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Electrocardiogram Abnormalities Suggest Aberrant Cardiac Conduction in Huntington's Disease
Christopher D Stephen1, Judy Hung2, Giovanni Schifitto3
1Departments of Neurology Center for Neuro-imaging of Aging and Neurodegenerative Diseases.
Insights
Huntington's disease (HD) significantly impacts the heart, with many early-stage patients showing cardiac conduction abnormalities on electrocardiograms (ECGs). These findings suggest a higher risk of arrhythmias in HD patients.
Area of Science:
- Neurology
- Cardiology
- Genetics
Background:
- Huntington's disease (HD) effects extend beyond the central nervous system.
- Cardiac dysfunction is increasingly recognized in HD, posing a risk for sudden cardiac death.
Purpose of the Study:
- To investigate the prevalence of cardiac abnormalities in early symptomatic Huntington's disease patients.
Main Methods:
- Analyzed 12-lead electrocardiograms (ECGs) from 590 early symptomatic HD patients (Stage 1 and 2).
- Excluded patients on certain medications or with confounding medical conditions.
Main Results:
- Prevalence of bradycardia was 28.3% (5.8% marked).
- Other findings included prolonged QRS (4.9%), intraventricular conduction delay (3.4%), right bundle branch block (1.3%), and QTc prolongation (3.7%).
Conclusions:
- A significant number of early symptomatic HD patients exhibit cardiac conduction abnormalities.
- Intraventricular conduction delays may increase arrhythmia risk, potentially exacerbated by QT-prolonging medications.
Background:
There is increasing evidence that the effects of Huntington's disease (HD) extend beyond the central nervous system. In particular, significant cardiac dysfunction has been described in transgenic mouse models and suggested in symptomatic patients, in whom cardiac involvement could provide an independent risk for sudden cardiac death.
Methods:
Standard 12-lead electrocardiograms (ECGs) obtained at screening from 590 early symptomatic (Stage 1 and 2) HD patients participating in a multi-site Phase III study were analyzed.
Results:
Evaluating only those ECGs in individuals not on medications or with potentially contributing medical conditions, the prevalence of bradycardia was 28.3% (marked in 5.8%), prolonged QRS 4.9%, intraventricular conduction delay 3.4%, right bundle branch block 1.3%, and QTc prolongation 3.7%.
Conclusion:
Significant cardiac abnormalities, characterized primarily by conduction abnormalities, were found in a larger than expected number of patients. Abnormal intraventricular conduction may lead to increased risk for arrhythmia and may be compounded by prescription of QT-prolonging medications.