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Author: Sean C Beinart, MD, Electrophysiology Fellow, Department of Internal Medicine, Division of Cardiology, Emory University School of Medicine

Sean C Beinart is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Physicians, and American Medical Association

Coauthor(s): Jonathan Langberg, MD; Spencer Rosero, MD, Assistant Professor, Department of Medicine, University of Rochester School of Medicine; Wojciech Zareba, MD, PhD, FACC, Associate Director of Heart Research, Associate Professor, Department of Medicine, Division of Cardiology, University of Rochester Medical Center; David Huang, MD, Assistant Professor, Department of Medicine, Cardiology Unit, University of Rochester School of Medicine and Dentistry

Editors: Alan D Forker, MD, Professor of Medicine, Program Director of Cardiovascular Fellowship, MidAmerica Heart Institute, University of Missouri at Kansas City School of Medicine; Director, Outpatient Lipid Diabetes Research Center, MidAmerica Heart Institute of Saint Luke's Hospital; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Steven J Compton, MD, FACC, FACP, Director of Cardiac Electrophysiology, Alaska Heart Institute, Providence and Alaska Regional Hospitals; Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital; Leonard Ganz, MD, Associate Professor of Medicine, Temple University School of Medicine; Cardiac Electrophysiologist, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Cent, West Penn Hospital

Author and Editor Disclosure

Synonyms and related keywords: junctional rhythm, accelerated junctional tachycardia, junctional bradycardia, tachycardia, atrioventricular block, AV block, bradycardia, sick sinus syndrome, sinus node dysfunction, heart block, complete heart block, digitalis toxicity, digoxin toxicity

Background

Cardiac rhythms arising from the atrioventricular (AV) junction occur as an automatic tachycardia or as an escape mechanism during periods of significant bradycardia with rates slower than the intrinsic junctional pacemaker.

The AV node (AVN) has intrinsic automaticity that allows it to initiate and depolarize the myocardium during periods of significant sinus bradycardia or complete heart block. This escape mechanism, with a rate of 40-60 beats per minute, produces a narrow QRS complex because the ventricle is depolarized using the normal conduction pathway. An accelerated junctional rhythm (rate >60) is a narrow complex rhythm that often supersedes a clinically bradycardic sinus node rate (see Images 1-2). The QRS complexes are uniform in shape, and evidence of retrograde P wave activation may or may not be present.

Less commonly, the AV junction develops abnormal automaticity and exceeds the sinus node rate at a time when the sinus rate would be normal (see Image 3). These junctional tachycardias are most often observed in the setting of digitalis toxicity, recent cardiac surgery, acute myocardial infarction, or isoproterenol infusion.

Pathophysiology

The junctional rhythm initiates within the AV nodal tissue. Accelerated junctional rhythm is a result of enhanced automaticity of the AVN that supersedes the sinus node rate. During this rhythm, the AVN is firing faster than the sinus node, resulting in a regular narrow complex rhythm. These rhythms may demonstrate retrograde P waves on ECG findings, and the rates can vary from 40-60 beats per minute.

Changes in autonomic tone or the presence of sinus node disease that is causing an inappropriate slowing of the sinus node may exacerbate this rhythm. Young healthy individuals, especially those with increased vagal tone during sleep, are often noted to have periods of junctional rhythm that is completely benign, not requiring any intervention.

Rarely, the AVN develops enhanced automaticity and overtakes a "normal" sinus node. This occasionally is observed in digitalis toxicity, following cardiac surgery (typically valve replacement), during acute myocardial infarction, or during isoproterenol infusion.

Frequency

United States

Junctional rhythms are common in patients with sick sinus syndrome or in patients who have significant bradycardia that allows the AV nodal region to determine the heart rate.

Mortality/Morbidity

  • The heart rate during a junctional rhythm often determines whether the patient has symptoms.
  • Presence of AV disassociation can lead to symptoms in patients because of atrial conduction and subsequent contraction when the tricuspid valve is closed.
  • Periods of junctional rhythm are not necessarily associated with an increase in mortality. If an obvious cause is present, such as complete heart block or sick sinus syndrome, then the morbidity or mortality is directly related to that and not to the junctional rhythm mechanism, which is serving as a "backup" during the periods of bradycardia. Accelerated junctional rhythms may be a sign of digitalis toxicity.

Sex

Junctional escape rhythms, which are common in younger and/or athletic individuals during periods of increased vagal tone (eg, sleep), occur equally in males and females.

Age

  • This rhythm may occur in persons of any age.
  • Junctional rhythms during sleep are common in children and in athletic adults.



History

Junctional rhythms may be accompanied by symptoms or may be entirely asymptomatic.

  • Palpitations, fatigue, or poor exercise tolerance: These may occur during a period of junctional rhythm in patients who are abnormally bradycardic for their level of activity.
  • Dyspnea: Sudden onset of symptoms and sudden termination of symptoms may occur, especially in the setting of complete heart block.
  • Presyncope (near syncope): The underlying cause of the junctional rhythm is the most significant predictor of symptoms (ie, complete heart block). Presyncope may be present during an acute decrease in heart rate.

Physical

  • A predominant junctional rhythm may be associated with structural heart disease, sick sinus syndrome, or both, during which the junctional escape rhythm supersedes the sinus rate and provides a safety mechanism.
  • During a predominant junctional rhythm, the pulse usually is regular and the heart rate may be within reference range. Frequently, the junctional rhythm is 40-60 beats per minute.
  • Prominent jugular venous pulsations (ie, cannon a waves) may be present due to the right atrium contracting with a closed tricuspid valve.

Causes

  • Sick sinus syndrome (including drug-induced)
  • Digoxin toxicity
  • Ischemia of the AVN, especially with acute inferior infarction
  • Acutely after cardiac surgery, especially in children within 4 days after surgery for congenital cardiac defects
  • Acute inflammatory processes (eg, acute rheumatic fever), which may involve the conduction system
  • Diphtheria
  • Other drugs (eg, beta-blockers, calcium blockers, most antiarrhythmic agents) that cause sinus bradycardia
  • Metabolic states with increased adrenergic tone
  • Isoproterenol infusion



Atrioventricular Block
Atrioventricular Dissociation
Atrioventricular Nodal Reentry Tachycardia (AVNRT)
Digitalis Toxicity
Sinus Node Dysfunction


Lab Studies

  • Evaluation of serum electrolyte levels is generally indicated for patients with comorbidities that may predispose them to accelerated junctional rhythms because of intrinsic bradycardia or AV block.
    • Check digoxin level in patients on digoxin and obtain a 12-lead ECG.
    • The standard approach includes an electrolyte evaluation, a 12-lead ECG, a detailed history, and a physical examination.
  • Junctional rhythms are common during sleep in younger patients.

Other Tests

  • The 12-lead ECG is essential to making the correct diagnosis of any junctional rhythm (Images 1-3). Telemetry strips demonstrating the onset and termination pattern of the unknown narrow complex rhythm often provide clues regarding the diagnosis. The 12-lead ECG findings also help identify patients with underlying structural heart disease or conduction abnormalities.
    • Determine if the rhythm is regular or irregular, if it is narrow or wide, if P waves are present, if the P waves are from the right atrium (upright in I and II, negative in aVL), how the rhythm was initiated and how it terminated, and, finally, the clinical setting in which the rhythm occurred.
    • A junctional rhythm usually presents with rates from 40-60 beats per minute.
    • Frequently, retrograde P-wave conduction may be notable as a negative P wave in leads I and II and positive in aVL. Because the arrhythmia is originating from within the nodal tissue, near simultaneous activation of the atrium and ventricle occurs. Thus, the P-wave or atrial activation may be hidden within the QRS complex and may not be noticeable on surface ECG findings.
    • Explore the differential diagnosis of a regular narrow complex tachycardia when interpreting the ECG findings. An unusually slow presentation of a tachycardia also may mimic a junctional escape rhythm. These include AV reentry via an accessory pathway, atrial tachycardia, and AV nodal reentrant tachycardia.
    • The differential of a junctional rhythm at lower rates includes a normal response to increased vagal tone during sleep and sinus bradycardia, inappropriate sinus bradycardia, and underlying AV block.
    • ECG findings may help rule out structural or congenital heart disease in patients with evidence of multiple forms of supraventricular arrhythmias.
  • A cardiac event monitor is indispensable for patients who are difficult to diagnose, such as those with transient symptoms of palpitations or minimal documentation of an abnormal rhythm. Patients may carry the event monitor for an indefinite period (usually 30 d) and press a button to record a rhythm strip during symptoms. The onset and termination of the rhythm is documented and may help guide therapy and may help exclude more potentially lethal arrhythmias, such as ventricular tachycardia, as a cause of the symptoms.
  • In patients with an accelerated junctional rhythm after cardiac surgery, documentation of AV conduction is imperative. The accelerated junctional rhythm may be a manifestation of inflammation and/or damage to the AV junction; once the accelerated rhythm resolves, AV block may be present. If atrial epicardial wires are present, pacing the atrium at a more rapid rate allows verification of AV conduction.
  • If the diagnosis is still not certain, an electrophysiologic study (EPS) or invasive electrophysiologic evaluation can be performed.

Procedures

  • An EPS should reveal a His bundle depolarization preceding every QRS complex. The His-ventricular interval should be normal unless conduction system disease is present. AV and VA conductions often fluctuate.



Medical Care

The decision to treat a junctional rhythm depends on the underlying cause and the stability of the patient.

  • No pharmacologic therapy is needed for asymptomatic, otherwise healthy individuals with junctional rhythms that result from increased vagal tone.
  • In patients with complete AV block, high-grade AV block, or symptomatic sick sinus syndrome (ie, sinus node dysfunction), a permanent pacemaker may be needed. The junctional rhythm serves as an escape mechanism to maintain the heart rate during periods of bradycardia or asystole and should not be suppressed.
  • Emergency department care can include evaluation of the 12-lead ECG findings, airway protection and oxygenation, and blood pressure support, depending on the cause of the rhythm.
  • If the junctional rhythm is due to digitalis toxicity, then atropine, digoxin immune Fab (Digibind), or both may be necessary. In refractory cases of symptomatic digitalis toxicity that results in junctional tachycardia and causes severe symptoms, then intravenous phenytoin can be used. This should be administered in a monitored setting because of possible hypotension or the need for a pacemaker after resolution of the ectopic junctional rhythm.

Surgical Care

  • If junctional rhythm is due to symptomatic sick sinus syndrome, permanent pacemaker implantation is indicated.
  • If ectopic junctional tachycardia, which usually occurs in the pediatric population, is incessant and symptomatic, then radiofrequency ablation via a percutaneous approach is indicated.

Consultations

  • Symptomatic cases may benefit from a consultation with a cardiologist and/or an electrophysiologist to better define the etiology and approach to prevention.



The goals of pharmacotherapy are to reduce morbidity and prevent complications.

Drug Category: Anticholinergics

Agents used to accelerate heart rate if symptomatic bradycardia is present.

Drug NameAtropine
DescriptionUsed to increase heart rate through vagolytic effects, causing an increase in cardiac output.
Adult Dose0.5-1 mg IV or ET q3-5min; not to exceed to 3 mg total (0.04 mg/kg)
Pediatric Dose0.02 mg/kg/dose IV; use a minimum of 0.1 mg
ContraindicationsDocumented hypersensitivity; thyrotoxicosis; narrow-angle glaucoma; tachycardia
InteractionsCoadministration with other anticholinergics have additive effects; pharmacologic effects of atenolol and digoxin may increase; antipsychotic effects of phenothiazines may decrease; tricyclic antidepressants with anticholinergic activity may increase effects
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsCaution in Down syndrome or children with brain damage to prevent hyperreactive response; caution in coronary heart disease, tachycardia, congestive heart failure, cardiac arrhythmias, hypertension, peritonitis, ulcerative colitis, hepatic disease, and hiatal hernia with reflux esophagitis; in prostatic hypertrophy, prostatism may result in dysuria and may require catheterization

Drug Category: Antidigitalis agents

Used to treat digitalis toxicity.

Drug NameDigoxin immune Fab (Digibind)
DescriptionImmunoglobulin fragment with a specific and high affinity for both digoxin and digitoxin molecules. Removes digoxin or digitoxin molecules from tissue binding sites.
Each vial of Digibind contains 40 mg of purified digoxin-specific antibody fragments, which bind approximately 0.6 mg of digoxin or digitoxin. Dose of antibody depends on total body load (TBL) of digoxin; estimates of TBL can be made in 3 ways, as follows:
(1) Estimate quantity of digoxin ingested in acute ingestion and assume 80% bioavailability (amount ingested [mg] X 0.8 = TBL).
(2) Obtain a serum digoxin concentration and, using a pharmacokinetics formula, incorporate the Vd of digoxin and the patient's body weight in kg (TBL = digoxin serum level [ng/mL] X 6 L/kg X body weight in kg).
(3) Use an empiric dose based on average requirements for an acute or chronic overdose in an adult or child.
If the quantity of ingestion cannot be estimated reliably, administer empirically (safest to use the largest calculated estimate); alternatively, be prepared to increase dosing if resolution is incomplete.
Adult DoseIf amount ingested or serum level unknown, administer 10 vials IV for acute toxicity or 5 vials IV for chronic toxicity
If amount ingested or serum level known, dose can be calculated
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity
InteractionsNone reported
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsCaution in cardiac and renal failure; hypokalemia may occur following reversal of digoxin intoxication

Drug Category: Antiarrhythmic agents, Class 1-B

These agents alter the electrophysiologic mechanisms responsible for arrhythmia.

Drug NamePhenytoin (Dilantin)
DescriptionDepresses spontaneous depolarization in ventricular tissues.
Adult Dose15-20 mg/kg IV infusion, rate <50 mg/min
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; sinoatrial block, second- and third-degree AV block, sinus bradycardia, or Adams-Stokes syndrome
InteractionsAmiodarone, benzodiazepines, chloramphenicol, cimetidine, fluconazole, isoniazid, metronidazole, miconazole, phenylbutazone, succinimides, sulfonamides, omeprazole, phenacemide, disulfiram, ethanol (acute ingestion), trimethoprim, and valproic acid may increase phenytoin toxicity; phenytoin effects may decrease when taken concurrently with barbiturates, diazoxide, ethanol (chronic ingestion), rifampin, antacids, charcoal, carbamazepine, theophylline, and sucralfate; phenytoin may decrease effects of acetaminophen, corticosteroids, dicumarol, disopyramide, doxycycline, estrogens, haloperidol, amiodarone, carbamazepine, cardiac glycosides, quinidine, theophylline, methadone, metyrapone, mexiletine, oral contraceptives, valproic acid
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsPerform blood counts and urinalyses when therapy is begun and at monthly intervals for several months thereafter to monitor for blood dyscrasias; discontinue use if a skin rash appears and do not resume use if rash is exfoliative, bullous, or purpuric; rapid IV infusion may result in death from cardiac arrest, marked by QRS widening; caution in acute intermittent porphyria and diabetes (may elevate blood sugars); discontinue use if hepatic dysfunction occurs



Further Inpatient Care

  • AV nodal junctional rhythms generally are well tolerated; however, bradycardia for prolonged periods often causes symptoms such as dizziness and presyncope or, rarely, frank syncope in younger patients. Patients with coronary artery disease, those with significant comorbidities, or elderly patients may not tolerate a secondary junctional rhythm well and, in the acute setting, may require aggressive intervention.
  • Currently, the choices of treatment strategy include determination of the underlying cause and whether it is a normal physiologic response (ie, that observed in a young athlete) or due to a primary cardiac abnormality such as heart block.

Further Outpatient Care

  • Most of the workup on an otherwise healthy patient can be completed in an outpatient setting. Documentation of the arrhythmia on a rhythm strip is essential to properly diagnose the rhythm and to help exclude other causes.

Complications

  • Complications are usually limited to symptoms such as dizziness, dyspnea, or presyncope.
  • Accidental injury may result from syncope if the arrhythmia is not tolerated well.
  • Exacerbation of cardiac comorbidities, such as congestive heart failure and rate-related cardiac ischemia, may occur.

Prognosis

  • No evidence suggests increased mortality.
  • Prognosis is good.



Medical/Legal Pitfalls

  • Failure to monitor for the development of severe bradycardia or hypotension during treatment because this may cause syncope or death in an unstable patient
  • Failure to consider recommending limitations on potentially hazardous activities (eg, driving, operating heavy machinery) if patients are symptomatic from their arrhythmias



Media file 1:  Junctional bradycardia due to profound sinus node dysfunction. No atrial activity is apparent.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  ECG

Media file 2:  Note the retrograde P waves that precede each QRS complex.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  ECG

Media file 3:  Accelerated junctional rhythm is present in this patient. Note the inverted P waves that precede each QRS complex, with a rate of 115 bpm.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  ECG



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Junctional Rhythm excerpt

Article Last Updated: Sep 5, 2006