Cardiac findings in an individual with neurofibromatosis 1 and sudden death.

摘要: Vascular lesions in neurofibromatosis 1 (NF1) are infrequently recognised as manifestations of the disease, yet they can produce serious complications. Most individuals with NF1 vasculopathy asymptomatic, which may contribute to underestimation its frequency. A recent study indicates that vascular changes mortality at younger ages. We report sudden death a young man NF1. On autopsy examination there was evidence an intramyocardial characteristic pathology previously described Other cardiac findings included non-specific cardiomyopathic changes, myocardial fibrosis, and “floppy” mitral valve. © 2001 Wiley-Liss, Inc.