title: Short QT Syndrome: a familial cause of sudden death.
abstract: BACKGROUND
  A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse-a short QT interval. Several members of 2 different families were referred for syncope, palpitations, and resuscitated cardiac arrest in the presence of a positive family history for sudden cardiac death. Autopsy did not reveal any structural heart disease. All patients had a constantly and uniformly short QT interval at ECG.
  
  
  RESULTS
  Six patients from both families were submitted to extensive noninvasive and invasive work-up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 patients underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural heart disease was excluded. At baseline ECG, all patients exhibited a QT interval <or=280 ms (QTc <or=300 ms). During electrophysiological study, short atrial and ventricular refractory periods were documented in all and increased ventricular vulnerability to fibrillation in 3 of 4 patients.
  
  
  CONCLUSIONS
  The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation. It is important to recognize this ECG pattern because it is related to a high risk of sudden death in young, otherwise healthy subjects.
authors.affiliation: Division of Cardiology, Ospedale Mauriziano Umberto I, Torino, Italy. gaitaf@libero.it
authors.forename: Fiorenzo
authors.initials: F
authors.lastname: Gaita
authors.forename: Carla
authors.initials: C
authors.lastname: Giustetto
authors.forename: Francesca
authors.initials: F
authors.lastname: Bianchi
authors.forename: Christian
authors.initials: C
authors.lastname: Wolpert
authors.forename: Rainer
authors.initials: R
authors.lastname: Schimpf
authors.forename: Riccardo
authors.initials: R
authors.lastname: Riccardi
authors.forename: Stefano
authors.initials: S
authors.lastname: Grossi
authors.forename: Elena
authors.initials: E
authors.lastname: Richiardi
authors.forename: Martin
authors.initials: M
authors.lastname: Borggrefe
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001145:Arrhythmias, Cardiac / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D001281:Atrial Fibrillation / Q000175:diagnosis / Q000209:etiology; D005117:Cardiac Complexes, Premature / Q000209:etiology; D002648:Child; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D022062:Electrophysiologic Techniques, Cardiac; D005080:Exercise Test; D005260:Female; D005424:Flecainide / Q000494:pharmacology; D006323:Heart Arrest / Q000209:etiology; D006801:Humans; D008297:Male; D010375:Pedigree; D015222:Sodium Channels / Q000187:drug effects; D013575:Syncope / Q000209:etiology; D013577:Syndrome
chemical_list: D015222:Sodium Channels; D005424:Flecainide