The frequency of familial dilated cardiomyopathy in a series of patients with idiopathic dilated cardiomyopathy

The New England Journal of Medicine
V V MichelsH D Tazelaar


Dilated cardiomyopathy is characterized by an increase in ventricular size and impairment of ventricular function. Most cases are believed to be sporadic, and familial dilated cardiomyopathy is usually considered to be a rare and distinct disorder. We studied the proportion of cases of idiopathic dilated cardiomyopathy that were familial in a large sequential series of patients whose first-degree relatives were investigated regardless of whether these relatives had cardiac symptoms. We studied relatives of 59 index patients with idiopathic dilated cardiomyopathy of obtaining a family history and performing a physical examination, electrocardiography, and two-dimensional, M-mode, and Doppler echocardiography. A total of 315 relatives were examined. Eighteen relatives from 12 families were shown to have dilated cardiomyopathy. Thus, 12 of the 59 index patients (20.3 percent) had familial disease. There was no difference in age, sex, severity of disease, exposure to selected environmental factors, or electrocardiographic or echocardiographic features between the index patients with familial disease and those with nonfamilial disease. A noteworthy finding was that 22 of 240 healthy relatives (9.2 percent) with normal ejection fract...Continue Reading


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Related Concepts

Cardiomyopathy, Familial Idiopathic
Neurologic Manifestations
Cardiac Symptom
Left Ventricular Function
2D Echocardiography
Idiopathic Dilation Cardiomyopathy
2D Doppler Echocardiography
Ventricular Function
Echocardiography, Doppler

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