Pulmonary arterial hypertension (PAH) is a debilitating vascular disease of the pulmonary circulation that leads to elevation in the pulmonary artery pressure and pulmonary vascular resistance with resultant right ventricular failure and death. Despite expanding therapeutic options, PAH continues to have unacceptably high morbidity and mortality. Endothelial cell dysfunction, impaired eicosanoid balance, inflammation, oxidative stress, thrombosis, vascular proliferation, and metabolic dysregulation have all been implicated in the pathogenesis of PAH. Early detection, risk assessment, and follow-up for disease progression are essential components of the clinical management of PAH. Currently, treatment decisions are based on assessment of disease severity determined by symptoms and exercise capacity. Unfortunately there is a significant amount of subjectivity and imprecision in these assessments. As a result, there has been increasing interest and research for the identification of useful biomarkers in PAH. The US Food and Drug Administration (FDA) defines a biomarker broadly as a “characteristic that is objectively measured and evaluated as an indicator of normal biological processes, pathogenic processes, or pharmacologic responses to a therapeutic.
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