Intervalle QT. 5. Court

The SQTS is a rare, sporadic, or autosomal dominant disorder characterized by markedly accelerated cardiac repolarization and manifested by dramatically shortened QT interval, atrial and ventricular arrhythmias, and sudden cardiac death. To date, mutations in potassium (KCNH2 , KCNQ1 , KCNJ2) and calcium channel subunit genes  have been identifi ed as causing SQTS. The clinical manifestations of this disorder have been characterized in a relatively small number of families and sporadic individuals, given the recent identifi cation and rare nature of SQTS.

SQTS is defined (Gollob, 2006) as a QTc interval ≤ 340 ms or QTc interval between 341 ms and 360 ms and 1 or more of the following: history of CA or syncope, a family history of unexplained CA at a young age (40 years of age or younger), or a family history of SQTS. 

The QTp was calculated using the formula developed by

Rautaharju et al. : [QTp = 656/(1 + heart rate/100)]

Plus le QTc est court plus le risque d’arythmie augmente (ex. QTc très court < 320 ms)

voir aussi

A Novel Familial Cardiac Arrhythmia Syndrome with Widespread ST-Segment Depression NEJM 2018


Gollob MH, Redpath CJ, Roberts JD. The short QT syndrome: proposed diagnostic criteria. J Am Coll Cardiol. 2011 Feb 15;57(7):802-12.

Mazzanti A, Kanthan A, Monteforte N, et al. Novel insight into the natural history of short QT syndrome. J Am Coll Cardiol. 2014 Apr 8;63(13):1300-8.

–> In this study, we showed that having survived a first occurrence of CA is a strong predictor of recurrences. This information is important because it supports the need to consider implanting an ICD for secondary prevention of CA even in young SQTS patients.

Anttonen O, Junttila J, Giustetto C, et al. T-Wave morphology in short QT syndrome. Ann Noninvasive Electrocardiol. 2009 Jul;14(3):262-7.