PT - JOURNAL ARTICLE AU - Stephen Y. Chan AU - Lewis J. Rubin TI - Metabolic dysfunction in pulmonary hypertension: from basic science to clinical practice AID - 10.1183/16000617.0094-2017 DP - 2017 Dec 31 TA - European Respiratory Review PG - 170094 VI - 26 IP - 146 4099 - http://err.ersjournals.com/content/26/146/170094.short 4100 - http://err.ersjournals.com/content/26/146/170094.full SO - EUROPEAN RESPIRATORY REVIEW2017 Dec 31; 26 AB - Pulmonary hypertension (PH) is an often-fatal vascular disease of unclear molecular origins. The pulmonary vascular remodelling which occurs in PH is characterised by elevated vasomotor tone and a pro-proliferative state, ultimately leading to right ventricular dysfunction and heart failure. Guided in many respects by prior evidence from cancer biology, recent investigations have identified metabolic aberrations as crucial components of the disease process in both the pulmonary vessels and the right ventricle. Given the need for improved diagnostic and therapeutic options for PH, the development or repurposing of metabolic tracers and medications could provide an effective avenue for preventing or even reversing disease progression. In this review, we describe the metabolic mechanisms that are known to be dysregulated in PH; we explore the advancing diagnostic testing and imaging modalities that are being developed to improve diagnostic capability for this disease; and we discuss emerging drugs for PH which target these metabolic pathways.Understanding metabolic pathways in PH provides opportunities for improved diagnostic and therapeutic options http://ow.ly/pFQb30guez6