World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology

Kurt W Prins; Thenappan Thenappan

doi:10.1016/j.ccl.2016.04.001

World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology

Cardiol Clin. 2016 Aug;34(3):363-74. doi: 10.1016/j.ccl.2016.04.001.

Authors

Kurt W Prins¹, Thenappan Thenappan²

Affiliations

¹ Cardiovascular Division, University of Minnesota Medical School, 420 Delaware Street Southeast, Minneapolis, MN 55455, USA.
² Section of Advanced Heart Failure and Pulmonary Hypertension, Cardiovascular Division, University of Minnesota Medical School, 420 Delaware Street Southeast, Minneapolis, MN 55455, USA. Electronic address: tthenapp@umn.edu.

Abstract

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.

Keywords: Epidemiology; Pathophysiology; Pulmonary arterial hypertension; Pulmonary vasculature; Right ventricle.

Publication types

Review

MeSH terms

Global Health
Humans
Hypertension, Pulmonary* / classification
Hypertension, Pulmonary* / epidemiology
Hypertension, Pulmonary* / physiopathology
Morbidity / trends
Pulmonary Wedge Pressure / physiology*
Ventricular Function, Right / physiology*
World Health Organization

Grants and funding

F32 HL129554/HL/NHLBI NIH HHS/United States