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Non-sustained microvolt level T-wave alternans in congenital long QT syndrome types 1 and 2
Authors:Jovil A. Kannampuzha  Prasanna Sengodan  Sravani Avula  Bartholomew White  Stephen J. Ganocy  Peter J. Leo  Elizabeth S. Kaufman
Affiliation:Heart and Vascular Research Center, MetroHealth Campus, Case Western Reserve University, Cleveland, OH, USA
Abstract:

Background

Patients with long QT syndrome (LQTS) are predisposed to polymorphic ventricular tachycardia (VT) during adrenergic stimulation. Microvolt T-wave alternans (MTWA) is linked to vulnerability to VT in structural heart disease. The prevalence of non-sustained MTWA (NS-MTWA) in LQTS is unknown.

Methods

31 LQT1, 42 LQT2, and 80 controls underwent MTWA testing during exercise. MTWA tests were classified per standardized criteria, and re-analyzed according to the modified criteria to account for NS-MTWA.

Results

LQT1 and LQT2 patients had a significantly higher frequency of late NS-MTWA (26% and 12%) compared to controls (0%). There was no significant difference between the groups with respect to sustained and early NS-MTWA. Late NS-MTWA was significantly associated with QTc.

Conclusion

LQT1 and LQT2 patients had a higher prevalence of late NS-MTWA during exercise than matched controls. NS-MTWA likely reflects transient adrenergically mediated dispersion of repolarization, and could be a marker of arrhythmic risk in LQTS.
Keywords:DoR  dispersion of repolarization  LQTS  long QT syndrome  LQT1  long QT syndrome type 1  LQT2  long QT syndrome type 2  TdP  torsades de pointes  MTWA  microvolt T-wave alternans  NS-MTWA  non-sustained microvolt T-wave alternans  HR  heart rate  GNFC  gene-negative family controls  UHC  unrelated healthy controls  VT  ventricular tachycardia  Microvolt T-wave alternans  Congenital long QT syndrome  Dispersion of repolarization  Sudden cardiac death
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