The specific features of the clinical picture and course of familial dilated cardiomyopathy

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Aim. To study prevalence, clinical and hemodynamic features, and life prognosis in patients with familial dilated cardiomyopathy (DCM). Subjects and methods. Two hundred and forty-four patients with DCM were examined during the period 2000 to 2010. According to their medical history records and the results of clinical and functional studies, familial cardiomyopathy was diagnosed in 29 (11.8%) of 244 patients. For comparative assessment, the authors formed two groups: 1) 29 patients with the familial form of DCM and 2) 83 patients with its sporadic form. Their examination included ECG, Holter ECG monitoring, 6-minute walk test, X-ray cardiometry, coronarography, and life prognosis assessment. Results. It has been established that the prevalence of the familial form of DCM accounts for 11.9% and is, unlike its non-familial form, associated with younger age; it is maternally inherited in one third of cases and characterized by the development of complete atrioventricular block in some patients. Conclusion. Investigating the life prognosis of the patients has shown that the familial form and age less than 30 years are characterized by a rapidly progressing course accompanied by a significant increase in death rates within the first 12 follow-up months.

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Особенности клинической картины и течения семейной формы дилатационной кардиомиопатии. - Резюме. Цель исследования. Изучить распространенность, клинико-гемодинамические особенности и прогноз жизни у больных с семейной формой дилатационной кардиомиопатии (ДКМП). Материалы и методы. За период с 2000 по 2010 г. обследовали 244 больных ДКМП. По результатам анамнеза и клинико-функциональных методов исследования семейная форма кардиомиопатии диагностирована у 29 (11,8%) из 244 больных. Для сравнительной оценки были сформированы две группы: 1-я - 29 больных с семейной формой ДКМП, 2-я - 83 больных со спорадической формой. Обследование включало ЭКГ, холтеровское мониторирование ЭКГ, тест с 6-минутной ходьбой, рентгенокардиометрию, коронарографию, оценку прогноза жизни. Результаты. Установлено, что распространенность семейной формы ДКМП составляет 11,9% и, в отличие от несемейной формы заболевания, ассоциируется с более молодым возрастом, в 1/3 случаев передается по материнской линии, у отдельных пациентов характеризуется развитием полной атриовентрикулярной блокады. Заключение. Изучение прогноза жизни больных показало, что семейная форма и возраст моложе 30 лет характеризуются быстро прогрессирующим течением, сопровождающимся достоверным увеличением смертности в течение первых 12 мес наблюдения.


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