Cardiac autonomic dysfunction in adult congenital heart disease

Purpose Due to recent advances in diagnosis and treatment, the number of adults with congenital heart disease (ACHD) has substantially increased. This achievement is mitigated by rhythm disorders. Here, we sought to determine alterations in heart rate variability (HRV) and their prognostic value in ACHD.Methods Ninety seven ACHD patients (39.2 +/- 14.1 years, 51.5% female) and 19 controls (39.7 +/- 15.0 years, 47.4% female) underwent 24-h Holter monitoring.Results As compared to controls, ACHD patients offered a significantly higher burden of premature ventricular contractions (p = 0.02) and decreased HRV indices (natural logarithmic transformation of very low frequency (lnVLF): 7.46 +/- 0.76 ms(2) vs. 7.91 +/- 0.92ms(2), p = 0.03; natural logarithmic transformation of low frequency (lnLF): 6.39 +/- 0.95ms(2) vs. 7.01 +/- 1.07ms(2), p = 0.01; natural logarithmic transformation of the ratio of low to high frequency spectra (lnLF/HF): 0.81 +/- 0.74 vs. 1.17 +/- 0.51, p = 0.04). No differences in HRV measures were observed across ACHD lesion groups. NT-proBNP levels were significantly related to both time and frequency domain indices (natural logarithmic transformation of the standard deviation of NN intervals (lnSDNN): Spearman<acute accent>s rho = -0.32, p = 0.001; natural logarithmic transformation of the standard deviation of the average NN intervals for each 5-min segment of a 24-h Holter monitoring (lnSDANN): Spearman<acute accent>s rho: -0.33, p = 0.001; natural logarithmic transformation of the total power (lnTP): Spearman<acute accent>s rho: -0.25, p = 0.01; lnVLF: Spearman<acute accent>s rho: -0.33, p = 0.001; lnLF: Spearman<acute accent>s rho: -0.35, p < 0.001; lnLF/HF: Spearman<acute accent>s rho: -0.34, p = 0.001).After a mean follow-up of 3.9 +/- 0.7 years, 8 patients died and 3 patients survived sudden cardiac death (SCD). Several HRV parameters were significantly higher in event-free ACHD patients than in those who died or survived SCD (natural logarithmic transformation of the average of the standard deviations of NN intervals for each 5-min segment of a 24-h Holter monitoring (lnASDNN): p = 0.04; lnPNN30: p = 0.04; lnVFL: p = 0.03; lnLF: p < 0.01). On univariate Cox regression analysis, the time domain indices lnSDNN, lnASDNN and lnPNN30, as well as the frequency domain parameters lnTP, lnVLF and lnLF were associated with death and survived cardiac arrest.Conclusion ACHD is accompanied by HRV impairment that carries prognostic implications on ACHD mortality and survived SCD.