Natural history of cardiac involvement in myotonic dystrophy: Correlation with CTG repeats
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Abstract
Article abstract The authors prospectively studied the natural course of cardiac involvement and its relationship to cytosine–thymine–guanine (CTG) expansion in 50 patients with myotonic dystrophy who were submitted to periodic cardiovascular EKG and EKG–Holter monitoring during a median follow-up of 56 months. Nineteen patients (38%) developed major EKG changes. CTG length was not correlated with the frequency of EKG abnormalities, but was inversely correlated with the age at onset of EKG abnormalities (p < 0.0001). CTG length influences the timing of cardiac complications in myotonic dystrophy.
- Received January 20, 2000.
- Accepted June 12, 2000.
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