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Pediatric Congenital Atrioventricular Block Treatment & Management

  • Author: Monesha Gupta, MD, MBBS, FAAP, FACC, FASE; Chief Editor: P Syamasundar Rao, MD  more...
Updated: Mar 02, 2016

Approach Considerations

Pharmacologic therapy

Little evidence suggests that the administration of steroids or immunoglobulins or the performance of plasmapheresis in the mother can reverse third-degree AV block in the fetus. However, these therapies may be helpful in early first- and second-degree heart block and in reducing associated myocardial dysfunction.

Regular and close monitoring for heart block and transplacental therapy with fluorinated steroids (dexamethasone) and beta sympathomimetics have been used by some centers with satisfactory results at the first evidence of heart block and bradycardia with hydrops, respectively. Prophylactic therapy is not currently indicated, because these medications can have maternal and fetal side effects.[14]

Children with CAVB due to heart disease, if associated with pulmonary atresia or severe stenosis, may need to be on prostaglandin therapy to keep the ductus patent.

In patients with CAVB, prophylactic antibiotic therapy during and following surgery appears to reduce the incidence of pacemaker system infections, although current studies do not reach statistical significance because of the relative rarity of this complication (approximately 1%).

Hydrops fetalis

Therapy for hydrops fetalis can be challenging in utero and after birth. A staged approach with stabilization of the patient, drainage of the effusions, the use of intravenous inotropes (dopamine) and chronotropes (isoproterenol), and placement of a pacemaker has shown some success. Some centers recommend cesarean and preterm delivery for fetuses with CAVB and hydrops fetalis.


In the perinatal period, fetal bradycardia may be an incidental finding. However, when persistent bradycardia is identified in the third trimester, emergency delivery is sometimes inappropriately carried out without differentiating acute causes of bradycardia related to fetal distress from the more chronic condition of isolated CAVB.


Pacemaker Therapy

The medical care of congenital atrioventricular block (CAVB) is currently focused on identifying the optimal timing of pacemaker therapy to ensure a positive outcome. Additional care is aimed at identifying and treating the associated conditions that arise. Patients who present with symptoms as fetuses or newborns may require earlier pacing than do those who present with symptoms later in childhood. Fetuses with hydrops fetalis secondary to maternal autoimmune disease have successfully received pacing in utero; however, this has not prevented fetal demise.

Symptoms referable to bradycardia are an indication for pacing in complete CAVB. Because severe symptoms (eg, syncope) occasionally arise suddenly or sudden cardiac death can occur, additional risk factors identified from monitoring have been recommended as indications for pacing.[15]

Temporary pacing can be achieved transcutaneously, transesophageally, or transvenously. However, permanent pacemaker placement is eventually needed in most children with CAVB. Major criteria for pacing based on ECG or Holter monitoring include an average heart rate of less than 50 bpm, a nighttime or sleeping average heart rate of less than 45 bpm, or pauses secondary to AV block that are longer than 3 seconds.

In the setting of borderline major criteria, other criteria that may influence the decision for timing of pacemaker implantation include cardiomegaly, a high atrial rate, junctional instability (eg, junctional exit block), a broad complex escape rhythm, diminished ventricular response to exercise, QT prolongation, and complex ventricular ectopy.

When criteria are met, surgical implantation of an epicardial pacemaker should be performed, with knowledge of the implications of pacing in children and recognition of the lifelong need for pacing and pacemaker lead access. Multiple backup pacing systems at the time of surgery (eg, temporary transvenous pacing, transthoracic pacing units) are helpful to avoid the extremes of bradycardia that may be associated with anesthesia and pacemaker surgery.

Venous obstruction, small patient size, and actual or potential right-left shunting are relative contraindications for transvenous pacing. Fortunately, epicardial pacing leads have greatly improved and allow for successful pacing system insertion under these conditions. Some centers are using neonatal endocardial pacing systems.

In children older than the neonatal period, an endocardial pacing with transvenous approach or robot-assisted thoracoscopic implantation of left ventricular leads is possible. The risk of venous occlusion appears to correlate with the ratio of available transvenous lead body sizes and the patient's body surface area.[16] Exceeding this ratio may lead to a high incidence of venous obstruction. Robot-assisted minimally invasive surgery has the advantage of avoiding intravascular endocarditis and thrombosis.

In addition, imaging of the subclavian-innominate venous channels before pacemaker lead placement is helpful for transvenous lead placement. The course of the lead should be evaluated in 2 planes to avoid inadvertent lead placement into the foramen ovale, an unroofed left superior vena cava to coronary sinus, or left-sided circulation via the subclavian artery. Either a subcutaneous or subpectoral pocket may be formed inferior to the clavicle.

Major lawsuits related to pacing in congenital atrioventricular block (CAVB) have arisen primarily because of inadvertent placements of pacing leads into the left-sided circulation. This complication can be recognized at or immediately following system placement using complementary radiographic views, usually lateral and posteroanterior (PA) or anteroposterior (AP).


Routine pacemaker follow-up visits should be maintained according to national or international guidelines. Transtelephonic pacemaker monitoring may allow for improved follow-up care and longer intervals between outpatient visits.


Patients with permanent pacing systems should be restricted from activities that result in repeated intentional direct trauma to the pacemaker area (eg, martial arts). Patients with permanent pacing systems should be restricted from exposure to high magnetic fields, such as direct magnetic resonance imaging (MRI) exposure.

Contributor Information and Disclosures

Monesha Gupta, MD, MBBS, FAAP, FACC, FASE Associate Professor of Pediatrics, Division of Pediatric Cardiology and Nephrology, Children's Memorial Hermann Hospital, University of Texas Medical School

Monesha Gupta, MD, MBBS, FAAP, FACC, FASE is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Medical Council of India

Disclosure: Nothing to disclose.


Robert Murray Hamilton, MD, MSc, FRCPC Electrophysiologist, 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 Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Heart Rhythm Society, Canadian Cardiovascular Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.


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

Alvin J Chin, MD Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

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.

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