Csanády M, Högye M, Kallai A, Forster T, Szárazajtai T
2nd Department of Medicine, Albert Szent-Györgyi Medical University, Szeged, Hungary.
Br Heart J. 1995 Aug;74(2):171-3. doi: 10.1136/hrt.74.2.171.
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.
通过回顾匈牙利规定必须进行的年度胸部X光片，确定扩张型心肌病（DCM）的发病时间。
对一组确诊的DCM病例的胸部X光片进行回顾性调查，以评估心脏扩大的发病时间。从诊断时间起平均随访六年，比较临床病程。
240例DCM患者（31例为家族性，209例为非家族性）。所有病例均通过超声心动图进行诊断，部分病例通过冠状动脉造影和心脏活检进行确诊。
诊断时，家族性组患者的平均年龄为31.8岁，非家族性组为39.6岁（P<0.05）。心脏扩大（心胸比率>0.45）开始至临床诊断的时间分别为8.0年和10.1年（P<0.05）。家族性组的六年生存率为6%，非家族性组为23%（P<0.05）。
家族性DCM是更恶性的形式：它比非家族性DCM发病年龄更早，进展更快。
