Assessment of right ventricular metabolism: An emerging tool for monitoring pulmonary artery hypertension

Excerpt

Pulmonary arterial hypertension (PAH) is a rare, progressive disease that is ultimately fatal without treatment. In the last two decades, the advances of PAH-specific therapies have made a significant impact on the lives of patients struggling with PAH. The new approved therapies aim to interfere with unopposed vasoconstriction and pulmonary vascular remodeling targeting one of the three major pathophysiologic pathways: the nitric oxide pathway, the endothelin-1 pathway, and the prostacyclin pathway. Nevertheless, even in the era of new therapies, the prognosis remains grim with a 3-year cumulative survival of only 55%, which is devastating especially when considering the relatively young age of the affected patient population.1 …