Junctional Ectopic Tachycardia 

  • Author: M Silvana Horenstein, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 28, 2011
 

Background

Junctional ectopic tachycardia (JET) is characterized by rapid heart rate for a person's age that is driven by a focus with abnormal automaticity within or immediately adjacent to the atrioventricular (AV) junction of the cardiac conduction system (ie, AV node–His bundle complex). It does not have the features associated with reentrant tachycardia (eg, AV node reentry) because this form of tachycardia does not respond to a single extrastimulus, does not convert with programmed stimulation or cardioversion, and may or may not have ventriculoatrial (VA) dissociation; also, administration of adenosine results in VA dissociation without termination.

JET primarily occurs in 2 forms: idiopathic chronic junctional ectopic tachycardia, which is observed in the setting of a structurally normal heart, and transient postoperative junctional ectopic tachycardia occurs following repair of congenital heart disease.

In addition, nonparoxysmal junctional tachycardia is a related but rare pattern of arrhythmia that can be observed in the setting of digoxin toxicity.

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Pathophysiology

The pathophysiology of JET is unclear. Postoperative JET is associated with manipulation within the crux of the heart. It is believed to be secondary to trauma, infiltrative hemorrhage, or inflammation of the conduction tissue.[1]

As implied by the synonym junctional automatic tachycardia, the mechanism may be automaticity. Others have suggested that triggered activity is responsible for this disorder.[2]

The location of the responsible tissue is probably truly ectopic to the primary conduction pathway of the AV junction because JET has been successfully treated by the application of radiofrequency catheter lesions without the production of AV block. Intracardiac mapping shows a normal heart volume interval and VA dissociation, or VA association if VA conduction is present.

Junctional acceleration, albeit at a lesser rate than typical JET, is a recognized phenomenon during and following radiofrequency energy delivery for modification of slow pathway conduction in the therapy of AV node reentry.

Histamine, eosinophil cation protein, or other products of mast cell, eosinophil, or basophil degranulation that are liberated in response to cardiopulmonary bypass have been implicated in the genesis of transient postoperative JET. The relative levels of various cytokines may also play a role. Low magnesium levels have been noted in children who develop JET following cardiopulmonary bypass surgery.

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Epidemiology

Frequency

United States

Postoperative JET occurred in 5.6% of 594 patients who underwent cardiac surgery.[3] JET was seen more frequently with postoperative use of dopamine and in younger patients.

International

In one series, postoperative JET was identified in 7.5% of young patients undergoing Fontan procedures. Another recent series described JET in 10.2% of 874 pediatric patients undergoing cardiopulmonary bypass.[4]

Postoperative JET that required intervention was identified in 1.5% of infants undergoing the arterial switch procedure. It was also seen in 21.9% of patients who had undergone cardiac surgery for tetralogy of Fallot.[1]

Mortality/Morbidity

Although not a frequent type of arrhythmia, JET is one of the most serious and difficult-to-treat supraventricular tachycardias. Rare case reports have suggested that JET may be associated with progression to complete AV block. This does not appear to be the case in postoperative JET and has not been the author's experience in the rare cases of idiopathic JET.

  • Postoperative JET is usually transient and begins upon rewarming the patient. Its morbidity and mortality relates to the fact that it occurs at an extremely vulnerable period following cardiac surgery, when nodal inflammation and ischemia may be present and ventricular function is often diminished. The additional insults of poor ventricular filling because of tachycardia and the loss of AV sequential contraction are considered to significantly contribute to morbidity and mortality. However, if the JET rate is not too fast or is somewhat faster than the sinus node rate, it can be well tolerated until JET spontaneously subsides.
  • In a large series of patients with postoperative JET, dopamine use and an age less than 6 months were associated with the development of this tachycardia.[3] However, only 39% of patients required intervention.
  • Congenital JET occurs in neonates and infants as an incessant tachycardia that usually results in tachycardia-induced cardiomyopathy. Mortality in these patients has been reported to be as high as 34% and may occur secondary to congestive heart failure, sudden onset of ventricular fibrillation, and sudden evolution to paroxysmal complete AV block and as result of proarrhythmic effect of drug therapy and medical interventions.
  • In fetuses with JET (as well as those with ventricular tachycardia) third-degree AV block should be ruled out.[5]

Age

  • JET is one of the rarest forms of supraventricular tachycardia in infants. Congenital JET is presumed to be present from birth but may not be identified until months or years later.
  • Postoperative JET most commonly occurs in younger patients (it was found to occur more frequently in patients younger than 6 mo) but is also known to occur in teenagers and adults after cardiopulmonary bypass surgery.
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Contributor Information and Disclosures
Author

M Silvana Horenstein, MD  Assistant Professor, Department of Pediatrics, University of Texas Medical School at Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Robert Murray Hamilton, MD, MSc, FRCPC  Section Head, Electrophysiology, Senior Associate Scientist, Physiology and Experimental Medicine, Labatt Family Heart Centre; Professor, Department of Pediatrics, University of Toronto Faculty of Medicine

Robert Murray Hamilton, MD, MSc, FRCPC is a member of the following medical societies: American Heart Association, Canadian Cardiovascular Society, Canadian Medical Association, Canadian Medical Protective Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Ontario Medical Association, Pediatric Electrophysiology Society, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Charles I Berul, MD  Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Hugh D Allen, MD  Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References
  1. Dodge-Khatami A, Miller OI, Anderson RH, et al. Surgical substrates of postoperative junctional ectopic tachycardia in congenital heart defects. J Thorac Cardiovasc Surg. Apr 2002;123(4):624-30. [Medline].

  2. Liu CF, Ip JE, Lin AC, Lerman BB. Mechanistic Heterogeneity of Junctional Ectopic Tachycardia in Adults. Pacing Clin Electrophysiol. Sep 28 2011;[Medline].

  3. Hoffman TM, Bush DM, Wernovsky G, et al. Postoperative junctional ectopic tachycardia in children: incidence, risk factors, and treatment. Ann Thorac Surg. Nov 2002;74(5):1607-11. [Medline].

  4. Andreasen JB, Johnsen SP, Ravn HB. Junctional ectopic tachycardia after surgery for congenital heart disease in children. Intensive Care Med. May 2008;34(5):895-902. [Medline].

  5. Zhao H, Cuneo BF, Strasburger JF, Huhta JC, Gotteiner NL, Wakai RT. Electrophysiological characteristics of fetal atrioventricular block. J Am Coll Cardiol. Jan 1 2008;51(1):77-84. [Medline].

  6. Imamura M, Dossey AM, Garcia X, Shinkawa T, Jaquiss RD. Prophylactic amiodarone reduces junctional ectopic tachycardia after tetralogy of Fallot repair. J Thorac Cardiovasc Surg. Oct 27 2011;[Medline].

  7. [Best Evidence] Saul JP, Scott WA, Brown S, et al. Intravenous amiodarone for incessant tachyarrhythmias in children: a randomized, double-blind, antiarrhythmic drug trial. Circulation. Nov 29 2005;112(22):3470-7. [Medline].

  8. Guccione P, Di Carlo D, Papa M, et al. [Hypothermia treatment of junctional ectopic tachycardia after surgical repair of congenital heart defects]. G Ital Cardiol. May 1990;20(5):415-8. [Medline].

  9. Pfammatter JP, Paul T, Ziemer G, Kallfelz HC. Successful management of junctional tachycardia by hypothermia after cardiac operations in infants. Ann Thorac Surg. Sep 1995;60(3):556-60. [Medline].

  10. Walsh EP, Saul JP, Sholler GF, et al. Evaluation of a staged treatment protocol for rapid automatic junctional tachycardia after operation for congenital heart disease. J Am Coll Cardiol. Apr 1997;29(5):1046-53. [Medline].

  11. Wu MH, Lin JL, Chang YC. Catheter ablation of junctional ectopic tachycardia by guarded low dose radiofrequency energy application. Pacing Clin Electrophysiol. Nov 1996;19(11 Pt 1):1655-8. [Medline].

  12. Asou T, Kado H, Shiokawa Y, et al. Successful management of junctional tachycardia by hypothermia after a Fontan operation. Ann Thorac Surg. Aug 1996;62(2):583-5. [Medline].

  13. Balaji S, Sullivan I, Deanfield J, James I. Moderate hypothermia in the management of resistant automatic tachycardias in children. Br Heart J. Sep 1991;66(3):221-4. [Medline].

  14. Berul CI, Hill SL, Wang PJ, et al. Neonatal radiofrequency catheter ablation of junctional tachycardias. J Interv Card Electrophysiol. Mar 1998;2(1):91-100. [Medline].

  15. Case CL, Gillette PC. Automatic atrial and junctional tachycardias in the pediatric patient: strategies for diagnosis and management. Pacing Clin Electrophysiol. Jun 1993;16(6):1323-35. [Medline].

  16. Cilliers AM, du Plessis JP, Clur SA, et al. Junctional ectopic tachycardia in six paediatric patients. Heart. Oct 1997;78(4):413-5. [Medline].

  17. Coumel P, Fidelle JE, Attuel P, et al. [Congenital bundle-of-his focal tachycardias. Cooperative study of 7 cases]. Arch Mal Coeur Vaiss. Sep 1976;69(9):899-909. [Medline].

  18. Davis J, Scheinman MM, Ruder MA, et al. Ablation of cardiac tissues by an electrode catheter technique for treatment of ectopic supraventricular tachycardia in adults. Circulation. Nov 1986;74(5):1044-53. [Medline].

  19. Dorman BH, Sade RM, Burnette JS, et al. Magnesium supplementation in the prevention of arrhythmias in pediatric patients undergoing surgery for congenital heart defects. Am Heart J. Mar 2000;139(3):522-8. [Medline].

  20. Ehlert FA, Goldberger JJ, Deal BJ, et al. Successful radiofrequency energy ablation of automatic junctional tachycardia preserving normal atrioventricular nodal conduction. Pacing Clin Electrophysiol. Jan 1993;16(1 Pt 1):54-61. [Medline].

  21. Emmel M, Sreeram N, Brockmeier K. Catheter ablation of junctional ectopic tachycardia in children, with preservation of atrioventricular conduction. Z Kardiol. Apr 2005;94(4):280-6. [Medline].

  22. 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. Nov 1998;21(11 Pt 1):2132-5. [Medline].

  23. Gillette PC. Diagnosis and management of postoperative junctional ectopic tachycardia. Am Heart J. Jul 1989;118(1):192-4. [Medline].

  24. Gillette PC. Evolving concepts in the management of congenital junctional ectopic tachycardia. Circulation. May 1990;81(5):1713-4. [Medline].

  25. Hamdan M, Van Hare GF, Fisher W, et al. Selective catheter ablation of the tachycardia focus in patients with nonreentrant junctional tachycardia. Am J Cardiol. Dec 1 1996;78(11):1292-7. [Medline].

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

  27. Kalman JM, VanHare GF, Olgin JE, et al. Ablation of 'incisional' reentrant atrial tachycardia complicating surgery for congenital heart disease. Use of entrainment to define a critical isthmus of conduction. Circulation. Feb 1 1996;93(3):502-12. [Medline].

  28. Kohli V, Young ML, Perryman RA, Wolff GS. Paired ventricular pacing: an alternative therapy for postoperative junctional ectopic tachycardia in congenital heart disease. Pacing Clin Electrophysiol. May 1999;22(5):706-10. [Medline].

  29. Kuck KH, Kunze KP, Schluter M, Duckeck W. Encainide versus flecainide for chronic atrial and junctional ectopic tachycardia. Am J Cardiol. Dec 20 1988;62(19):37L-44L. [Medline].

  30. Lupoglazoff JM, Denjoy I, Luton D, et al. Prenatal diagnosis of a familial form of junctional ectopic tachycardia. Prenat Diagn. Aug 1999;19(8):767-70. [Medline].

  31. Mandapati R, Byrum CJ, Kavey RE, et al. Procainamide for rate control of postsurgical junctional tachycardia. Pediatr Cardiol. Mar-Apr 2000;21(2):123-8. [Medline].

  32. Maragnes P, Fournier A, Davignon A. Usefulness of oral sotalol for the treatment of junctional ectopic tachycardia. Int J Cardiol. May 1992;35(2):165-7. [Medline].

  33. Paul T, Reimer A, Janousek J, Kallfelz HC. Efficacy and safety of propafenone in congenital junctional ectopic tachycardia. J Am Coll Cardiol. Oct 1992;20(4):911-4. [Medline].

  34. Raja P, Hawker RE, Chaikitpinyo A, et al. Amiodarone management of junctional ectopic tachycardia after cardiac surgery in children. Br Heart J. Sep 1994;72(3):261-5. [Medline].

  35. Rodriguez EV, Mejia LM. Radiofrequency catheter ablation of junctional ectopic tachycardia in adults. Int J Cardiol. Jul 1 1999;70(1):75-81. [Medline].

  36. 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. Apr 1991;121(4 Pt 1):1237-9. [Medline].

  37. Rossi L, Piffer R, Turolla E, et al. Multifocal Purkinje-like tumor 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. Mar 1985;87(3):340-5. [Medline].

  38. Ruder MA, Davis JC, Eldar M, et al. Clinical and electrophysiologic characterization of automatic junctional tachycardia in adults. Circulation. May 1986;73(5):930-7. [Medline].

  39. Rychik J, Marchlinski FE, Sweeten TL, et al. Transcatheter radiofrequency ablation for congenital junctional ectopic tachycardia in infancy. Pediatr Cardiol. Nov-Dec 1997;18(6):447-50. [Medline].

  40. Sarubbi B, Musto B, Ducceschi V. Congenital junctional ectopic tachycardia in children and adolescents: a 20 year experience based study. Heart. Aug 2002;88(2):188-90. [Medline].

  41. Seghaye MC, Duchateau J, Grabitz RG, et al. Histamine liberation related to cardiopulmonary bypass in children: possible relation to transient postoperative arrhythmias. J Thorac Cardiovasc Surg. May 1996;111(5):971-81. [Medline].

  42. Sluysmans T, Moulin D, Jaumin P, et al. Ventricular paired pacing to control intractable junctional tachycardia following open heart surgery in a child. Intensive Care Med. 1989;15(3):203-5. [Medline].

  43. Till JA, Ho SY, Rowland E. Histopathological findings in three children with His bundle tachycardia occurring subsequent to cardiac surgery. Eur Heart J. May 1992;13(5):709-12. [Medline].

  44. Till JA, Rowland E. Atrial pacing as an adjunct to the management of post-surgical His bundle tachycardia. Br Heart J. Sep 1991;66(3):225-9. [Medline].

  45. Van Hare GF, Velvis H, Langberg JJ. Successful transcatheter ablation of congenital junctional ectopic tachycardia in a ten-month-old infant using radiofrequency energy. Pacing Clin Electrophysiol. Jun 1990;13(6):730-5. [Medline].

  46. Villain E, Vetter VL, Garcia JM, et al. Evolving concepts in the management of congenital junctional ectopic tachycardia. A multicenter study. Circulation. May 1990;81(5):1544-9. [Medline].

  47. Wilken M, Paul T, Ziemer G, Kallfelz HC. [Therapy of postoperative ectopic junctional tachycardia by surface hypothermia]. Z Kardiol. Jun 1993;82(6):376-9. [Medline].

  48. Wu JM, Young ML, Wu MH, et al. Junctional ectopic tachycardia in infancy: report of two cases. J Formos Med Assoc. May 1991;90(5):517-9. [Medline].

  49. Young ML, Mehta MB, Martinez RM, et al. Combined alpha-adrenergic blockade and radiofrequency ablation to treat junctional ectopic tachycardia successfully without atrioventricular block. Am J Cardiol. Apr 1 1993;71(10):883-5. [Medline].

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Lead II rhythm strip of a surface ECG from a patient with postoperative JET. Atrial activity (P) is marked with blue lines and ventricular depolarization (QRS) is marked in red. Note the narrow QRS complexes due to their origin at the AV junction. Also note the dissociation between atrial and ventricular depolarizations where some of the QRS complexes seem to "follow" the P waves. However, this is not possible because the PR intervals are exceedingly short to allow conduction. In addition, some of the P waves fall after the QRS.
 
 
 
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