4. Secondary Prevention Of Sudden Cardiac Death In Patient With Long Qt Syndrome

Authors

  • A.K. Baimbetov A.N. Syzganov’s National Scientific Center of Surgery, Almaty, Kazakhstan
  • K.A. Bizhanov A.N. Syzganov’s National Scientific Center of Surgery, Almaty, Kazakhstan
  • B.A. Bairamov A.N. Syzganov’s National Scientific Center of Surgery, Almaty, Kazakhstan
  • I.Y. Sagatov A.N. Syzganov’s National Scientific Center of Surgery, Almaty, Kazakhstan

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Abstract

Congenital long QT syndrome (LQTS) is an abnormally prolonged repolarization of the stomach due to hereditary defects in the sodium and potassium channels of the heart, which predispose patients with syn-cope, gastrointestinal arrhythmias, and sudden cardiac death. Early diagnosis and prophylactic treatment play an important role in preventing sudden cardiac death in patients with congenital LQTS. The diagnostic criteria for congenital LQTS are based on specific electrocardiographic data, clinical data, and adrenaline test. results. Recently, a genotype-specific electrocardiographic pattern of congenital LQTS has also been described. Recent studies suggest the feasibility of genotype-specific treatment for LQTS, and soon, muta-tion-specific treatment is likely to become a new approach to this potentially deadly syndrome. We present a case report that is verified by electrocardiographic and clinical diagnostic criteria, indicat-ing LQTS. In this case, the cardioverter-defibrillator is implanted to the patient for the secondary preven-tion of sudden cardiac death. The patient experiences attacks of sudden palpitations with fainting, and the implanted defibrillator leads therapy from life-threatening ventricular tachycardia.

Keywords

sudden cardiac death

References

  1. Mullally J., Goldenberg I., Moss A. et al. Risk of life-threatening cardiac events among patients with long QT syndrome and multiple mutations. Heart Rhythm 2013; 10 (3): 378-382
  2. Surawicz B., Knilans T. Chou’s Electrocardiography in clinical practice. 6th. ed. Saunders Elsevier. 2008
  3. Schwartz P.J., Crotti L., Insolia R. Long-QT syn-drome: from genetics to management. Circulation 2012; 5 (4):868–877
  4. Moss AJ, Zareba W, Kaufman ES. Increased risk of arrhythmic events in long QT syndrome with muta-tions in the pore region of the human ether-a-a-go-go-related gene potassium channel. Circulation. 2002; 105:794–9
  5. Schwartz P.J., Stramba-Badiale M., Crotti L. et al. Prevalence of the Congenital Long QT Syndrome. Circulation 2009; 120 (18):1761–1767
  6. Priori S.G., Schwartz P.J., Napolitano C. et al. Risk
  7. Mitcheson JS, Chen J, Lin M, et al. A structural basis for drug-induced long-QT syndrome. Proc Natl Acad Sci USA. 2000; 97: 12329–1233
  8. Schwartz P., Priori S., Spazzolini C., et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhyth-mias. Circulation. 2001; 103:89–95
  9. Priori SG, Napolitano C, Schwartz PJ. Low pen-etrance in the long QT syndrome: clinical impact. Circulation. 1999; 99:529–33
  10. Moss A., Zareba W., Hall W. et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation. 2000; 101:616–23
  11. Shimizu W, Antzelevitch C. Differential response to beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome. J Am Coll Cardiol. 2000; 35:778–86
  12. Barsheshet A., Goldenberg I., O-Uchi J. et al. Mu-tations in cytoplasmic loops of the KCNQ1 channel and the risk of life-threatening events: implications for mutation-specific response to beta-blocker ther-apy in type 1 long-QT syndrome. Circulation 2012; 125:1988– 1996
  13. Viskin S. Long QT syndromes and torsades de pointes. Lancet. 1999; 354: 1625–33
  14. Schwarttz P.J., Spazzolini C., Priori S.G. et al. Who Are the Long QT Syndrome Patients Who Receive an Implantable Cardioverter Defibrillator and What Happens to Them? Data from the European Long-QT Syndrome Implantable Cardioverter-Defibril-lator (LQTS ICD) Registry. Circulation 2010; 122: 1272–1282

Published

2020-07-01

Issue

No. 2 (2020)

Section

Статьи