Pathologic assessment of vasculopathies in pulmonary hypertension

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Abstract

Pulmonary arterial hypertension (PAH) includes various forms of pulmonary hypertension of different etiology but similar clinical presentation and functional derangement. Histopathological vascular changes in all forms of PAH are qualitatively similar but with quantitative differences in the distribution and prevalence of pathological changes in various portions of the pulmonary vascular bed. The documentation of these topographic variations in the response of the pulmonary vasculature to injury may be important to understand the pathogenesis of the various subsets of PAH. To standardize the precise histopathological documentation of the pulmonary vasculopathy in PAH we propose a histopathological classification that includes both the predominant segment of the pulmonary vasculature affected and the possible coexistence of pathological changes in other vascular segments.

Abbreviations

BMPR2
Bone morphometric protein receptor-2
HIV
human immunodeficiency virus
PAH
pulmonary arterial hypertension
PCH
pulmonary capillary hemangiomatosis
PM
pulmonary microvasculopathy
POV
pulmonary occlusive venopathy
PPH
primary pulmonary hypertension
PVOD
pulmonary veno-occlusive disease

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