Sick Sinus Syndrome

  • Ira Shetty
  • William A. ScottEmail author


Sick sinus syndrome and its possible etiologies are discussed, symptoms are outlined, and electrocardiographic features are summarized. Various presentations are detailed. Electrophysiologic procedures, although rarely indicated, are outlined and treatment is summarized.


Autoimmune disorders Congenital heart disease surgery Congenital sick sinus syndrome Postsurgical syndrome Sinoatrial conduction time Sinoatrial exit block Sinus arrest Sinus node recovery time Sinus pauses Tachy–brady syndrome 

Suggested Reading

  1. Anderson JB, Benson DW. Genetics of sick sinus syndrome. Card Electrophysiol Clin. 2010;2(4):499–507.PubMedCentralCrossRefPubMedGoogle Scholar
  2. Benson DW, Wang DW, Dyment M, Knilans TK. Congenital sick sinus syndrome caused by recessive mutations in the cardiac sodium channel gene (SCN5A). J Clin Invest. 2003;112(7):1019–28.PubMedCentralCrossRefPubMedGoogle Scholar
  3. Calzolari V, Angelini A, Basso C, Livi U, et al. Histologic findings in the conduction system after cardiac transplantation and correlation with electrocardiographic findings. Am J Cardiol. 1999;84:756–9.CrossRefPubMedGoogle Scholar
  4. Campbell RM, Dick M, Crowley DC, Rocchini AP, et al. Atrial pacing to estimate total sinoatrial conduction time in children. Pediatr Cardiol. 1988;9:85–9.CrossRefPubMedGoogle Scholar
  5. Crawford MH, Bernstein SJ, Deedwania PC, DiMarco JP, et al. ACC/AHA guidelines for ambulatory electrocardiography. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Revise the Guidelines for Ambulatory Electrocardiography). Developed in collaboration with the North American Society for Pacing and Electrophysiology. J Am Coll Cardiol. 1999;34:912–48.CrossRefPubMedGoogle Scholar
  6. Cuneo BF, Strasburger JF, Niksch A, Ovadia M, et al. An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease. J Matern Fetal Neonatal Med. 2009;22(3):233–8.PubMedCentralCrossRefPubMedGoogle Scholar
  7. Dilawar M, Bradley SM, Saul JP, Stroud MR, et al. Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit fontan procedures. Pediatr Cardiol. 2003;24:284–8.CrossRefPubMedGoogle Scholar
  8. Dobrzynski H, Boyett MR, Anderson RH. New insights into pacemaker activity: promoting understanding of sick sinus syndrome. Circulation. 2007;115(14):1921–32.CrossRefPubMedGoogle Scholar
  9. Elhendy A, Mahoney D, Khandheria B, Burger K, et al. Prognostic significance of impairment of heart rate response to exercise: impact of left ventricular function and myocardial ischemia. JACC. 2003;42(5):823–30.CrossRefPubMedGoogle Scholar
  10. Ferrer MI. The sick sinus syndrome. JAMA. 1968;206:206–45.CrossRefGoogle Scholar
  11. Garrigue S, Bordier P, Jais P, Shah D, et al. Benefit of atrial pacing in sleep apnea syndrome. N Engl J Med. 2002;346:404–12.CrossRefPubMedGoogle Scholar
  12. Gelatt M, Hamilton RM, McCrindle BW, Connoly M, et al. Arrhythmia and mortality after the mustard procedure: a 30-year single-center experience. J Am Coll Cardiol. 1997;29:194–201.CrossRefPubMedGoogle Scholar
  13. Gregoratos G, Abrams J, Epstein AE, Freedman RA, et al. ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines). J Cardiovasc Electrophysiol. 2002;13:1183–99.CrossRefPubMedGoogle Scholar
  14. Helbing WA, Hansen B, Ottenkamp J, Rohmer J, et al. Long-term results of atrial correction for transposition of the great arteries. Comparison of Mustard and Senning operations. J Thorac Cardiovasc Surg. 1994;108:363–72.PubMedGoogle Scholar
  15. Hilma H, Gudbjartsson DF, Sulem P, et al. A rare variant in MYH6 is associated with high risk of sick sinus syndrome. Nat Genet. 2011;43:316–20.CrossRefGoogle Scholar
  16. Janousek J, Paul T, Luhmer I, Wilken M, et al. Atrial baffle procedures for complete transposition of the great arteries: natural course of sinus node dysfunction and risk factors for dysrhythmias and sudden death. Z Kardiol. 1994;83:933–8.PubMedGoogle Scholar
  17. Kardelen F, Celiker A, Ozer S, Ozme S, et al. Sinus node dysfunction in children and adolescents: treatment by implantation of a permanent pacemaker in 26 patients. Turk J Pediatr. 2002;44:312–6.PubMedGoogle Scholar
  18. Kavey RE, Gaum WE, Byrum CJ, Smith FC, et al. Loss of sinus rhythm after total cavopulmonary connection. Circulation. 1995;92:II304–8.CrossRefPubMedGoogle Scholar
  19. Lauer M, Okin P, Larson M, Evans J, Levy D. Impaired heart rate response to graded exercise. Circulation. 1996;93:1520–6.CrossRefPubMedGoogle Scholar
  20. Lauer M, Francis G, Okin P, Pashkow F, et al. Impaired chronotropic response to exercise stress testing as a predictor of mortality. JAMA. 1999;281(6):524–9.CrossRefPubMedGoogle Scholar
  21. Lotze U, Maisch B. Humoral immune response to cardiac conducting tissue. Springer Semin Immunopathol. 1989;11:409–22.CrossRefPubMedGoogle Scholar
  22. Meijboom F, Szatmari A, Deckers JW, Utens EM, et al. Long-term follow-up (10 to 17 years) after Mustard repair for transposition of the great arteries. J Thorac Cardiovasc Surg. 1996;111:1158–68.CrossRefPubMedGoogle Scholar
  23. Menon A, Silverman ED, Gow RM, Hamilton RM. Chronotropic competence of the sinus node in congenital complete heart block. Am J Cardiol. 1998;82:1119–21. A9.CrossRefPubMedGoogle Scholar
  24. Oberhoffer R, von Bernuth G, Lang D, Gildein HP, Weismuller P. Sinus node dysfunction in children without heart defect. Z Kardiol. 1994;83:502–6.PubMedGoogle Scholar
  25. Ozer S, Schaffer M. Sinus node reentrant tachycardia in a neonate. Pacing Clin Electrophysiol. 2001;24:1038–40.CrossRefPubMedGoogle Scholar
  26. Paul T, Ziemer G, Luhmer L, Bertram H, et al. Early and late atrial dysrhythmias after modified Fontan operation. Pediatr Med Chir. 1998;20:9–11.PubMedGoogle Scholar
  27. Weese-Mayer DE, Silvestri JM, Menzies LJ, Morrow-Kenny AS, et al. Congenital central hypoventilation syndrome: diagnosis, management, and long-term outcome in thirty-two children. J Pediatr. 1992;120(3):381–7.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, New York 2015

Authors and Affiliations

  1. 1.Pediatric CardiologyAdvocate Children’s HospitalOak LawnUSA
  2. 2.Department of PediatricsUT Southwestern Medical CenterDallasUSA

Personalised recommendations