension is a prevalent disorder, and the list of its causes is long and varied. Included in the list, which is densely populated by so-called secondary types of pulmonary hypertension, is the category of unexplained (primary) pulmonary hypertension, presumably the final common pathway for multiple unidentified causes. For those concerned with the pathogenesis of the more common and diverse pulmonary hypertensive disorders that make up the category of secondary pulmonary hypertension, interest in primary pulmonary hypertension is high because it represents a model of “pure” intrinsic pulmonary vascular disease, uncomplicated by concomitant disease of the heart or lungs. Those concerned with therapy continue to direct their attention at relief of pulmonary vasoconstriction, which seems to be involved in the pathogenesis of primary pulmonary hypertension (1).
Before epoprostenol (prostacyclin) became available for clinical use, primary pulmonary hypertension was a calamitous disorder, destined to pursue a relentlessly downhill course that ended in right ventricular failure within 2 to 3 years. All sorts of systemic vasodilators were tried as pulmonary vasodilators, with largely inconsistent or futile results. The advent of prostacyclin introduced the most potent vasodilator known; other useful attributes of this agent are antiplatelet aggregation and adhesion inhibition of smooth-muscle proliferation. It marked the end of inevitability of early death and the beginning of a brighter outlook for quality of life in primary pulmonary hypertension.
The first applications of prostacyclin were diagnostic. They led to the recognition of prostacyclin as the “gold standard” for testing the ability of the hypertensive pulmonary circulation to undergo vasodilation (2). The pulmonary hypertension centers that were established by the National Heart, Lung, and Blood Institute as part of the National Registry on Primary Pulmonary Hypertension played an important role in the recruitment of sufficient numbers of patients with primary pulmonary hypertension. The collective …
