Other Pediatric Cardiac Conditions

Miller, Dylan V.

doi:10.1007/978-1-61779-403-2_35

Cited by 1 publication

(1 citation statement)

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“…None of the macrophages demonstrated lipid vacuole-laden cytoplasm and the hyperplastic regions showed no cholesterol-clefting. These findings are in dual contrast to normal arterial media, which has continuous sheets of elastin fibers and no macrophage infiltration, as well as to atherosclerotic plaques, which demonstrate "foamy" lipid-laden macrophages and cholesterol clefting (Miller 2014). Similar findings were seen focally in the aortic intimal plaques (Fig.…”

Section: Histopathologymentioning

confidence: 72%

Cardiovascular Histopathology of a 11-Year Old with Mucopolysaccharidosis VII Demonstrates Fibrosis, Macrophage Infiltration, and Arterial Luminal Stenosis

Lew

Peña²,

Edwards

et al. 2017

JIMD Reports

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Mucopolysaccharidosis type VII (MPS VII) is caused by β-glucuronidase deficiency, resulting in lysosomal accumulation of glycosaminoglycans (GAGs) and multisystemic disease. We present cardiovascular gross and histopathology findings from a 11-year-old MPS VII male, who expired after developing ventricular fibrillation following anesthesia induction. Gross anatomic observations were made at autopsy; postmortem formalin-fixed paraffin-embedded samples of the carotid artery, aorta, myocardium, and valves were sectioned and stained with hematoxylin-eosin, Verhoeff-Van Gieson, CD68, and trichrome stains. Gross heart findings include an enlarged, dilated heart, mitral valve prolapse with thick, shortened chordae tendinae, and thickened aortic valve cusps. The aorta contained raised intimal plaques mimicking conventional atherosclerosis. Cardiac myocytes included hypertrophic nuclei, subendocardial fibrosis, and increased interfascicular collagen. Coronary lumens were 40-70% stenosed by fibrointimal hyperplasia containing storage material-laden cells, CD68 macrophages, and fragmented elastin laminae. Similar findings were visualized in aortic intimal plaques. We confirm that arterial plaques, elastin fragmentation, and activated CD68 macrophage infiltration occur in human MPS VII, consistent with previously observed findings in murine and canine MPS VII. We also confirm ultrasonographically observed carotid intimal-medial thickening is an in vivo correlate of histopathologic vascular fibrointimal hyperplasia. MPS VII patients should be regularly monitored for cardiac disease, with methods such as Holter monitors and stress testing; MPS VII-directed treatments should effectively address cardiovascular disease.

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Section: Histopathologymentioning

confidence: 72%