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Medical records and follow-up data were reviewed in 297 genetically proven myotonic dystrophy type 2 (DM2) patients. Patients were selected by the criteria of cardiac sudden death before age 45. Sudden death occurred in four patients, three of whom were cardiological asymptomatic, and one with a history of heart f ailure. Cardiac histopathology showed dilated cardiomyopathy in all, and conduct ion system fibrosis in two patients. Pathogenetic CCUG ribonuclear inclusions we re demonstrable in cardiomyocytes.
Patients were selected by the criteria of cardiac sudden death before age 45. Sudden death occurred in four patients, three of whom were cardiological asymptomatic, and one with a history of heart f ailure. Cardiac histopathology showed dilated cardiomyopathy in all, and conduct ion system fibrosis in two patients. Pathogenetic CCUG ribonuclear inclusions we re demonstrable in cardiomyocytes.