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

World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology

Kurt W Prins  1 , Thenappan Thenappan  2
Affiliations
Review

World Health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology

Kurt W Prins et al. Cardiol Clin. 2016 Aug.

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.

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Figures

Figure 1
Figure 1
Examples of pathological changes observed in lung samples from patients with PAH Adapted from Rich S, Pogoriler J, Husain AN, et al. Long-term effects of epoprostenol on the pulmonary vasculature in idiopathic pulmonary arterial hypertension. Chest 2010;138(5):1234-9; with permission.
Figure 2
Figure 2
Examples of adaptive and maladaptive RV remodeling in PAH Adapted from Rich S, Pogoriler J, Husain AN, et al. Long-term effects of epoprostenol on the pulmonary vasculature in idiopathic pulmonary arterial hypertension. Chest 2010;138(5):1234-9; with permission.
Figure 3
Figure 3
Schematic representation of imbalance of vasodilatory and vasocontricting substances present in the pulmonary circulation in PAH leading to pulmonary vascular dysfunction in PAH.
Figure 4
Figure 4
Multiple pathways converge to induce pulmonary vascular proliferation in PAH.
Figure 5
Figure 5
Mechanisms that contribute to RV failure in PAH.
See this image and copyright information in PMC

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