Heart Rate Variability Analysis May Identify Individuals With Williams-Beuren Syndrome at Risk of Sudden Death
Authors' conclusion
Individuals with WBS demonstrate significant HRV abnormalities consistent with diminished autonomic reserve.
Study context
- Population
- Individuals with Williams-Beuren syndrome and age- and sex-matched controls
- Participants
- 18 vs 18
- Exposure
- Williams-Beuren syndrome (WBS) vs matched controls
- WBSn = 18
- Controlsn = 18reference
- Outcome
- Autonomic activity as reflected by heart rate variability (HRV) measures on 24-hour ECG (nonlinear, time and frequency domain)
Methodological detailsprospective, age- and sex-matched controls
- Prospective
- Age- and sex-matched controls
Main finding
As reported in the abstract
HRV and indirect markers of parasympathetic activity
- Diminished SD of NN intervals in WBS
- P = 0.0001
- Diminished SD of average NN interval for 5-min intervals over 24 h in WBS
- P < 0.0001
- Diminished average of 5-min SDs of NN intervals for 24 h in WBS
- P = 0.0002
- Diminished root mean square of successive differences of NN intervals in WBS
- P = 0.0004
- Diminished short axis of Poincaré plot (SD1) in WBS
- P < 0.0001
- Diminished long axis of Poincaré plot in WBS
- P < 0.0001
- Diminished percent of NN intervals different from previous by ≥50% of local average in WBS
- P < 0.0007
- Diminished natural log high-frequency power in WBS
- P = 0.0038
- Decreased natural log very low-frequency power in WBS
- P = 0.0002
- Decreased natural log low-frequency power in WBS
- P = 0.0024
- Decreased SD1 divided by long axis of Poincaré plot in WBS
- P < 0.0001
Observational data: associations, not effects.
Original publication
Heart Rate Variability Analysis May Identify Individuals With Williams-Beuren Syndrome at Risk of Sudden Death. JACC Clin Electrophysiol. Published 30 November 2022. doi:10.
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