World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology

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95 Scopus citations


Pulmonary arterial hypertension (PAH) is a debilitating disease characterized by pathologic remodeling of the resistance pulmonary arteries, ultimately leading to right ventricular (RV) failure and death. In this article we discuss the definition of PAH, the initial epidemiology based on the National Institutes of Health Registry, and the updated epidemiology gleaned from contemporary registries, pathogenesis of pulmonary vascular dysfunction and proliferation, and RV failure in PAH.

Original languageEnglish (US)
Pages (from-to)363-374
Number of pages12
JournalCardiology Clinics
Issue number3
StatePublished - Aug 1 2016

Bibliographical note

Funding Information:
Funding: T. Thenappan was funded by AHA Scientist Development Grant 15SDG25560048 and K.W. Prins was funded by National Institutes of Health F32 grant HL129554 .

Publisher Copyright:
© 2016 Elsevier Inc.


  • Epidemiology
  • Pathophysiology
  • Pulmonary arterial hypertension
  • Pulmonary vasculature
  • Right ventricle


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