on August 18, 2003
Circulation. 2003
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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Submitted on March 17, 2003
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. * To whom correspondence should be addressed. E-mail: gaitaf{at}libero.it.
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 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.
Revised on June 2, 2003
Accepted on June 3, 2003
Short QT Syndrome. A Familial Cause of Sudden Death
Fiorenzo Gaita MD*,
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.
Key words: short QT interval • death, sudden • fibrillation
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