Mitochondrial Dysfunction: Metabolic Drivers of Pulmonary Hypertension

Pulmonary hypertension (PH) is a progressive disease characterized by pulmonary vascular remodeling and lung vasculopathy. The disease displays progressive dyspnea, pulmonary artery uncoupling and right ventricular (RV) dysfunction. The overall survival rate is ranging from 28-72%. The molecular events that promote the development of PH are complex and incompletely understood. Metabolic impairment has been proposed to contribute to the pathophysiology of PH with evidence for mitochondrial dysfunction involving the electron transport chain proteins, antioxidant enzymes, apoptosis regulators, and mitochondrial quality control. It is vital to characterize the mechanisms by which mitochondrial dysfunction contribute to PH pathogenesis. This review focuses on the currently available publications that supports mitochondrial mechanisms in PH pathophysiology. Further studies of these metabolic mitochondrial alterations in PH could be viable targets of diagnostic and therapeutic intervention.