Abordaje de la hipertensión arterial pulmonar para el clínico: fisiopatología, diagnóstico y tratamiento

Pulmonary arterial hypertension (PAH) is a rare and serious pathology in which abnormal pulmonary vascular remodeling occurs causing increased pulmonary vascular resistance, pulmonary pressures and right ventricular afterload, leading to right heart failure due to right ventricular failure, which is the main cause of death. Advances in the understanding of the pathophysiological mechanisms of the disease have allowed the development of new molecular compounds aimed at partially reversing the alterations in the pulmonary vasculature, providing additional mechanisms of action and therapeutic targets to the classic pulmonary vasodilator drugs. This work covers a review of the pathophysiological mechanisms, the diagnostic process of PAH, considering how to integrate the different clinical elements and diagnostic tests for an early and correct diagnosis, and presents the available and developing molecular compounds with an analysis of the potential role of the latter in current therapeutic algorithms.