Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience

Abstract

Epoprostenol was the first therapy to be approved for the treatment of pulmonary arterial hypertension (PAH). In the 20 years since the introduction of this prostacyclin analogue, the outlook for patients with PAH has improved, with survival rates now double those from the era before the development of disease-specific treatments. Today, there are a large amount of data on the clinical role of prostacyclin treatments and a body of evidence attesting the efficacy of epoprostenol in improving exercise capacity, key haemodynamic parameters and PAH symptoms, as well as in reducing mortality. The place of epoprostenol in the therapeutic management of PAH continues to evolve, with the development of new formulations and use in combination with other drug classes. In this review, we provide a historical perspective on the first 20 years of epoprostenol, a therapy that led to evidence-based study of PAH-specific treatments and the subsequent expansion of treatment options for PAH.

FIGURE 1

The effects of prostanoids on vasculature and blood cells; a variety of vascular cells, platelets and leukocytes have been identified as targets for the antiproliferative, anti-inflammatory and anti-aggregatory actions of prostaglandins. SMC: smooth muscle cells; EC: endothelial cells; Mono: mononuclear cells; NF: nuclear factor; TNF: transforming nuclear factor; IL: interleukin; MA: macrophages; MAPK: mitogen-activated protein kinase; iNOS: inducible nitric oxide synthase; PMN: polymorphonuclear neutrophils; Burst: generation of reactive oxygen species. Reproduced from [38] with permission from the publisher.