LAMP2 cardiomyopathy in a 12-year-old boy with sudden ...

LAMP2 cardiomyopathy in a 12-year-old boy with sudden death (Table 1; patient #1). A. At autopsy, massive asymmetric LV hypertrophy. Ventricular septal (VS) thickness is 65 mm (heart weight, 1425 grams), exceeding all hearts reported to date; LV cavity is small. Ao = aorta; LVFW = left ventricular free wall. B. Disorganized LV architecture. Adjacent cardiac muscle cells (myocytes), or groups of cells, are arranged at perpendicular or oblique angles. Masson’s trichrome stain x100. C. Area of LV wall, demarcated by the broken line in A, showing subepicardial necrosis and scarring. D. Abnormal intramural coronary artery with thickened wall and narrowed lumen. PAS stain x20. E. Intracardiac ventricular electrogram. The ICD elicited a defibrillation shock (arrow) which failed to interrupt ventricular fibrillation (280 beats/min). This event was repeated 5 times until ICD capacity was extinguished and death occurred.

#Clinical #Gross #Pathology #HOCM #HCM #Danon #Disease #LAMP2 #Hypertrophic #Obstructive #Cardiomyopathy
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