Congenital Short QT Syndrome

Antzelevitch, Charles and Francis, Johnson (2004) Congenital Short QT Syndrome. [Journal (Paginated)]

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Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms).2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS) was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death.

Item Type:Journal (Paginated)
Keywords:short QT syndrome; genetic studies
Subjects:JOURNALS > Indian Pacing and Electrophysiology Journal
ID Code:4198
Deposited By: Indian Pacing and Electrophysiology, Journal
Deposited On:13 Apr 2005
Last Modified:11 Mar 2011 08:55

References in Article

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1. Jervell A, Lange-Nielsen F. Congenital deaf-mutism, functional heart disease with prolongation of the QT interval and sudden death. Am Heart J. 1957;54:59-68.

2. Algra A, Tijssen JGP, Roelandt JRTC, Pool J, Lubsen J. QT interval variables from 24-Hour electrocardiography and the 2- Year risk of sudden death. Br Heart J. 1993;70:43-48.

3. Gussak I, Brugada P, Brugada J, Wright RS, Kopecky SL, Chaitman BR, Bjerregaard P. Idiopathic short QT interval: a new clinical syndrome? Cardiology. 2000;94:99-102.

4. Gussak I, Brugada P, Brugada J, Antzelevitch C, Osbakken M, Bjerregaard P. ECG phenomenon of idiopathic and paradoxical short QT intervals. Cardiac Electrophysiology Review. 2002;6:49-53.

5. Gaita F, Giustetto C, Bianchi F, Wolpert C, Schimpf R, Riccardi R, Grossi S, Richiardi E, Borggrefe M. Short QT Syndrome: a familial cause of sudden death. Circulation. 2003;108:965-970.

6. Cheng TO. Digitalis administration: an underappreciated but common cause of short QT interval. Circulation. 2004;109:e152.

7. Brugada R, Hong K, Dumaine R, Cordeiro JM, Gaita F, Borggrefe M, Menendez TM, Brugada J, Pollevick GD, Wolpert C, Burashnikov E, Matsuo K, Wu YS, Guerchicoff A, Bianchi F, Giustetto C., Schimpf R, Brugada P, Antzelevitch C. Sudden Death associated with Short QT Syndrome linked to Mutations in HERG. Circulation. 2004;109:30-35.

8. Cordeiro JM, Brugada R, Hong K, Antzelevitch C, and Dumaine R. Short QT syndrome mutation in HERG abolishes inactivation. Biophysical Journal 86, 134a. 2004. (Abstract)

9. Splawski I, Shen J, Timothy KW, Vincent GM, Lehmann MH, Keating MT. Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1. Genomics. 1998;51:86-97.

10. Bellocq C, van Ginneken A, Bezzina CR, Alders M, Escande D, Mannens MM, Baro I, Wilde AAM. A molecular and pathophysiological substrate for the short QT interval syndrome. Circulation. 2004;In press.

11. Schimpf R, Wolpert C, Bianchi F, Giustetto C, Gaita F, Bauersfeld U, Borggrefe M. Congenital short QT syndrome and implantable cardioverter defibrillator treatment: inherent risk for inappropriate shock delivery. J Cardiovasc Electrophysiol. 2003;14:1273-1277


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