Cardiac Causes of Sudden Infant Death

Year 2014, Volume: 12 Issue: 1, 37 - 42, 01.06.2014

Fatih Aygün , Nilgün Köksal

Abstract

The mechanism and causes of sudden infant death syndrome SIDS is still remains its mystery. The pathophysiology of SIDS is not clear. Sleeping in prone position is thought to contribute to pathophysiology. Schwartz was the first physician suggesting that SIDS can be associated with heart and autonomic nervous system. Congenital long QT syndrome may trigger SIDS by causing ventricular tachycardia. Normal cardiac rhythm is regulated by ion channels and specific proteins, but genetical analyses clearly demonstrated that 5 ion channel genes were responsible for rare arrhythmias. The infections, fever and sleeping in prone position can increase the risk of long QT syndrome in babies having mutations in cardiac ion channels. In this review we tried to draw attention to SIDS as an important cause of death in childhood period and association of SIDS with long QT syndrome which is not necessarily noticed by physicians.

Keywords

Sudden infant death, cardiac causes, ion channel, arrhythmias, long QT

Ani Bebek Ölümünün Kardiyak Nedenleri

Abstract

Ani bebek ölümü sendromu ABÖS halen sebepleri ve mekanizmasıyla sırrını korumaktadır. ABÖS’e neden olan patofizyolojik mekanizma tam olarak anlaşılamamıştır. Yüz üstü pozisyonda uyumanın bu patofizyolojik olaylara katkısının olduğu düşünülmektedir. İlk defa Schwartz tarafından ABÖS ile kalp ve otonom sinir sistemi arasında ilişki olabileceği hipotezi öne sürülmüştür. Konjenital uzun QT sendromunun ventriküler taşiaritmi yaparak bazı ABÖS vakalarının sebebi olabileceği düşünülmektedir. Normal kardiyak ritimden birçok iyon kanalı ve diğer proteinlersorumlu iken, nadir bulunan aritmik bozukluklardan, genetik analizler sonucu, 5 iyon kanalı geni kesinlikle sorumlu tutulmaktadır. Özellikle kardiyak iyon kanallarında mutasyonu olan bebeklerde enfeksiyonlar, ateş, yüzüstü pozisyonda yatma gibi birçok faktörün uzun QT’ye eğilimi arttırdığı düşünülmektedir. Bu derlemede çocukluk çağında ölümlerin önemli bir nedenini oluşturan ve klinisyenlerin nispeten daha az önemsediği ABÖS’e dikkat çekilmek istenmiş ve ABÖS ile uzun QT ilişkisi değerlendirilmiştir

Keywords

Ani bebek ölümü, kardiyak neden, iyon kanalı, aritmi, uzun QT

References

• 1. Tester DJ, Ackerman MJ. Sudden infant death syndrome: how significant are the cardiac channelopathies? Cardiovascular Rese 2005;67:388-96.
• 2. Guyer B, Hoyert DL, Martin JA, Ventura SJ, MacDorman MF, Strobino DM. Annual summary of vital statistics-1998. Pediatrics 1999;104:1229-46.
• 3. Heron M, Sutton PD, Xu J, Ventura SJ, Strobino DM, Guyer B. Annual summary of vital statistics: 2007. Pediatrics 2010;125:4-15.
• 4. Harper RM, Kinney HC, Fleming PJ, Thach BT. Sleep influences on homeostatic functions: implications for sudden infant death syndrome. Respir Physiol 2000;119:123-32.
• 5. Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G, et al. Prevalence of the congenital long-QT syndrome. Circulation 2009;120:1761-7.
• 6. Schwartz PJ. Cardiac sympathetic innervation and the sudden infant death syndrome: A possible pathogenic link. Am J Med 1976;60:167-72.
• 7. Klaver EC, Versluijs GM, Wilders R. Cardiac ion channel mutations in the sudden infant death syndrome. Int J Cardiol 2011;152:162-70.
• 8. Schwartz PJ, Stramba-Badiale M, Segantini A, Austoni P, Bosi G, Giorgetti R, et al. Prolongation of the QT interval and the sudden infant death syndrome. N Engl J Med 1998;338:1709-14.
• 9. Schwartz PJ, Priori SG, Dumaine R, Napolitano C, Antzelevitch C, Stramba-Badiale M, et al. A molecular link between the sudden infant death syndrome and the long-QT syndrome. N Engl J Med 2000;343:262-7.
• 10. Wilde AA, Postema PG, Di Diego JM, Viskin S, Morita H, Fish JM, et al. he pathophysiological mechanism underlying Brugada syndrome: depolarization versus repolarization. J Mol Cell Cardiol 2010;49:543-53.
• 11. Albert CM, MacRae CA, Chasman DI, VanDenburgh M, Buring JE, Manson JE, et al. Common variants in cardiac ion channel genes are associated with sudden cardiac death. Circ Arrhythm Electrophysiol 2010;3:222-9.
• 12. Horigome H, Nagashima M, Sumitomo N, Yoshinaga M, Ushinohama H, Iwamoto M, et al. Clinical characteristics and genetic background of congenital long-QT syndrome diagnosed in fetal, neonatal, and infantile life: a nationwide questionnaire survey in Japan. Circ Arrhythm Electrophysiol 2010;3:10-7.
• 13. Splawski I, Shen J, Timothy KW, Lehmann MH, Priori S, Robinson JL, et al. Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation 2000;102:1178-85.
• 14. TesterDJ,WillML,HaglundCM,AckermanMJ.Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing. Heart Rhythm 2005;2:507-17.
• 15. Ackerman MJ, Anson BD, Tester DJ. Molecular autopsy of HERG defects in sudden infant death syndrome. J Am Coll Cardiol 2002;39:111-2.
• 16. Hartmann HA, Colom LV, Sutherland ML, Noebels JL. Selective localization of cardiac SCN5A sodium channels in limbic regions of rat brain. Nat Neurosci 1999;2:593-5.
• 17. Sarkozy A, Brugada P. Sudden cardiac death: what is inside our genes? Can J Cardiol 2005;21:1099-110.
• 18. Splawski I, Shen J, Timothy KW, Lehmann MH, Priori S, Robinson JL, et al. Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation 2000;102:1178-85.
• 19. Napolitano C, Priori SG, Schwartz PJ, Bloise R, Ronchetti E, Nastoli J, et al. Genetic testing in the long QT syndrome: development and validation of an efficient approach to genotyping in clinical practice. JAMA 2005;294:2975-80.
• 20. Zareba W, Moss AJ, Schwartz PJ, Vincent GM, Robinson JL, Priori SG, et al. Influence of genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. N Engl J Med 1998;339:960-5.
• 21. Ackerman MJ, Siu BL, Sturner WQ, Tester DJ, Valdivia CR, Makielski JC, et al. Postmortem molecular analysis of SCN5A defects in sudden infant death syndrome. JAMA 2001;286:2264-9.
• 22. Tester DJ, Ackerman MJ. Sudden infant death syndrome: how significant are the cardiac channelopathies? Cardiovasc Res 2005;67:388-96.
• 23. Tan BH, Pundi KN, Van Norstrand DW, Valdivia CR, Tester DJ, Medeiros-Domingo A, and et al. Sudden infant death syndrome-associated mutations in the sodium channel beta subunits. Heart Rhythm 2010;7:771-8.
• 24. Gregersen M, Rajs J, Laursen H, (eds). Pathologic criteria for the Nordic study of SIDS. In: Rognum TO, (ed). Sudden Infant Death Syndrome: NewTrends in the Nineties. Oslo, Norway: Scandinavian University Press; 1995;8.
• 25. Vege A, Rognum TO. Use of new Nordic criteria for classification of SIDS to re-evaluate diagnoses of sudden unexpected infant death in the Nordic countries. Acta Paediatr. 1997;86:391-6.
• 26. Mohamed U, Napolitano C, Priori SG. Molecular and electrophysiological bases of catecholaminergic polymorphic ventricular tachycardia. J Cardiovasc Electrophysiol 2007;18:791-7.
• 27. Tester DJ, Spoon DB, Valdivia HH, Makielski JC, Ackerman MJ. argeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner’s cases. Mayo Clin Proc 2004;79:1380-4.
• 28. Tester DJ, Dura M, Carturan E, Reiken S, Wronska A, Marks AR, et al. A mechanism for sudden infant death syndrome (SIDS): stress-induced leak via ryanodine receptors. Heart Rhythm 2007;4:733-9.
• 29. Wang DW, Desai RR, Crotti L, Arnestad M, Insolia R, Pedrazzini M, et al. Cardiac sodium channel dysfunction in sudden infant death syndrome. Circulation 2007;115:368-76.
• 30. Witchel HJ, Hancox JC. Familial and acquired long qt syndrome and the cardiac rapid delayed rectifier potassium current. Clin Exp Pharmacol Physiol 2000;27:753-66.
• 31. Amin AS, Herfst LJ, Delisle BP, Klemens CA, Rook MB, Bezzina CR, et al. Fever-induced QTc prolongation and ventricular arrhythmias in individuals with type 2 congenital long QT syndrome. J Clin Invest 2008;118:2552-61.