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Arrhythmias in Williams Syndrome
Anna M Deitch1, Heather M Giacone1, Henry Chubb1
1Department of Pediatric Cardiology, Lucile Packard Children's Hospital, Stanford University, Stanford, California.
The American Journal of Cardiology
|April 10, 2023
Summary
Atrial arrhythmias are common in Williams syndrome (WS), a genetic disorder. Routine ECG monitoring is recommended for WS patients due to increased sudden cardiac death risk.
Area of Science:
- Cardiology
- Genetics
- Pediatrics
Background:
- Williams syndrome (WS) is a genetic disorder associated with a high prevalence of cardiovascular abnormalities.
- Patients with WS have a significantly increased risk of sudden cardiac death and abnormal cardiac repolarization.
Purpose of the Study:
- To determine the prevalence of primary arrhythmias in patients with Williams syndrome.
- To investigate whether QTc prolongation influences arrhythmia risk in WS patients.
Main Methods:
- Retrospective review of ambulatory electrocardiogram (ECG) monitoring data from 74 WS patients (October 2017 - January 2022).
- Analysis of arrhythmia presence, premature ventricular/atrial complex burden, QTc interval changes, and clinical factors.
- Primary outcome was the presence of any arrhythmia.
Main Results:
- Arrhythmias were detected in 12% of WS patients; 33% of those with arrhythmias had more than one type.
- Atrial tachycardia was the most frequent arrhythmia, identified in 8% of the total cohort.
- Older age and symptoms like syncope were associated with arrhythmias, not the degree of structural heart disease.
Conclusions:
- Atrial arrhythmias are the most common cardiac rhythm disturbances in Williams syndrome.
- Routine ambulatory ECG and intermittent rhythm monitoring are indicated for WS patients.
- Monitoring is crucial given the elevated risk of sudden cardiac death in this population.
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