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F1000Res. 2018 Jul 24;7. pii: F1000 Faculty Rev-1128. doi: 10.12688/f1000research.14984.1. eCollection 2018.

Recent advances in pulmonary arterial hypertension.

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Department of Medicine, Imperial College London, London, UK.


Pulmonary arterial hypertension (PAH) is a rare disorder with a high mortality rate. Treatment options have improved in the last 20 years, but patients still die prematurely of right heart failure. Though rare, it is heterogeneous at the genetic and molecular level, and understanding and exploiting this is key to the development of more effective treatments. BMPR2, encoding bone morphogenetic receptor type 2, is the most commonly affected gene in both familial and non-familial PAH, but rare mutations have been identified in other genes. Transcriptomic, proteomic, and metabolomic studies looking for endophenotypes are under way. There is no shortage of candidate new drug targets for PAH, but the selection and prioritisation of these are challenges for the research community.


BMPR2; bone morphogenetic receptor type 2; new drug targets; pulmonary hypertension

Conflict of interest statement

Competing interests: Martin R. Wilkins, John Wharton, and Christopher Rhodes received advisory board funding from Actelion.Competing interests: David Badesch serves on multiple advisory boards and steering committees, and as a consultant, to a number of biopharmaceutical companies developing products in the field, including Actelion, Gilead, United Therapeutics / Lung LLC, Arena, Belleraphon, Respira, Acceleron, and Complexa.No competing interests were disclosed.

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