Metabolic dysfunction in pulmonary hypertension: from basic science to clinical practice

Eur Respir Rev. 2017 Dec 20;26(146):170094. doi: 10.1183/16000617.0094-2017. Print 2017 Dec 31.


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.

Publication types

  • Review

MeSH terms

  • Animals
  • Antihypertensive Agents / therapeutic use
  • Arterial Pressure* / drug effects
  • Energy Metabolism* / drug effects
  • Heart Failure / metabolism
  • Heart Failure / physiopathology
  • Humans
  • Hypertension, Pulmonary / diagnostic imaging
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / metabolism*
  • Hypertension, Pulmonary / physiopathology
  • Mitochondria / metabolism
  • Mitochondria / pathology
  • Pulmonary Artery / drug effects
  • Pulmonary Artery / metabolism*
  • Pulmonary Artery / pathology
  • Pulmonary Artery / physiopathology
  • Vascular Remodeling
  • Ventricular Dysfunction, Right / metabolism
  • Ventricular Dysfunction, Right / physiopathology


  • Antihypertensive Agents