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Through family evaluation, 33 additional genetic carriers were identified. The study included 54 genetic carriers 56 men with a mean age of 41 В 17 years; 33 hypertrophic cardiomyopathies, 9 dilated and 1 non-compacted, with a maximum thickness of 18. 5 В 6mm; with ventricular dysfunction 30 and a history of sudden death 62. During follow-up, 4 died and 14 33 received a defibrillator 8 probands, 6 family members. The median survival was 54 years. Arg92Gln carriers had early development, high penetrance, high risk of sudden death, high rate of defibrillator implantation, and high frequency of mixed phenotype. Enfin, la mise Г disposition du traitement spГcifique et adГquat, au sein mГme civalex leur communautГ, aide Г faire comprendre quвune telle violence civalex de graves consГquences mГdicales et quвelle ne devrait pas Гtre acceptГe ni acceptable.
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