title: Electrocardiographic transmural dispersion of repolarization in patients with inherited short QT syndrome.
abstract: BACKGROUND
  Short QT syndrome (SQTS) carries an increased risk for sudden cardiac death. However, only a short QT interval does not express the risk of ventricular arrhythmias. Thus, additional evaluation of the repolarization abnormality in SQTS patients is essential. In experimental models of SQTS, increased transmural dispersion of repolarization (TDR) and its electrocardiographic counterpart T-wave peak to T-wave end interval (TPE) appeared critical for induction of polymorphic ventricular tachycardia (PMVT). In a clinical study with acquired long QT syndrome patients, TPE/QT ratio > 0.28 indicated arrhythmia risk. We hypothesized that the TPE/QT ratio would be greater in SQTS patients than in control subjects.
  
  
  RESULTS
  We compared the behavior of the electrocardiographic TDR in three seriously symptomatic SQTS patients of unknown genotype presenting baseline QTc values < 320 ms and in nine healthy age-matched control subjects. We determined QT and TPE intervals as well as TPE/QT ratio from 24-hour ECG recordings using a computer-assisted program. Diurnal average of TPE/QT ratio was 0.28 +/- 0.03 in SQTS patients and 0.21 +/- 0.02 in control subjects (P = 0.01). SQTS patients had also lesser capacity to change TPE intervals from steady-state conditions to abrupt maximal values than control subjects.
  
  
  CONCLUSIONS
  SQTS patients have increased and autonomically uncontrolled electrocardiographic TDR. According to experimental SQTS models, the present results may in part explain increased vulnerability of SQTS patients to ventricular arrhythmias.
authors: Department of Medicine, Päijät-Häme Central Hospital, Lahti, Finland. olli.anttonen@phsotey.fi
authors: Olli
authors: O
authors: Anttonen
authors: Heikki
authors: H
authors: Väänänen
authors: Juhani
authors: J
authors: Junttila
authors: Heikki V
authors: HV
authors: Huikuri
authors: Matti
authors: M
authors: Viitasalo
mesh_terms: D000293:Adolescent; D000369:Aged, 80 and over; D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000235:genetics*; D016022:Case-Control Studies; D004562:Electrocardiography / Q000379:methods*; D015716:Electrocardiography, Ambulatory / Q000379:methods; D022062:Electrophysiologic Techniques, Cardiac / Q000379:methods; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006329:Heart Conduction System / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction / Q000502:physiology; D010375:Pedigree; D011336:Probability; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D013577:Syndrome; D020257:Ventricular Remodeling / Q000502:physiology