Circulation. 2003;108:965-970
Published online before print August 18, 2003, doi: 10.1161/01.CIR.0000085071.28695.C4
Published online before print August 18, 2003, doi: 10.1161/01.CIR.0000085071.28695.C4
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(Circulation. 2003;108:965.)
© 2003 American Heart Association, Inc.
Clinical Investigation and Reports |
Short QT Syndrome
A Familial Cause of Sudden Death
From the Division of Cardiology (F.G., C.G., F.B., R.R., S.G., E.R.), Ospedale Mauriziano Umberto I, Torino, Italy; and the 1st Department of Medicine (C.W., R.S., M.B.), University Hospital Mannheim, University of Heidelberg, Mannheim, Germany.
Correspondence to Fiorenzo Gaita, MD, Division of Cardiology, Ospedale Mauriziano Umberto I, Largo Turati, 62, 10126 Torino, Italy. E-mail gaitaf{at}libero.it
Received March 17, 2003; revision received June 2, 2003; accepted June 3, 2003.
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.
Methods and 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 
280 ms (QTc 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.
Key Words: short QT interval • death, sudden • fibrillation
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|
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|
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|
|
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|
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|
|
|
|
|
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|
|
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|
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|
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|
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|
|
|
|
|
|
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|
|
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|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
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|
|