La tunisie Medicale - 2021 ; Vol 99 ( n°06 ) : 614-622
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Introduction: Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease.
Aim: To describe the electrocardiographic features observed in Ebstein's disease
Methods:We conducted a retrospective descriptive study that enrolled 26 patients followed for ED.
Results:The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome.
Conclusion:Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.

Key - Words
  1. 1. AttenhoferJost CH, Connolly HM, Edwards WD, Hayes D, Warnes CA, Danielson GK. Ebstein’s anomaly: review of a multifaceted congenital cardiac condition. Swiss Med Wkly. 2005;135:269 –281. 2. Geerdink LM, Kapusta L. Dealing with Ebstein anomaly. Cardiol Young 2014 Jul; 24:191-200. 3. Pérez-Riera AR, Barbosa-Barros R, Daminello-Raimundo R, de Abreu LC, NikusK.Electro-vectorcardiographic and electrophysiological aspects of Ebstein’s anomaly. Ann Noninvasive Electrocardiol. 2019 May;24(3):e12590. 4. Kaushik, M. L., Sharma, M., &Kashyap, R. ’Himalayan’ p wave. Journal of the Association of Physicians of India, 2007:55, 856. 5. Ho, S. Y., Goltz, D., McCarthy, K., Cook, A. C., Connell, M. G., Smith, A., S. Antit & al. - Aspects électocardiographiques dans la maladie d’Ebstein 622 & Anderson, R. H. The atrioventricular junctions in Ebstein malformation. Heart,2000: 83(4), 444–449. 6. Hebe, J. Ebstein’s anomaly in adults. Arrhythmias: Diagnosis and therapeutic approach. Thoracic and Cardiovascular Surgeon, 2000: 48(4), 214–219. 7. Barbara, D. W., Edwards, W. D., Connolly, H. M., &Dearani, J. A. Surgical pathology of 104 tricuspid valves (2000–2005) with classic right‐sided Ebstein’s malformation. Cardiovascular Pathology, 2008;17(3), 166–171. 8. Houyel L. Anomalie d’Ebstein: aspects anatomiques. Arch Mal cœur, 2002 ;95 :513-7. 9. Cappato R, Schluter M, Weiß C, Antz M, Koschyk DH, Hofmann T, Kuck KH. Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein’s anomaly. Circulation 1996;94:376–383. 10. Shinohara T, Tsuchiya T, Takahashi N, Saikaw T, Yoshimatsu H. The characteristics of an abnormal electrogram on the atrialized right ventricle in a patient with Ebstein’s anomaly. Pacing ClinElectrophysiol 2009;32:269–272. 11. Assenza GE, Valente AM, Geva T, Graham D, Pluchinotta FR, Sanders SP et al. QRS duration and QRS fractionation on surface electrocardiogram are markers of right ventricular dysfunction and atrialization in patients with Ebstein anomaly. Eur Heart J. 2013 Jan;34(3):191-200. 12. Das MK, Maskoun W, Shen C, Michael MA, Suradi H, Desai M, Subbarao R, Bhakta D. Fragmented QRS on twelve-lead electrocardiogram predicts arrhythmic events in patients with ischemic and non-ischemic cardiomyopathy. Heart Rhythm 2010;7:74–80. 13. Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y. Right ventricular dysplasia: a report of 24 adult cases. Circulation 1982;65:384–398. 14. Kastor JA, Goldreyer BN, Josephson ME, Perloff JK, Scharf DL, Manchester JK, Shelburne JC, Hirshfeld JW. Electrophysiologic characteristics of Ebstein’s anomaly of the tricuspid valve. Circulation 1975;52:987–995. 15. Tede NH, Shivkumar K, Perloff JK, Middlekauff HR, Fishbein MC, Child JS, Laks H. Signal-averaged electrocardiograms in Ebstein’s anomaly. Am J Cardiol 2004;93: 432–436. 16. Acharya, P., Ang, J. R., &Gitler, B. Ebstein anomaly with QRS fragmentation on electrocardiogram. Journal of Investigative Medicine High Impact Case Reports, 2017 5(1), 2324709616688710. 17. Assenza GE, Volpe M, Cecchin F. Letter to the Editor, ‘Electrocardiographic features of arrhythmogenic right ventricular dysplasia’. Circulation 2010; 121:e405. 18. Ueshima, K., Nakamura, Y., Takeno, S., Miyake, T., &Takemura, T. AtriofascicularMahaim with Ebstein anomaly: A case report. Journal of Arrhythmia, 2017:33(5), 508–510. 19. Wei, W., Zhan, X., Xue, Y., Fang, X., Liao, H., Deng, H., Wu, S. Features of accessory pathways in adult Ebstein’s anomaly. Europace, 2014:16(11), 1619–1625. 20. Olson TM, Porter CJ. Electrocardiographic and electrophysiologic findings in Ebstein’s anomaly. Ann ThoracSurg, 2001;71;547-52. 21. Michael C. Viray, Philip C. Wiener, UyangaBatnyam, Lorena Rasquin, Gregg S. Pressman, SumeetMainigi. A Young Woman With Recurrent Palpitations: A Case of Ebstein Anomaly With Mahaim Fiber Tachycardia. CASE (Phila) 2019 Aug; 3(4): 145–148. 22. Mabo P. Anomalie d’Ebstein: troubles du rythme et leur traitement. Arch Mal Cœur, 2002 ; 95 :522-4. 23. Iturralde P, Guevara-Valdivia M, Rodriguez-Chavez L, Medeiros A and Coln. Radiofrequency ablation of multiple accessoiry pathways, 2002 ;4 :273-80. 24. Okishige K, Azegami K, Goseki Y, Ohira H, Sasano T, Yamashita K et al. Radiofrequency ablation of tachyarryhythmias in patients with Ebstein’s anomaly. International J Cardiol, 1997;60:171-80. 25. Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, Li Z, Hodge DO, Driscoll DJ. The outcomes of operations for 539 patients with Ebstein anomaly. J ThoracCardiovascSurg 2008;135:1120–1136. 26. Brown ML, Dearani JA, Danielson GK, Cetta F, Connolly HM, Warnes CA, et al. Functional status after operation for Ebstein Anomaly. J Am CollCardiol 2008;52:460–466. 27. Park SJ, Chung S, On YK, Kim JS, Yang JH, Jun TG et al. Fragmented QRS Complex in Adult Patients with Ebstein Anomaly and Its Association With Arrhythmic Risk and the Severity of the Anomaly. CircArrhythmElectrophysiol. 2013 Dec;6(6):1148-55
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