Pathologic assessment of vasculopathies in pulmonary hypertension.
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.
Pietra
Giuseppe G
GG
Department of Pathology, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania, USA. gkpietra@freesurf.ch
Capron
Frederique
F
Stewart
Susan
S
Leone
Ornella
O
Humbert
Marc
M
Robbins
Ivan M
IM
Reid
Lynne M
LM
Tuder
R M
RM
D006801:Humans; D006976:Hypertension, Pulmonary / Q000473:pathology* / Q000503:physiopathology; D008168:Lung / Q000098:blood supply*; D009131:Muscle, Smooth, Vascular / Q000473:pathology / Q000503:physiopathology; D011651:Pulmonary Artery / Q000473:pathology / Q000503:physiopathology; D011652:Pulmonary Circulation / Q000502:physiology; D017539:Tunica Intima / Q000473:pathology / Q000503:physiopathology; D014652:Vascular Diseases / Q000473:pathology*; D014655:Vascular Resistance / Q000502:physiology; D014661:Vasoconstriction / Q000502:physiology