Frequency of Familial DCM Among Patients With Idiopathic Cardiomyopathy
DCM had been considered to be familial in only a small number of cases until 1992, when an unselected series of DCM patients was enrolled in a study to prospectively evaluate their first-degree relatives, regardless of whether or not there was any suspicion of familial disease. In that study, relatives underwent a complete review of medical history, a cardiovascular examination, an electrocardiogram, and an M mode and two-dimensional Doppler echocardiogram. It was determined that 24% of patients with DCM had at least one first-degree relative with DCM based on echocardiographic findings of left ventricular ejection fraction (LVEF) <50% and left ventricular (LV) size above the 95th percentile for age and body size, and absence of coronary disease or other possible etiologies for decreased ventricular function. Coronary disease was excluded by coronary angiography in patients aged 40 years or older. Similar studies were published confirming and extending these observations.[5,6] In these studies, cardiac catheterization was performed in the affected relatives to exclude coronary atherosclerosis. The frequency varied from 25%-35%, depending on the strictness of the criteria (e.g., whether patients with an enlarged LV without systolic dysfunction were included). These observations prompted a search for the molecular genetic etiologies of DCM.
Cardiovasc Rev Rep. 2004;25(5) © 2004 Le Jacq Communications, Inc.
Cite this: Familial Dilated Cardiomyopathy - Medscape - Sep 01, 2004.