Short QT interval syndrome
Abstract
Every year, sudden cardiac death (SCD) takes lives of 400 000 American citizens, and in 10-20% ofthe cases, fatal outcome is explained by hereditary pathology. Short QT interval syndrome remains rarely diagnosed, and virtually unknown, as it was described just recently. Its clinical course is characterized by syncope or fainting episodes, and SCD in patients with corrected QT interval <320 ms. In patients with short QT syndrome, syncope can be caused by atrial fibrillation (AF) paroxysms or ventricular arrhythmias. AF episodes are typically the first manifestation ofthe disease, being registered more often in children and adolescents; some cases in new-born babies have been described. According to genetic examination results, short QT syndrome is a hereditary pathology, explained by various mutations of potassium channel gene. Genetic polymorphism in short QT syndrome manifests during antiarrhythmic treatment. At present, the single effective therapeutic method for such patients is cardioverter-defibrillator implantation.
About the Authors
I. G. Fomina
I.M. Sechenov Moscow Medical Academy. Moscow
Russian Federation
Russian Federation
A. I. Tarzimanova
I.M. Sechenov Moscow Medical Academy. Moscow
Russian Federation
Russian Federation
A. V. Vetluzhsky
I.M. Sechenov Moscow Medical Academy. Moscow
Russian Federation
Russian Federation
References
1. Киселева И.И., Макаров Л.М., Чупрова С.Н. Укорочение интервала QT в семьях с отягощенным анамнезом по внезапной смерти в молодом возрасте. Вест аритм 2004; 35: 59.
2. Antzelevich С. Molecular genetics of arrhythmias and cardiovascular conditions associated with arrhythmias. J Cardiovas Electrophysiol 2003; 14: 1259-72.
3. Brugada R., Hong K., Dumaine R., et al. Sudden death associated with short-QT syndrome linked to mutations in HERG. Circulation 2004; 109: rl51-6.
4. Barhanin J., Lesage F., Guillemare E., et al. KvLQTl and lsK (minK) proteins associate to form the IKs cardiac potassium current. Nature 1996; 384: 78-80.
5. Chen Y.H., Xu S.J., Bendahhou S., et al. KCNQ1 gain-of-function mutation in familial atrial fibrillation. Science 2003; 299: 251-4.
6. Chen Q., Kirsch G.E., Zhang D., et al. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature 1998; 19(32): 293-6.
7. Cheng T.O. Digitalis administration: an underappreciated but common cause of short QT interval. Cumulation 2004; 109: 152-5.
8. Gaita F., Giustetto C., Bianchi F., et al. Short QT syndrome: a familial cause of sudden death. Circulation 2003; 108: 965-70.
9. Gussak I., Brugada P., Brugada J. Idiopathic short QT interval: a new clinical syndrome? Cardiology 2000; 94: 99-102.
10. Keating M., Atkinson D., Dunn C., et al. Linkage of a cardiac arrythmia, the long QT syndrome, and the Harvey ras -1 gene. Science 1991; 252: 704-6.
11. Liu X.K., Katchman A., Whitfield B.H., et al. In vivo androgen treatment shortens the QT interval and increases the densitiens of inward and delayed rectifier potassium currents in orchiec-tomised male rabbits. Cardiovasc Res 2003; 57: 28-36.
12. Priebe L., Beuckelmann D.J. Simulation study of cellular electric properties in heart failure. Circ Res 1998; 82: 1206-23.
13. Seebohm G., Scherer C.R., Busch A.E., et al. Identification of specific pore residues mediating KCNQ1 inactivation. A novel mechanism for long QT syndrome. J Biol Chem 2001; 276: 13600-5.
14. Schimpf R., Wolpert C., Gaita F., et al. Short QT syndrome. Cardiovasc Res 2005; 103: 13-23.
15. Spooner P.M., Albert C., Benjamin E.J., et al. Sudden cardiac death, genes, and arrhythmogenesis. Circulation 2001; 103: 2361-4.
16. Tristani-Firouzi M., Sanguinetti M.C. Voltage-dependent inactivation of the human K+ channel KvLQT 1 is eliminated by association with minimal K+ channel (minK) subunits. J Physiol 1998;510:37-45.
17. Wever E.F., Robles de Medina E.O. Sudden death in patients without structural heart disease. JACC 2004; 43: 1137-44.
18. Wolpert C., Schimpf R., Giustetto C., et al. Further insights into the effect of quinidin in short QT syndrome caused by a mutation in HERG. J Cardiovasc Electrophysiol 2005; 16: 54-8.
Review
For citations:
Fomina I.G., Tarzimanova A.I., Vetluzhsky A.V. Short QT interval syndrome. Cardiovascular Therapy and Prevention. 2006;5(7):83-85. (In Russ.)
Views:
1156
Email this article 
