eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Supraventricular Tachycardia, Junctional Ectopic Tachycardia

Author: M Silvana Horenstein, MD, Associate in Pediatric and Fetal Cardiac Diagnostic, Diagnostico Gineco-Obstetrico, PC; Associate Director, Legacy Department, Best Doctors, Inc
Coauthor(s): Robert Murray Hamilton, MD, MSc, FRCPC, Section Head, Electrophysiology, Director, High-Risk Hereditary Heart Conditions Clinic, Labatt Family Heart Centre; Professor, Department of Pediatrics, Associate Scientist, Physiology and Experimental Medicine, The Hospital for Sick Children and Research Institute, University of Toronto, Canada
Contributor Information and Disclosures

Updated: Jul 16, 2008

Introduction

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 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, which is a related but rare pattern of arrhythmia, can be observed in the setting of digoxin toxicity.

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.

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.

Frequency

United States

Postoperative JET occurred in 5.6% of 594 patients who underwent cardiac surgery.2  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.3

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.2 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.4

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.

Clinical

History

In general, postoperative junctional ectopic tachycardia (JET) occurs in the hospital with rapid hemodynamic instability, whereas congenital JET may have a more insidious course before producing signs of congestive heart failure.

  • Postoperative JET usually begins 6-72 hours following cardiopulmonary bypass surgery for repair of congenital heart lesions. It is usually identified during monitoring in the ICU. A fall in blood pressure and cardiac output usually occurs concomitantly.
  • The onset of congenital JET is often insidious. The clinical presentation of congenital JET may occur from birth to age 4 weeks. However, sporadic cases of intrauterine tachycardia have been reported in infants who presented with JET at birth. Prolonged moderate tachycardia may not be recognized until myocardial dysfunction and signs of congestive heart failure ensue. Heart rate variability is decreased; the heart rate is very regular except for occasional sinus capture beats.

Physical

Patients with congenital JET present with moderate tachycardia and signs of congestive heart failure. If VA dissociation has occurred, which is usually the case, cannon waves may be present in the jugular venous pulse, and the intensity of the first heart sound varies.

Causes

The speculative causes of postoperative JET are discussed in Pathophysiology. The one fairly uniform finding is a preceding cardiopulmonary bypass surgery.

  • The cause of congenital JET is unknown. A family history of JET has been reported in 50-55% patients.
  • Postoperative JET occurs more often after tetralogy of Fallot repair. It has been associated with resection of muscle bundles, increased traction through the right atrium for relief of right ventricular outflow tract obstruction, and with higher bypass temperatures.
  • The nonparoxysmal form of junctional tachycardia, which may be a triggered arrhythmia, is observed following digoxin overdose.

More on Supraventricular Tachycardia, Junctional Ectopic Tachycardia

Overview: Supraventricular Tachycardia, Junctional Ectopic Tachycardia
Differential Diagnoses & Workup: Supraventricular Tachycardia, Junctional Ectopic Tachycardia
Treatment & Medication: Supraventricular Tachycardia, Junctional Ectopic Tachycardia
Follow-up: Supraventricular Tachycardia, Junctional Ectopic Tachycardia
Multimedia: Supraventricular Tachycardia, Junctional Ectopic Tachycardia
References
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References

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Further Reading

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Keywords

supraventricular tachycardia, junctional ectopic tachycardia, JET, junctional automatic tachycardia, Coumel's tachycardia, idiopathic chronic junctional ectopic tachycardia, transient postoperative junctional ectopic tachycardia, congenital heart disease, junctional tachycardia, atrioventricular block, AV block, Fontan procedure, congestive heart failure, complete AV block, ventricular fibrillation, third-degree AV block, cardiopulmonary bypass, myocardial dysfunction, tetralogy of Fallot, right ventricular outflow tract obstruction

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Contributor Information and Disclosures

Author

M Silvana Horenstein, MD, Associate in Pediatric and Fetal Cardiac Diagnostic, Diagnostico Gineco-Obstetrico, PC; Associate Director, 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, Director, High-Risk Hereditary Heart Conditions Clinic, Labatt Family Heart Centre; Professor, Department of Pediatrics, Associate Scientist, Physiology and Experimental Medicine, The Hospital for Sick Children and Research Institute, University of Toronto, Canada
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.

Medical Editor

Charles Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Heart Rhythm Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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.

 
 
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