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The total absence of atrial automaticity in a child with sinus node dysfunction

Abstract

Background A routine sports evaluation identified constant alternation between a junctional and idioventricular rhythm in a 9-year-old child. During exercise testing, electrography demonstrated that the child was in junctional rhythm without any apparent P waves, and had a reduced increase in heart rate. Endocardial atrial pacing captured the atrium and demonstrated that atrioventricular conduction was normal, but the recovery time of the ectopic rhythm was very long. Three-dimensional electrophysiological mapping revealed 1:1 retrograde homogeneous conduction through the right atrium.

Investigations Electrocardiography, Holter monitoring, echocardiography, exercise stress testing, atrial pacing, three-dimensional electroanatomical mapping of the right atrium and genetic testing.

Diagnosis Sinus node dysfunction.

Management Pacemaker implantation was postponed until a later stage in the patient's development.

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Figure 1: Electrocardiograms demonstrating the total absence of atrial activity
Figure 2: Three-dimensional electroanatomical maps of the patient's right atrium, performed with the CARTO® XP Navigation System (Cordis Corporation, Miami Lakes, FL)
Figure 3: Sequential three-dimensional bipolar voltage maps of the patient's right atrium, demonstrating the progression of electrical activation (shown in red) in this chamber

References

  1. Calzolari A et al. (1994) Exercise testing on treadmill in normal children. Int J Sports Cardiol 3: 27–34

    Google Scholar 

  2. Drago F (2007) Successful radiofrequency ablation of atrial tachycardias in surgically repaired Ebstein anomaly using the CARTO XP system and the QuikStar catheter. J Cardiovasc Med 8: 459–462

    Article  Google Scholar 

  3. Martin AB and Kugler JD (1999) Sinus node dysfunction. In Clinical Pediatric Arrhythmias, edn 2, 51–62 (Eds Gillette PC and Garson A Jr) Philadelphia: WB Saunders

    Google Scholar 

  4. Friedly B (1997) Sino-atrial disease. In Paediatric Cardiac Arrhythmias, 174–199 (Eds Wren C and Campbell RW) New York: Oxford University Press

    Google Scholar 

  5. Mehta AV et al. (1995) Familial symptomatic sinus bradycardia: autosomal dominant inheritance. Pediatr Cardiol 16: 231–234

    Article  CAS  Google Scholar 

  6. Benson DW (2003) Congenital sick sinus syndrome caused by recessive mutations in the cardiac sodium channel gene (SCN5A). J Clin Invest 112: 1019–1028

    Article  CAS  Google Scholar 

  7. Veldkamp MW et al. (2003) Contribution of sodium channel mutations to bradycardia and sinus node dysfunction in LQT3 families. Circ Res 92: 976–983

    Article  CAS  Google Scholar 

  8. Marini M et al. (2004) Atrial standstill: a paralysis of cardiological relevance [Italian]. Ital Heart J Suppl 5: 681–686

    PubMed  Google Scholar 

  9. Schwartz PJ (2005) Management of long QT syndrome. Nat Clin Pract Cardiovasc Med 2: 346–351

    Article  Google Scholar 

  10. Schott JJ et al. (1995) Mapping of a gene for long QT syndrome to chromosome 4q25–27. Am J Hum Genet 57: 1114–1122

    CAS  PubMed  PubMed Central  Google Scholar 

  11. Mohler PJ et al. (2003) Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden death. Nature 421: 634–639

    Article  CAS  Google Scholar 

  12. Milanesi R et al. (2006) Familial sinus bradycardia associated with a mutation in the cardiac pacemaker channel. N Engl J Med 354: 151–157

    Article  CAS  Google Scholar 

  13. Gepstein L et al. (1997) A novel method for nonfluoroscopic catheter-based electroanatomical mapping of the heart: in vitro and in vivo accuracy results. Circulation 95: 1611–1622

    Article  CAS  Google Scholar 

  14. Walsh EP (1992) Electrocardiography and introduction to electrophysiology techniques. In Nadas' Pediatric Cardiology, 117–159 (Ed Fyler DC) Philadelphia: Hanley and Belfus

    Google Scholar 

  15. Gregoratos G et al. (2002) 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 13: 1183–1199

    Article  Google Scholar 

Download references

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Correspondence to Fabrizio Drago.

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Drago, F., Fazio, G., Di Pino, A. et al. The total absence of atrial automaticity in a child with sinus node dysfunction. Nat Rev Cardiol 4, 513–517 (2007). https://doi.org/10.1038/ncpcardio0966

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