Assuntos
Hipertensão Arterial Pulmonar , Transposição dos Grandes Vasos , Artérias , Criança , Hipertensão Pulmonar Primária Familiar , Humanos , Prevalência , Artéria Pulmonar , Sistema de Registros , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/epidemiologia , Transposição dos Grandes Vasos/genéticaRESUMO
Introduction: Pulmonary arterial hypertension (PAH) is a rare, progressive, and severe disease. Since the first demonstration of survival benefit of intravenous epoprostenol in monotherapy in 1996, prostanoids remain the cornerstone for PAH patients at high risk. This review is essential to understand the current situation of this drug among all the therapeutic possibilities concerning this entity.Areas covered: The aim of this article is to review the pharmacological properties of epoprostenol and to update its clinical evidence in different specific clinical scenarios. A deep literature search was carried out on the MEDLINE database for published literature before March 2021.Expert opinion: Epoprostenol is still the best treatment option for high-risk PAH patients. Nevertheless, there are many unsolved questions: drug dosing, its use in combination therapy with other pulmonary vasodilators, and the utility in PAH associated with connective tissue disease, congenital heart disease, or pulmonary veno-occlusive disease are only a few examples. Its safety and efficacy are supported by clinical trials, observational work, and experience, both as monotherapy and in combination therapy. Epoprostenol should be considered a destination treatment in high-risk patients and not only as a bridge to pulmonary transplantation. However, it remains underused in clinical practice.
