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01.08.2009 | Case Report

Short QT Syndrome in a Pediatric Patient

verfasst von: Juan Villafane, Ming Lon Young, Philippe Maury, Christian Wolpert, Olli Anttonen, Robert Hamilton, Prince J. Kannankeril, Peter S. Fischbach

Erschienen in: Pediatric Cardiology | Ausgabe 6/2009

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Abstract

Short QT syndrome (SQTS) is a recently described genetic syndrome characterized by abnormally brisk ventricular repolarization. Similar to long QT syndrome, SQTS might result in ventricular arrhythmias, syncope, and sudden death. The clinical diagnosis of SQTS is supported by the finding of an abnormally short QT interval on the resting electrocardiogram in combination with a suggestive clinical or family history. To date, few pediatric cases have been reported and the ideal therapy is unknown. We report a teenage boy who suffered a witnessed ventricular fibrillation arrest and was subsequently diagnosed with SQTS. Additional data from nine other pediatric patients diagnosed with SQTS are presented.

Anttonen O, Junttila MJ, Rissanen H et al (2007) Prevalence and prognostic significance of short QT interval in a middle-aged Finnish population. Circulation 116:714–720PubMedCrossRef

Antzelevitch C, Pollevick GD, Cordeiro JM et al (2007) Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-segment elevation, short QT intervals, and sudden cardiac death. Circulation 115:442–449PubMedCrossRef

Bellocq C, van Ginneken ACG, Bezzina CR et al (2004) Mutation in the KCNQ1 gene leading to the short QT-interval syndrome. Circulation 109:2394–2397PubMedCrossRef

Bjerregaard P, Gussak I (2005) Short QT syndrome. Ann Noninvas Electrocardiol 10:436–440CrossRef

Brugada R, Hong K, Dumaine R et al (2004) Sudden death associated with short-QT syndrome linked to mutations in HERG. Circulation 109:30–35PubMedCrossRef

Gaita F, Giustetto C, Bianchi F et al (2003) Short QT syndrome: a familial cause of sudden death. Circulation 108:965–970PubMedCrossRef

Gallagher MM, Magliano G, Yap et al (2006) Distribution and prognostic significance of QT intervals in the lowest half centile in 12, 012 apparently healthy persons. Am J Cardiol 98:933–935PubMedCrossRef

Giustetto C, Di Monte F, Wolpert C et al (2006) Short QT syndrome: clinical findings and diagnostic-therapeutic implications. Eur Heart J 27:2440–2447PubMedCrossRef

Gussak I, Brugada P, Brugada J et al (2000) Idiopathic short QT interval: a new clinical syndrome? Cardiology 94:99–102PubMedCrossRef

10.

Jervell A, Lange-Nielsen F (1957) Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death. Am Heart J 54:59–68PubMedCrossRef

11.

Moss A (1993) Measurement of the QT interval and the risk associated with QTc interval prolongation: a review. Am J Cardiol 72:23B–25BPubMedCrossRef

12.

Priori SG, Pandit SV, Rivolta I et al (2005) A novel form of short QT syndrome (SQT3) is caused by a mutation in the KCNJ2 gene. Circ Res 96:800–807PubMedCrossRef

13.

Romano C, Pongiglione R (1963) Aritmie cardiache rare dell’eta’pediatrica II Accessi sincopali per fibrillazione ventricolare parossistica. Clin Peditr (Bologna) 45:656–683

14.

Schimpf R, Bauersfeld U, Gaita F, Wolpert C (2005) Short QT syndrome: successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis. Heart Rhythm 2:416–417PubMedCrossRef

15.

Schimpf R, Veltmann C, Giustetto C, et al. (2007) In vivo effects of mutant HERG K(+) channel inhibition by disopyramide in patients with a Short QT-1 syndrome: a pilot study. J Cardiovasc Electrophysiol 18: 1157–1160CrossRef

16.

Schimpf R, Wolpert C, Bianchi F et al (2003) Congenital short QT syndrome and implantable cardioverter defibrillator treatment: inherent risk for inappropriate shock delivery. J Cardiovasc Electrophysiol 14:1273–1277PubMedCrossRef

17.

Schimpf R, Wolpert C, Gaita F, Giustetto C, Borggrefe M (2005) Short QT syndrome. Cardiovasc Res 67:357–366PubMedCrossRef

18.

Ward OC (1964) A new familial cardiac syndrome in children. J Irish Med Assoc 54:103–106

19.

Wolpert C, Schimpf R, Giustetto C et al (2005) Further insights into the effect of quinidine in short QT syndrome caused by a mutation in HERG. J Cardiovasc Electrophysiol 16:54–58PubMedCrossRef

20.

Wolpert C, Schimpf R, Veltmann C et al (2005) Clinical characteristics and treatment of short QT syndrome. Expert Rev Cardiovasc Therapy 3:611–617CrossRef

Titel: Short QT Syndrome in a Pediatric Patient
verfasst von: Juan Villafane
Ming Lon Young
Philippe Maury
Christian Wolpert
Olli Anttonen
Robert Hamilton
Prince J. Kannankeril
Peter S. Fischbach
Publikationsdatum: 01.08.2009
Verlag: Springer-Verlag
Erschienen in: Pediatric Cardiology / Ausgabe 6/2009
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-009-9439-9

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