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

Pacemaker Therapy

Author: Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Coauthor(s): Jennifer N Avari, MD, Fellow, Pediatric Electrophysiology, Children's Hospital Boston
Contributor Information and Disclosures

Updated: Nov 25, 2008

Introduction

Pacemaker therapy in children involves unique issues regarding patient size, growth, development, and possible presence of congenital heart disease. This chapter reviews unique aspects of pediatric pacemaker implantation and follow-up, with particular attention to the difficulties encountered with smaller children and patients with coexistent congenital heart defects.

History of the Procedure

Permanent pacing has been feasible for only the past 50 years. Since its inception, the technique has advanced remarkably and is now a routinely prescribed therapy.

Transvenous pacemaker implantation in young patients previously was limited by generator size and lead diameter in comparison to vascular dimensions and capacitance. Historically, epicardial pacing was more common in children. As technology has improved, generators and leads have become smaller and more advanced, allowing transvenous pacing systems in children; pacemaker therapy is even technically feasible in infants and neonates.

Problem

Pacemaker therapy in children is primarily used for abnormalities of sinus node or atrioventricular (AV) node function that lead to insufficient heart rate.

Chronotropic incompetence is the term used to describe the inability of the sinus node to increase heart rate adequately as needed for degree of activity. Sinus node dysfunction is a related term that describes inappropriately low heart rate (either sinus or nonsinus) due to abnormal activity of the normal sinus node.

AV blocks can range in severity from first degree, where the impulse is conducted through the AV node albeit delayed, to second degree, where not all impulses are conducted through the AV node, to third degree, where AV block is complete and none of the impulses are conducted across the AV node.

In addition, pacemakers are used less commonly for other disorders such as congenital long QT syndrome and cardiomyopathy. Most recently, a specialized type of pacemaker therapy (ie, cardiac resynchronization therapy [CRT]) has been used as adjunctive therapy for pediatric patients with heart failure.1,2  

Etiology

Causes of sinus node dysfunction or AV node dysfunction can be divided into 2 distinct categories: congenital and acquired. Congenital causes include congenital AV block and congenital sinus node dysfunction, which is notably less common. Congenital heart block is often due to autoantibody production from maternal systemic lupus erythematosus (SLE). Also, congenital heart block may be associated with congenital heart disease. 

Acquired forms of heart block or sinus node dysfunction are caused by infection or injury. Infections include viral myocarditis and Lyme disease. Surgical repair of congenital heart disease is the predominant cause of nodal or conduction tissue injury.

Indications

Although indications for child and adult pacing differ, both include abnormalities in sinus node and atrioventricular (AV) node function. The American Heart Association (AHA) and American College of Cardiology (ACC) published updated guidelines for implanting pacing systems in children.3 The AHA's scientific statements on pacing in children and adolescents offer the following guidelines as "indications for pacemaker implantation in children:"

Class I - Pacing indicated

  1. Advanced 2° or 3° AV block with symptomatic bradycardia, ventricular dysfunction, or low cardiac output
  2. Sinus node dysfunction with symptoms during age-inappropriate bradycardia (definition varies with patient's age and expected heart rate)
  3. Postoperative advanced 2° or 3° AV block that is not expected to resolve or that persists at least 7 days
  4. Congenital 3° AV block with a wide QRS escape rhythm, complex ventricular ectopy, or ventricular dysfunction
  5. Congenital 3° AV block in an infant with a heart rate less than 50-55 beats per minute (bpm) or with congenital heart disease and heart rate less than 70 bpm
  6. Sustained pause-dependent ventricular tachycardia, with or without prolonged QT, with thoroughly documented pacing efficacy

Class IIa - General agreement that pacing is indicated

  1. Bradycardia-tachycardia syndrome with need for long-term antiarrhythmic treatment
  2. Congenital 3° AV block beyond first year of life, with an average heart rate less than 50 bpm, abrupt pauses in ventricular rate that are 2-3 times the basic cycle length, or associated with symptoms due to chronotropic incompetence
  3. Long QT syndrome with 2:1 AV or 3° AV block
  4. Asymptomatic sinus bradycardia in a child with complex heart disease, with heart rate less than 40 bpm or pauses exceeding 3 seconds
  5. Patients with congenital heart disease and impaired hemodynamics due to sinus bradycardia or loss of AV synchrony

Class IIb - No consensus, reasonable guidelines for implantation

  1. Transient postoperative 3° AV block that reverts to sinus rhythm with residual bifascicular block
  2. Congenital 3° AV block in an asymptomatic infant, child, or young adult with acceptable rate and function and narrow QRS
  3. Asymptomatic sinus bradycardia in congenital heart disease, with heart rate less than 40 bpm or pauses exceeding 3 seconds
  4. Neuromuscular disease with any degree of AV block (including first degree) with or without symptoms, as there may be unpredictable progression of AV block 
Class III - Not indicated
  1. Transient postoperative AV block with return of normal AV conduction
  2. Asymptomatic postoperative bifascicular block with or without 1° AV block
  3. Asymptomatic type I 2° AV block
  4. Asymptomatic sinus bradycardia in adolescents with longest R-R less than 3 seconds and minimum heart rate more than 40 bpm

Relevant Anatomy

Congenital heart disease in pacemaker recipients

Congenital (or acquired) structural heart disease presents additional issues for pacemaker therapy in children. These patients may have a more critical reliance on adequate hemodynamic status. Optimal hemodynamic performance is achieved with atrial synchronous pacing. Maintaining atrioventricular (AV) synchrony in this population is often more important than in children who have structurally normal hearts. For example, in a newborn with congenital complete heart block but a structurally normal heart, an epicardial ventricular pacing system initially suffices to meet hemodynamic needs. In contrast, a newborn with significant structural heart disease and congestive heart block may benefit more from a dual-chamber system to obtain AV synchrony and meet hemodynamic needs.

In general, the transvenous route is a reasonable approach for children weighing at least 10 kg, although other authors have reported successful transvenous pacing in neonates without complications. Physical considerations include absence of intracardiac shunting, low-flow states, and anatomic barriers (eg, mechanical valves that block pacing leads).

Transvenous lead placement in congenital heart patients often requires nonstandard positioning because of variations in venous and intracardiac anatomy. The atrial appendage is often concurrently amputated with cannulation for cardiac bypass, and atrial anatomy is often different from usual in pacemaker patients. The use of active-fixation leads allows for easier sampling of nonstandard pacing sites and easier removal. In specific congenital heart surgeries, such as the Fontan procedure, the right medial wall often is viable; in postoperative Mustard and Senning procedures, superior aspects of the systemic venous atrium are most optimal. High-output pacing is imperative testing for diaphragmatic or phrenic nerve stimulation, particularly in lateral pacing sites. The active pacing lead tip may be used for mapping of optimal tissue implant sites.

Although not universally recommended, anticoagulation may help patients who have leads implanted in low-flow chambers. Also consider the possibility of future rhythm complications in children who have undergone palliative repairs. For example, a child who develops heart block after a Fontan procedure needs ventricular pacing. These children also risk developing sinus node dysfunction, so placing an atrial pacing lead concurrent with ventricular lead placement may prove beneficial.

Certain populations also have greater risk of developing atrial tachycardia; therefore, pacemaker type is important. A patient with sinus node dysfunction who is at risk for atrial tachycardia should probably have a generator placed that also allows for antitachycardia pacing, either manually or automatically. There are now automatic atrial antitachycardia pacemakers that have been shown to be effective in pediatrics and congenital heart disease patients.

Additional concerns include patients who require a pacemaker but who also are at risk of developing ventricular arrhythmias (eg, an older patient with previous repaired tetralogy of Fallot). If these patients require a pacemaker, avoiding placement in the left subclavian vein and pectoral pocket may be prudent, thus reserving this area for future potential placement of an implantable cardioverter defibrillator (ICD).

For those patients with heart failure, cardiac resynchronization therapy (CRT) may be used in conjunction with medical therapy to improve cardiac function and quality of life. Although indications for CRT in pediatrics remains controversial, important anatomical considerations are noted. CRT requires 2 strategically placed leads in the ventricles (typically one in the right ventricle and one in the left ventricle) that pace 2 areas of the ventricles to synchronize ventricular contraction. A transvenous approach requires a patent coronary venous system that is accessed via the coronary sinus. Typically, the left ventricular lead is placed through the coronary sinus and then carefully maneuvered into one of the tributary cardiac veins of the left ventricle. Patients with congenital heart disease often have distorted coronary venous anatomy; in these patients, CRT may require an epicardial approach.

Contraindications

See Class III guidelines for general nonindications for pacemaker implantation in children. Expected survival of less than 6 months is a relative contraindication to permanent pacing therapy for patients who are terminally ill.

Patients who fully regain normal conduction after transient postoperative heart block typically do not need to receive a permanent pacemaker.

More on Pacemaker Therapy

Overview: Pacemaker Therapy
Workup: Pacemaker Therapy
Treatment: Pacemaker Therapy
Follow-up: Pacemaker Therapy
Multimedia: Pacemaker Therapy
References
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References

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Further Reading

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Keywords

pacemaker therapy, pacing, permanent pacing, pacemaker implantation, transvenous pacemaker, implanted cardioverter/defibrillator, ICD, congenital heart disease, sinus node dysfunction, atrioventricular node function, heart rate, long QT syndrome, cardiomyopathy, cardiac resynchronization therapy, CRT, congenital atrioventricular block, acquired atrioventricular block, congenital AV block, acquired AV block, systemic lupus erythematosus, SLE, congenital heart block, Lyme disease, viral myocarditis, symptomatic bradycardia, ventricular dysfunction, or low cardiac output, ventricular tachycardia, bradycardia-tachycardia syndrome, tetralogy of Fallot

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Contributor Information and Disclosures

Author

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I 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.

Coauthor(s)

Jennifer N Avari, MD, Fellow, Pediatric Electrophysiology, Children's Hospital Boston
Jennifer N Avari, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and Heart Rhythm Society
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, Heart Rhythm Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Division of Pediatric Cardiology, Mattel Children's Hospital of University of California at Los Angeles
John W Moore, MD, MPH is a member of the following medical societies: Society for Cardiac Angiography and Interventions
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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