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. Author manuscript; available in PMC: 2022 Jun 24.
Published in final edited form as: Free Radic Biol Med. 2021 Aug 31;175:95–107. doi: 10.1016/j.freeradbiomed.2021.08.238

Figure 1: Pulmonary hypertension in SCD results in lung vascular and right ventricle tissue remodeling.

Figure 1:

(A) Human main pulmonary artery (MPA) tissue sections from SCD patients with PH (SCD PH (+)) demonstrate distinctive pathology when compared to SCD patients without PH (SCD PH (−)) and controls. H&E staining (a-c), Masson’s trichrome (d-f) and Verhoeff Van Gieson elastic stain, EVG (g-i ). SCD PH + patient H&E stained tissues sections show intimal hyperplasia (black arrows), medial thickening and loss of vascular smooth muscle cell integrity (c, inset). Collagen deposition (f) and elastin fragmentation (i) are observed in the MPA media. (B) Human peripheral lung tissue sections are shown from top to bottom stained for H&E (a-c), Perls iron (non-heme iron) (d-f), 4-hydroxynonenal (4-HNE) (g-i) and Masson’s trichrome (j-l). Staining in SCD PH + patient peripheral lung tissue shows arterial recanalization, vascular plexiform lesions, and intimal thickening (c). Iron positive cellular staining is observed in lung vascular adventitia (f) and immune reactivity to 4-HNE within outer medial and adventitia accumulated cells consistent with iron localization (i). Peripheral arterial lung fibrosis is a prominent pathologic feature of SCD PH and visually observed across all structures of the vessel (l). Limited indicators of pathological processes are visualized in SCD PH - patient tissue sections, except for oxidative stress visually observed as 4-HNE immune reactivity in adventitia accumulated cells (h). No indications of peripheral lung vascular pathology are observed in control tissue sections. (C) Right ventricular pathology is observed in SCD PH (+) as loss of cardiac muscle striations (black arrows) and eosinophilia (back box) (c). Cardiac iron was not observed in any of the tissue sections (e-f). 4-HNE immune reactivity was most intense visually in SCD PH + patient tissue sections (i) and to a lesser extent in SCD PH – tissue sections (h). Interstitial collagen accumulation is visualized in SCD PH + patient tissue sections (l), to a lesser extent in SCD PH – tissue sections (k) and not at all in control in tissue sections. Magnification main PA - 10x; distal PA- 10x; right ventricle- 20x