Heart rate variability in infants with West syndrome

• During hypsarrhythmia the WS-patients had significantly lower heart rate variability.
• At the final examination the patients no longer differed from the controls.
• No prognostic factors concerning neither aetiology nor clinical outcome were found.

PurposeWest syndrome (WS) is a severe age-related acute epileptic encephalopathy of infancy characterized by infantile spasms, hypsarrhythmia and psychomotor delay. The aim of this study was to investigate if patients with WS had an altered autonomic output to the heart.MethodsIn 23 patients with WS the heart rate variability (HRV) was investigated by examining time- and frequency-domain parameters of HRV at the time of the diagnosis of hypsarrhythmia and compared to 22 age-matched controls. For the WS patients the same dataset was obtained and compared again at the end of the study period, when hypsarrhythmia was no longer present.ResultsCompared to controls, patients with WS during hypsarrhythmia had significantly lower SDNN (the standard deviation of the NN interval, i.e. the square root of variance) (19.2 ms; p = 0.007, Mann–Whitney's U-Test) and total power (242 ms2; p = 0.044, Mann–Whitney's U-Test) in the awake state, indicating an abnormal autonomic output to the heart. Comparing the initial to the final examination demonstrated a significant increase in the HRV parameters SDNN (31.3 ms) and total power (757 ms2; p = 0.001 and p = 0.013, Wilcoxon Signed Ranked Test). In addition, at the final examination the WS-patients no longer differed significantly from the controls.ConclusionOur data suggest that the initial reduction in HRV in patients at the time of onset of WS is transient and related to the presence of hypsarrhythmia.