PMC9834556_01
CMRI and coronary angiography of the systemic sclerosis heart. (A-D) Late gadolinium-enhancement CMRI of the explanted heart. An extensive subendocardial enhancement of the left ventricle and multifocal enhancement in the right ventricle was noted (white arrows). (E, F) Coronary angiography of the heart. (E) Left coronary angiography and (F) right coronary angiography revealed no significant epicardial coronary artery stenosis.. CMRI = cardiac magnetic resonance imaging.
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F1
Cardiac magnetic resonance imaging (CMRI) showed extensive late gadolinium enhancement (LGE) subendocardium of left ventricular walls, which suggested multi-vessel coronary artery occlusion (Figure 1A-D). However, there was no significant epicardial coronary artery obstruction on coronary angiography (Figure 1E and F).
Pathologic findings. (A) Gross appearance of the explanted heart. Cross-sections of the entire heart specimen showed ventricular dilation and prominent endocardial fibrosis in both ventricles (arrow, whitish tissues). (B, C) Low magnification of the inferior wall of the left ventricle showed wall thinning, endocardial thickening with fibrosis, and patchy myocardial fibrosis. (B) Hematoxylin and eosin stain; (C) Masson's trichrome stain.. Endo = endocardium; Epi = epicardium; Myo = myocardium.
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F2
Pathologic examination of the explanted heart revealed severe endocardial fibrosis and multifocal patch fibrosis of the myocardium in both ventricles (Figure 2).
Microvascular alterations in pathologic specimens of the explanted systemic sclerosis heart. (A-D) Microscopic examination reveals fibromuscular dysplastic changes in the small vessels of the subendocardial myocardium. These micro-vasculatures show luminal narrowing due to fibrosis and smooth muscle hyperplasia in the tunica intima and media. Fragmentation or duplication of the internal elastic lamina is also noted. (A) Hematoxylin and eosin stain, x100; (B) Masson's trichrome stain, x100; (C) Verhoeff's elastic stain, x100; (D) Immunohistochemical stain for smooth muscle actin, x100. (E, F) An atrioventricular nodal artery shows luminal narrowing and vascular wall fibrosis. (E) Hematoxylin and eosin stain, x50; (F) Masson's trichrome stain, x50. (E, F) White arrows indicate a nodal artery and (E) blue ink indicates the right side of the interventricular septum (arrowhead).
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F3
Most intracardiac arteries including atrioventricular nodal artery and arterioles showed fibromuscular dysplastic changes with luminal narrowing (Figure 3) consistent with ischemic pattern LGE in CMRI.