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Degree Of Diminution In Vagal-Cardiac Activity Predicts Sudden Death In Familial Dysautonomia When Resting Tachycardia Is AbsentPatients with familial dysautonomia (FD) have an increased risk of sudden death, but sensitive and specific predictors of sudden death in FD are lacking. Methods. We recorded 10-min resting high-fidelity 12-lead ECGs in 14 FD patients and in 14 age/gender-matched healthy subjects and studied 25+ different heart rate variability (HRV) indices for their ability to predict sudden death in the FD patients. Indices studied included those from 4 "nonlinear" HRV techniques (detrended fluctuation analysis, approximate entropy, correlation dimension, and PoincarC analyses). The predictive value of PR, QRS, QTc and JTc intervals, QT dispersion (QTd), beat-to-beat QT and PR interval variability indices (QTVI and PRVI) and 12- lead high frequency QRS ECG (150-250 Hz) were also studied. FD patients and controls (C) differed (Pless than 0.0l) with respect to 20+ of the HRV indices (FD less than C) and with respect to QTVI and PRVI (FDBC) and HF QRS- related root mean squared voltages (FDBC) and reduced amplitude zone counts (FD less than C). They differed less with respect to PR intervals (FD less than C) and JTc intervals (FD greater than C) (P less than 0.05 for both) and did not differ at all with respect to QRS and QTc intervals and to QTd. Within 12 months after study, 2 of the 14 patients succumbed to sudden cardiac arrest. The best predictor of sudden death was the degree of diminution in HRV vagal-cardiac (parasympathetic) parameters such as RMSSD, the SDl of Poincare plots, and HF spectral power. Excluding the two FD patients who had resting tachycardia (HR greater than 100, which confounds traditional HRV analyses), the following criteria were independently 100% sensitive and 100% specific for predicting sudden death in the remaining 12 FD patients during spontaneous breathing: RMSSD less than 13 ms and/or PoincarC SD1 less than 9 ms. In FD patients without supine tachycardia, the degree of diminution in parasympathetic HRV parameters (by high-fidelity ECG) predicts incipient death.
Document ID
20050217297
Acquisition Source
Johnson Space Center
Document Type
Conference Paper
Authors
Schlegel, T. T.
(NASA Johnson Space Center Houston, TX, United States)
Marthol, H.
(NASA Johnson Space Center Houston, TX, United States)
Bucchner, S.
(NASA Johnson Space Center Houston, TX, United States)
Tutaj, M.
(NASA Johnson Space Center Houston, TX, United States)
Berlin, D.
(NASA Johnson Space Center Houston, TX, United States)
Axelrod, F. B.
(NASA Johnson Space Center Houston, TX, United States)
Hilz, M. J.
(NASA Johnson Space Center Houston, TX, United States)
Date Acquired
August 23, 2013
Publication Date
January 1, 2004
Subject Category
Life Sciences (General)
Meeting Information
Meeting: American Autonomic Society Meeting
Location: Amsterdam
Country: Netherlands
Start Date: October 20, 2004
End Date: October 23, 2004
Distribution Limits
Public
Copyright
Work of the US Gov. Public Use Permitted.

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