RT Journal Article
SR Electronic
T1 Short QT syndrome
JF Canadian Medical Association Journal
JO CMAJ
FD Canadian Medical Association
SP 1349
OP 1354
DO 10.1503/cmaj.050596
VO 173
IS 11
A1 Ramon Brugada
A1 Kui Hong
A1 Jonathan M. Cordeiro
A1 Robert Dumaine
YR 2005
UL http://www.cmaj.ca/content/173/11/1349.abstract
AB 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.