Pulmonary arterial hypertension: Navigating the pathways of progress in diagnosis, treatment, and patient care

Abstract

Pulmonary arterial hypertension (PAH) is a form of precapillary pulmonary hypertension caused by a complex process of endothelial dysfunction and vascular remodeling. If left untreated, this progressive disease presents with symptoms of incapacitating fatigue causing marked loss of quality of life, eventually culminating in right ventricular failure and death. Patient management is complex and based on accurate diagnosis, risk stratification, and treatment initiation, with close monitoring of response and disease progression. Understanding the underlying pathophysiology has enabled the development of multiple drugs directed at different targets in the pathological chain. Vasodilator therapy has been the mainstay approach for the last few years, significantly improving quality of life, functional status, and survival. Recent advances in therapies targeting dysfunctional pathways beyond endothelial dysfunction may address the fundamental processes underlying the disease, raising the prospect of increasingly effective options for this high-risk group of patients with a historically poor prognosis.

Keywords: Agonistas dos recetores IP das prostaciclinas; Antagonistas dos recetores da endotelina; Análogos das prostaciclinas; Diagnosis; Diagnóstico; Endothelin receptor antagonists; Estimuladores da guanilato ciclase solúvel; Estratificação de risco; Hipertensão arterial pulmonar; Hipertensão pulmonar; Inibidores da fosfodiesterase tipo 5; Phosphodiesterase type 5 inhibitors; Prostacyclin IP receptor agonists; Prostacyclin analogs; Pulmonary arterial hypertension; Pulmonary hypertension; Risk stratification; Soluble guanylate cyclase stimulators.