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Cardiomyopathie hypertrophiqueby: P. Charron, M. Komajda
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AbstractResumeLa cardiomyopathie hypertrophique est caracterisee par une hypertrophie typiquement asymetrique predominant sur le septum interventriculaire. Dans un quart des cas s'y associe un gradient de pression intraventriculaire gauche. La maladie est le plus souvent familiale et les genes responsables codent pour des proteines du sarcomere. L'evolution est le plus souvent favorable mais les complications redoutees sont la mort subite et l'insuffisance cardiaque progressive. La prise en charge therapeutique reste difficile. Si les symptomes ne sont pas controles par les betabloquants ou le verapamil, et s'il existe un gradient de pression, la chirurgie de myotomie-myectomie peut etre proposee. Le stimulateur cardiaque double chambre et l'alcoolisation d'artere coronaire septale constituent deux alternatives a la chirurgie. En cas de risque de mort subite le traitement fait appel au defibrillateur implantable, ou parfois a l'amiodarone.AbstractHypertrophic cardiomyopathy is characterized by an asymmetric hypertrophy of the left ventricle, especially in the interventricular septum. An outflow gradient in the left ventricle is present in twenty-five percent of cases. The disease is usually hereditary and genes responsible for the disease encode for the sarcomere proteins. The natural course is generally favourable but sometimes complications may occur, such as sudden death or congestive heart failure. Treatment remains difficult. If symptoms are not controlled by beta-blockers or verapamil, and if a pressure gradient is present, surgery with myotomy-myectomy may be proposed. Other treatments in such case are dual-chamber pacing or non-surgical septum reduction (alcohol injection). In patients with high-risk of sudden death, amiodarone or implantable cardioverter-defibrillator can be proposed.
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