Title:Pharmacological Agents and Potential New Therapies in Pulmonary
Arterial Hypertension
Volume: 22
Issue: 3
Author(s): Renata Trabach Santos, Maria Eduarda de Sá Freire Onofre, Dayene de Assis Fernandes Caldeira, Adriane Bello Klein, Patricia Rieken Macedo Rocco, Fernanda Ferreira Cruz and Pedro Leme Silva*
Affiliation:
- Laboratory of Pulmonary Investigation, Carlos Chagas Filho Institute of Biophysics, Federal University of Rio de
Janeiro, Rio de Janeiro, Brazil
Keywords:
Pulmonary hypertension, pulmonary arterial hypertension, metabolic therapy, metabolism, pharmacological agents, new therapies, regenerative medicine.
Abstract: Pulmonary arterial hypertension (PAH) is a progressive disease characterized by an imbalance
between vasoactive mediators, which causes vascular remodeling, increased pulmonary vascular
resistance, and right ventricular overload, ultimately leading to heart failure and death. A metabolic
theory has been suggested to explain the pathophysiology of PAH whereby abnormalities in
mitochondrial biogenesis can trigger a hyperproliferative and apoptosis-resistant phenotype in cardiopulmonary
and malignant cells, leading to mitochondrial dysfunction, which in turn causes the
Warburg effect. This can culminate in the mitophagy of pulmonary vessels and cardiomyocytes. The
present narrative review focuses on the pathophysiology of PAH, the pharmacological agents currently
available for its treatment, and promising and challenging areas of therapeutic investigation.