Natural history of cardiac involvement in myotonic dystrophy: correlation with CTG repeats.
prospective_cohort · Level II
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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.
Medical subject headings
- Heart Diseases
- Myotonic Dystrophy
- Trinucleotide Repeats