Short QT Syndrome

Classification according to ICD-10
I45.8	Other specified cardiac conduction disorders
ICD-10 online (WHO version 2019)

EKG with QT interval in blue

The Short QT syndrome ( SQTS ) is a congenital heart disease. It is characterized by a short QT interval (less than 370 ms) in the ECG , which hardly changes with the heart rate, and a high risk of sudden cardiac death from ventricular tachycardia or ventricular fibrillation . Another abnormality in the ECG are high, pointed T waves, similar to those in hyperkalemia .

The disease was first described in 2000. In molecular genetic studies, mutations were found in genes for certain potassium and calcium channels in the heart muscle cells.

Short QT syndrome appears to be inherited in an autosomal dominant manner. The prevalence is unknown. With fewer than 200 published cases worldwide (as of June 2017), it seems extremely rare.

literature

• Barry J. Maron et al .: AHA Scientific Statement: Contemporary Definitions and Classification of the Cardiomyopathies. Circulation. 2006; 113: 1807-1816.

Web links

• www.shortqtsyndrome.org

See also

• Long QT Syndrome

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1. ^ The Short QT Syndrome: Proposed Diagnostic Criteria . In: Journal of the American College of Cardiology . tape 57 , no. 7 , February 15, 2011, ISSN  0735-1097 , p. 802-812 , doi : 10.1016 / j.jacc.2010.09.048 ( sciencedirect.com [accessed February 5, 2018]).
2. ↑ Ihor Gussak, Pedro Brugada, Josep Brugada, R. Scott Wright, Stephen L. Kopecky: Idiopathic Short QT Interval: A New Clinical Syndrome? In: Cardiology . tape 94 , no. 2 , 2000, ISSN  0008-6312 , p. 99-102 , doi : 10.1159 / 000047299 ( karger.com [accessed February 5, 2018]).
3. ↑ Charles Antzelevitch, Guido D. Pollevick, Jonathan M. Cordeiro, Oscar Casis, Michael C. Sanguinetti: 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 . In: Circulation . tape 115 , no. 4 , January 30, 2007, ISSN  0009-7322 , p. 442-449 , doi : 10.1161 / CIRCULATIONAHA.106.668392 , PMID 17224476 ( ahajournals.org [accessed February 5, 2018]).
4. ↑ Chinmay Patel, Gan-Xin Yan, Charles Antzelevitch: Short QT Syndrome: From Bench to Bedside . In: Circulation: Arrhythmia and Electrophysiology . tape 3 , no. 4 , August 1, 2010, ISSN  1941-3149 , p. 401-408 , doi : 10.1161 / CIRCEP.109.921056 , PMID 20716721 ( ahajournals.org [accessed February 5, 2018]).
5. ↑ Andrea Mazzanti, Katherine Underwood, Dmitriy Nevelev, Shanna Kofman, Silvia G. Priori: The new kids on the block of arrhythmogenic disorders: Short QT syndrome and early repolarization . In: Journal of Cardiovascular Electrophysiology . tape 28 , no. 10 , October 1, 2017, ISSN  1540-8167 , p. 1226-1236 , doi : 10.1111 / jce.13265 ( wiley.com [accessed February 7, 2018]).

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