CMAJ • November 22, 2005; 173 (11). doi:10.1503/cmaj.050596.
© 2005 CMA Media Inc. or its licensors
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Review

Short QT syndrome

Ramon Brugada, Kui Hong, Jonathan M. Cordeiro and Robert Dumaine

From the Montreal Heart Institute (Brugada), Montréal, Qué.; Baylor College of Medicine (Hong), Houston, Texas; Masonic Medical Research Laboratory (Cordeiro), Utica, New York; and the University of Sherbrooke (Dumaine), Sherbrooke, Qué.

Correspondence to: Dr. Ramon Brugada, Montreal Heart Institute, 5000 rue Belanger, Montréal QC H1T 1C8; ramon{at}brugada.org

Abstract

The QT interval on an electrocardiogram signifies the time required for the heart to repolarize after depolarization. It has long been appreciated that a long QT interval predisposes patients to life-threatening ventricular arrhythmia. Short QT syndrome is a newly described disease characterized by a shortened QT interval and by episodes of syncope, paroxysmal atrial fibrillation or life-threatening cardiac arrhythmias. The syndrome usually affects young and healthy people with no structural heart disease and may be present in sporadic cases as well as in families. Our understanding of a new disease has rarely benefitted so quickly from research in genetics, molecular biology and biophysics. It was first described in 2000 in a handful of patients, and since then 3 different genes associated with the disease and the biophysical basis have been described, and therapy has been made available. Here we review the current understanding of the pathophysiology, clinical presentation and treatment of short QT syndrome.




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