Fatal vacuolar cardiomyopathy: clinicopathological correlate

KK Poh, P Chai, RCC Wong, KB Tan - Journal of the American College of …, 2012 - jacc.org
KK Poh, P Chai, RCC Wong, KB Tan
Journal of the American College of Cardiology, 2012jacc.org
The coronary angiogram was normal. Myocardial biopsy was performed. The histopathology
featured hypertrophic cardiomyocytes with enlarged and hyperchromatic nuclei. There were
prominent intracytoplasmic perinuclear vacuoles (E, arrows). There was mild interstitial
fibrosis, but no significant inflammatory infiltrate, glycogen accumulation, or amyloid
deposition. Electron microscopy showed presence of membrane-bound vacuoles (F). Unlike
lysosomal disorders and glycogenoses, the vacuoles appeared empty, reflecting an …
The coronary angiogram was normal. Myocardial biopsy was performed. The histopathology featured hypertrophic cardiomyocytes with enlarged and hyperchromatic nuclei. There were prominent intracytoplasmic perinuclear vacuoles (E, arrows). There was mild interstitial fibrosis, but no significant inflammatory infiltrate, glycogen accumulation, or amyloid deposition. Electron microscopy showed presence of membrane-bound vacuoles (F). Unlike lysosomal disorders and glycogenoses, the vacuoles appeared empty, reflecting an idiopathic form of vacuolar cardiomyopathy (1). Mitochondria were focally increased, but did not show abnormal cristae patterns or crystalline inclusions. Despite optimal medical therapy, the patient expired soon after discharge.
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