Usefulness of Signal-Averaged Electrocardiography in Evaluation of Idiopathic-Dilated Cardiomyopathy in Families

Family studies have confirmed that familial dilated cardiomyopathy is common and that a large proportion of relatives have mild left ventricular enlargement and therefore potentially early disease. Previous studies have shown that patients with idiopathic dilated cardiomyopathy have abnormalities of the signal-averaged electrocardiogram (ECG). We assessed the usefulness of analysis of the signal-averaged ECG in the evaluation of familial dilated cardiomyopathy. Signal-averaged electrocardiographic recordings were obtained from 58 patients with idiopathic dilated cardiomyopathy, from 161 of their relatives (35 of whom had left ventricular enlargement), and from 59 healthy subjects. Signal-averaged ECGs were analyzed using both time domain and spectral turbulence analysis techniques. The time domain and spectral turbulence analysis variables were markedly abnormal in patients with idiopathic dilated cardiomyopathy compared with relatives considered normal and healthy controls (p <0.05). Late potentials were more common in patients with idiopathic dilated cardiomyopathy (17%) and in relatives with left ventricular enlargement (20%) than in normal relatives (5%) or healthy controls (5%) (p = 0.003). Spectral turbulence analysis was abnormal in 24% of patients with idiopathic dilated cardiomyopathy, 14% of relatives with left ventricular enlargement, 6% of normal relatives, and 5% of healthy controls (p = 0.0006). The sensitivity, specificity, and positive predictive accuracy for identification of relatives with left ventricular enlargement were 20%, 95%, and 54% for time domain analysis and 14%, 94%, and 42% for spectral turbulence analysis. Similar positive predictive accuracy was achievable with spectral turbulence analysis and with time domain analysis. In conclusion, the signal-averaged ECG is frequently abnormal in patients with idiopathic dilated cardiomyopathy and relatives with left ventricular enlargement.