Catecholamine-provoked microvoltage T wave alternans in genotyped long QT syndrome

Jan Nemec, Michael John Ackerman, David J. Tester, Joseph Hejlik, Win Kuang Shen

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

Macrovoltage T wave alternans (TWA) has been described in congenital long QT syndrome (LQTS). Microvoltage T wave alternans (μ V-TWA) at low heart rate (HR) is a marker of arrhythmogenic risk in many conditions, but its significance in LQTS has not been established. Twenty-three genotypically heterogeneous patients with LQTS and 16 control subjects were studied at rest and during phenylephrine and dobutamine provocation. Genotyping was established by PCR amplification and DNA sequencing of the three most common LQTS genes; KCNQ1/KVLQT1 (LQT1), KCNH2/HERG (LQT2), and SCN5A (LQT3). μ V-TWA was determined using Fast Fourier transform. Precluded by ectopy, μ V-TWA could not be assessed in 8 of 23 patients with LQTS. In the remaining 15 patients with LQTS, μ V-TWA occurred at lower HR in LQTS than in controls (117 ± 49 vs 153 ± 37 beats/min; P < 0.05). Patients with LQTS developed μ V-TWA at HR < 150 beats/min more often than controls (10/15 vs 2/16; P = 0.003). However, μ V-TWA was not detected in the 3 individuals with a history of out-of-hospital cardiac arrest including a 14-year-old male with an F339del-KVLQT1 mutation (LQT1) who had dobutamine-provoked polymorphic ventricular tachycardia requiring external defibrillation. Catecholamine-provoked μ V-TWA occurs at lower HR in patients with LQTS than in healthy people but does not identify high risk subjects.

Original languageEnglish (US)
Pages (from-to)1660-1667
Number of pages8
JournalPACE - Pacing and Clinical Electrophysiology
Volume26
Issue number8
DOIs
StatePublished - Aug 1 2003

Fingerprint

Long QT Syndrome
Catecholamines
Heart Rate
Dobutamine
Out-of-Hospital Cardiac Arrest
Phenylephrine
Fourier Analysis
Ventricular Tachycardia
DNA Sequence Analysis
Polymerase Chain Reaction
Mutation

Keywords

  • Dobutamine
  • Genetics
  • Ion channels
  • Long QT syndrome
  • Noninvasive risk stratification
  • T Wave alternans
  • Torsade de pointes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Catecholamine-provoked microvoltage T wave alternans in genotyped long QT syndrome. / Nemec, Jan; Ackerman, Michael John; Tester, David J.; Hejlik, Joseph; Shen, Win Kuang.

In: PACE - Pacing and Clinical Electrophysiology, Vol. 26, No. 8, 01.08.2003, p. 1660-1667.

Research output: Contribution to journalArticle

Nemec, Jan ; Ackerman, Michael John ; Tester, David J. ; Hejlik, Joseph ; Shen, Win Kuang. / Catecholamine-provoked microvoltage T wave alternans in genotyped long QT syndrome. In: PACE - Pacing and Clinical Electrophysiology. 2003 ; Vol. 26, No. 8. pp. 1660-1667.
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