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Familial dilated cardiomyopathy: a worse prognosis compared with sporadic forms.
  1. M. Csanády,
  2. M. Högye,
  3. A. Kallai,
  4. T. Forster,
  5. T. Szárazajtai
  1. 2nd Department of Medicine, Albert Szent-Györgyi Medical University, Szeged, Hungary.


    OBJECTIVE--To establish the time of onset of dilated cardiomyopathy (DCM) by review of annual chest x rays, which are obligatory in Hungary. DESIGN--A retrospective survey of chest x rays of a cohort of confirmed cases of DCM, to assess time of onset of cardiomegaly. Clinical course was compared by follow up over a mean of six years from the time of diagnosis. SUBJECTS--240 patients with DCM (31 familial, 209 non-familial). Diagnosis was made by echocardiography in all cases and confirmed by coronary angiography and heart biopsy in some cases. MAIN RESULTS--At diagnosis, the mean age of the patients was 31.8 years in the familial group and 39.6 years in the non-familial group (P < 0.05). The time between the onset of cardiomegaly (cardiothoracic ratio > 0.45) and clinical diagnosis was 8.0 and 10.1 years respectively (P < 0.05). The six year survival was 6% in the familial group and 23% in the non-familial group (P < 0.05). CONCLUSIONS--The familial form of DCM is the more malignant form: it occurs at an earlier age and progresses more rapidly than non-familial DCM.

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