PMID: 7546997Aug 1, 1995Paper

Familial dilated cardiomyopathy: a worse prognosis compared with sporadic forms

British Heart Journal
M CsanádyT Szárazajtai

Abstract

To establish the time of onset of dilated cardiomyopathy (DCM) by review of annual chest x rays, which are obligatory in Hungary. 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. 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. 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). 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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Citations

Feb 1, 1997·Trends in Cardiovascular Medicine·T M Olson, M T Keating
Feb 1, 2003·European Journal of Heart Failure·Ekkehard GrünigHugo A Katus
Jul 30, 2011·The American Journal of Cardiology·Mario PetrettaDomenico Bonaduce
Apr 20, 1999·Circulation·X JouvenP Ducimetière
Oct 1, 2001·Journal of Genetic Counseling·E L Hanson, R E Hershberger
Jul 7, 2020·Circulation Journal : Official Journal of the Japanese Circulation Society·Kyohei MarumeSatoshi Yasuda
Nov 13, 2020·ESC Heart Failure·Folkert W AsselbergsUNKNOWN Cardiomyopathy & Myocarditis Registry Investigators Group

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