Congenital Junctional Ectopic Tachycardia: Presentation And Outcome

Sarubbi, Berardo and Vergara, Pasquale and D’Alto, Michele and Calabrò, Raffaele (2003) Congenital Junctional Ectopic Tachycardia: Presentation And Outcome. [Journal (Paginated)]

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Junctional ectopic tachycardia (JET) is a rare type of supraventricular arrhythmia. Even if its management has improved in recent years, it remains a great challenge for the cardiologist. Two are the possible clinical presentations of this arrhythmia: as a primary idiopathic disorder during infancy, configuring the so called “congenital” JET, or more often as a transient phenomenon immediately after surgery for congenital heart disease, giving rise to the “post-operative” variety. The congenital form, firstly described as a distinct entity by Coumel et al. in 19761, usually occurs in the first six months of life presenting as a persistent sustained form, lasting up to 90% of the time. Its clinical presentation may be dramatic, being associated in up to 60% of cases with cardiomegaly and/or heart failure. Congenital JET is hampered by high mortality. Secondary dilated cardiomyopathy, ventricular fibrillation and sudden cardiac death have also been reported 2,3.

Item Type:Journal (Paginated)
Keywords:Junctional Ectopic Tachycardia, Congenital
Subjects:JOURNALS > Indian Pacing and Electrophysiology Journal
ID Code:4278
Deposited By: Indian Pacing and Electrophysiology, Journal
Deposited On:24 Apr 2005
Last Modified:11 Mar 2011 08:55

References in Article

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1. Coumel P., Fidelle J.E., Attuel P., Brechenmacher C., Batisse A., Bretagne J., Clementy J., Gerard R., Grolleau R., Huault G., Mouy A., Nouvailles J., Kachaner J., Ribierre M., Toumieux M.C.: Tachycardies focales hisiennes congenitales: etude cooperative de sept cas. Arch. Mal. Coeur. 1976; 69:899-903.

2. Villain E., Vetter V.L., Garcia J.M., Herre J., Cifarelli A., Garson A. Jr.: Evolving concepts in the management of congenital junctional ectopic tachycardia. A multicenter study. Circulation 1990; 81:1544-1549.

3. Brechenmacher C., Coumel P, James TN. Intractable tachicardia in infancy. Circulation 1976; 53:377.

4. Villazon E., Fouron J.C., Fournier A., Proulx F.: Prenatal diagnosis of junctional ectopic tachycardia. Pediatr. Cardiol. 2001 22:160-162.

5. Benito Bartolome F., Jimenez Casso S.: Hydrops fetalis secondary to junctional ectopic tachycardia. Rev. Esp. Cardiol. 2000;53:1529-1530.

6. Sarubbi B, Musto B, Ducceschi V, D'Onofrio A, Cavallaro C, Vecchione F, Musto C, Calabro R. Congenital junctional ectopic tachycardia in children and adolescents: a 20 year experience based study. Heart. 2002; 88(2):188-90.

7. Garson A, Gillette PC. Junctional ectopic tachycardia in children: electrocardiography, electrophysiology and pharmacological response. Am J Cardiol 1979;44:298:302

8. Rossi AF, Kipel G, Golinko RJ, Griepp RB. Use of adenosine in postoperative junctional ectopic tachycardia with 1:1 retrograde atrial conduction. Am Heart J. 1991 Apr;121(4 Pt 1):1237-9.

9. Scheinman MM, Gonzalez RP, Cooper MW, Lesh MD, Lee RJ, Epstein LM. Clinical and electrophysiologic features and role of catheter ablation techniques in adult patients with automatic atrioventricular junctional tachycardia. Am J Cardiol 1994;74:565-572

10. Henneveld H, Hutter P, Bink-Boelkens M, Sreeram N. Junctional ectopic tachycardia evolving into complete heart block. Heart. 1998 Dec;80(6):627-8.

11. Brechenmacher C., Coumel P., James T.N.: Intractable tachycardia in infancy. Circulation 1976; 53: 377-381.

12. Till J.A., Ho S.Y., Rowland E.: Histopathological findings in three children with His bundle tachycardia occurring subsequent to cardiac surgery. Eur. Heart J. 1192; 13:709-712

13. Bharati S., Moskowitz W.B., Scheinman M., Estes N.A., Lev M.: Junctional tachycardias: anatomic substrate and its significance in ablative procedures. J. Am. Coll. Cardiol. 1991; 18:179-186.

14. Rossi L., Piffer R., Turolla E., Frigerio B., Coumel P., James T.N.: Multifocal Purkinje like tumour of the heart: occurrence with other anatomic abnormalities in the atrioventricular junction of an infant with junctional tachycardia, Lown-Ganong-Levine syndrome and sudden death. Chest 1985; 87:340-343.

15. Yap S.C., Hoomtje T., Sreeram N: Polymorphic ventricular tachycardia after use of intravenous amiodarone for postoperative junctional tachycardia. Int. J. Cardiol. 2000; 76:245-247.

16. Wren C.: Incessant tachycardias: Eur. Heart J. 1998; 19 suppl E: E32-E36.

17. Walsh E.P., Saul P., Sholler G.F., Triedman J.K., Jonas R.A., Mayer J.E., Wessel D.L.: Evaluation of a staged treatment protocol for rapid automatic junctional tachycardia after operation for congenital heart disease. J. Am. Coll. Cardiol 1997; 29:1046-1053

18. Paul T, Reimer A, Janousek J, Kallfelz HC. Efficacy and safety of Propafenone in congenital junctional ectopic tachycardia. J An Coll Cardiol 1992;20:911-4

19. Kuck KH, Kunze KP, Schlüter M, Duckeck W. Encainide versus flecainide for chronical atrial and junctional ectopic tachycardia.. Am J Cardiol 1988; 62:37L-44L

20. Maragnès P, Fournier A, Davignon A. Usefulness of oral sotalol for the treatment of junctional ectopic tachycardia. International Journal of Cardiology 1992;35:165-167

21. Dorman BH, Sade RM, Burnette JS, Wiles HB, Pinosky ML, Reeves ST, Bond BR, Spinale FG. Magnesium supplementation in the prevention of arrhythmias in pediatric patients undergoing surgery for congenital heart defects.Am Heart J. 2000 Mar;139(3):522-8

22. Gallagher JJ, Svenson RH, Kasell JH, et al Catheter technique for closet chest ablation of the atrioventricular conduction system. A therapeutic alternative for the treatment of supraventricular tachycardia. N Engl J Med 306:194,1982

23. Gillette PC, Garson A, Porter CJ, Ott D, McVey P, Zinner A, Blair H. Junctional automatic ectopic tachicardia: new proposed treatment by transcatheter His bundle ablation. Am Heart J 1983; 106:619-623

24. Fishberger SB, Rossi AF, Messina JJ, Saul JP. Successful radiofrequency catheter ablation of congenital junctional ectopic tachycardia with preservation of atrioventricular conduction in a 9-month-old infant. Pacing Clin Electrophysiol. 1998 Nov;21(11 Pt 1):2132-5

25. Walsh EP, Saul JP, Triedman JK Cardiac arrhythmias in children and young adults with congenital heart disease. Lippincott Williams and Wilkins 2001


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