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

Atrioventricular Block, Third Degree, Congenital: Follow-up

Author: Monesha Gupta, MD, MBBS, FAAP, FACC, Assistant Professor, Division of Pediatric Cardiology, University of Texas Medical School, Children's Memorial Hermann Hospital
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: Feb 2, 2009

Follow-up

Further Inpatient Care

  • In patients with congenital atrioventricular block (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%).

Further Outpatient Care

  • 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.
  • Family and patient should be instructed to avoid medications that can cause atrioventricular (AV) block (eg, calcium channel blockers, beta-blockers, other antiarrhythmic agents).

Transfer

  • According to a long-term follow-up study by Michaelsson and colleagues, adults with complete CAVB who did not receive pacing systems had a poorer prognosis than those with pacing because of multiple complications related to their disease.2 Therefore, in the adolescent who has not yet developed indications for pacing (an unusual case), recommendations for a pacing system should be considered, regardless of symptoms or underlying escape rate.

Complications

  • Long-term potential complications in all patients include development of ventricular dilatation and dysfunction. Patients without pacemakers may develop AV valve regurgitation, atrial rhythm disorders, thromboembolism, congestive failure, or sudden death. Patients with a pacemaker may develop pacing system–related complications, including lead fracture, malsensing, and pacing system infections.

Prognosis

  • The prognosis in complete CAVB is relatively good but may be influenced by the patient's age at presentation. Congenital complete heart block is an increasingly recognized cause of fetal loss. In addition, patients presenting as fetuses or at birth have significantly higher morbidity and mortality rates than patients presenting later in childhood.

Patient Education

  • Parents who are at risk of having a child with CAVB must be informed that this disease is easily identifiable and relatively easily treated after birth. The stigma of pacing as a therapy associated with elderly persons should be avoided. Parents should recognize that their affected offspring are likely to receive and benefit from pacing therapy at some point during childhood but that pacemaker therapy is intentionally deferred until indications are present to preserve lifelong access for pacing systems.

Miscellaneous

Medicolegal Pitfalls

  • 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).
  • Other important pitfalls relate to patients with CAVB who have had a morbid or fatal event secondary to not receiving a permanent pacemaker.

Special Concerns

  • Fetuses with hydrops fetalis secondary to maternal autoimmune disease have successfully received pacing in utero; however, this has not prevented fetal demise.
 


More on Atrioventricular Block, Third Degree, Congenital

Overview: Atrioventricular Block, Third Degree, Congenital
Differential Diagnoses & Workup: Atrioventricular Block, Third Degree, Congenital
Treatment & Medication: Atrioventricular Block, Third Degree, Congenital
Follow-up: Atrioventricular Block, Third Degree, Congenital
References
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References

  1. Figa FH, McCrindle BW, Bigras JL, et al. Risk factors for venous obstruction in children with transvenous pacing leads. Pacing Clin Electrophysiol. Aug 1997;20(8 Pt 1):1902-9. [Medline].

  2. Michaelsson M, Jonzon A, Riesenfeld T. Isolated congenital complete atrioventricular block in adult life. A prospective study. Circulation. Aug 1 1995;92(3):442-9. [Medline]. [Full Text].

  3. Boutjdir M, Chen L, Zhang ZH, et al. Arrhythmogenicity of IgG and anti-52-kD SSA/Ro affinity-purified antibodies from mothers of children with congenital heart block. Circ Res. Mar 1997;80(3):354-62. [Medline].

  4. Boutjdir M, Chen L, Zhang ZH, et al. Serum and immunoglobulin G from the mother of a child with congenital heart block induce conduction abnormalities and inhibit L-type calcium channels in a rat heart model. Pediatr Res. Jul 1998;44(1):11-9. [Medline].

  5. Claus R, Hickstein H, Kulz T, et al. Identification and management of fetuses at risk for, or affected by, congenital heart block associated with autoantibodies to SSA (Ro), SSB (La), or an HsEg5-like autoantigen. Rheumatol Int. Aug 2006;26(10):886-95. [Medline].

  6. Copel JA, Buyon JP, Kleinman CS. Successful in utero therapy of fetal heart block. Am J Obstet Gynecol. Nov 1995;173(5):1384-90. [Medline].

  7. Costedoat-Chalumeau N, Amoura Z, Villain E, et al. Anti-SSA/Ro antibodies and the heart: more than complete congenital heart block? A review of electrocardiographic and myocardial abnormalities and of treatment options. Arthritis Res Ther. 2005;7(2):69-73. [Medline].

  8. Costedoat-Chalumeau N, Georgin-Lavialle S, Amoura Z, et al. Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block. Lupus. 2005;14(9):660-4. [Medline].

  9. Cutler NG, Karpawich PP, Cavitt D, et al. Steroid-eluting epicardial pacing electrodes: six year experience of pacing thresholds in a growing pediatric population. Pacing Clin Electrophysiol. Dec 1997;20(12 Pt 1):2943-8. [Medline].

  10. Friedman DM, Kim MY, Copel JA, et al. Utility of cardiac monitoring in fetuses at risk for congenital heart block: the PR Interval and Dexamethasone Evaluation (PRIDE) prospective study. Circulation. 2008;117:485-93. [Medline].

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  12. Hamilton R, Gow R, Bahoric B, et al. Steroid-eluting epicardial leads in pediatrics: improved epicardial thresholds in the first year. Pacing Clin Electrophysiol. Nov 1991;14(11 Pt 2):2066-72. [Medline].

  13. Hamilton RM, Chiu C, Gow RM, Williams WG. A comparison of two stab-on unipolar epicardial pacing leads in children. Pacing Clin Electrophysiol. Mar 1997;20(3 Pt 1):631-6. [Medline].

  14. Jaeggi ET, Hornberger LK, Smallhorn JF, Fouron JC. Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol. Jul 2005;26(1):16-21. [Medline].

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  17. Miranda-Carus ME, Boutjdir M, Tseng CE. Induction of antibodies reactive with SSA/Ro-SSB/La and development of congenital heart block in a murine model. J Immunol. Dec 1 1998;161(11):5886-92. [Medline].

  18. Moak JP, Barron KS, Hougen TJ, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela. J Am Coll Cardiol. Jan 2001;37(1):238-42. [Medline].

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  20. Rao V, Williams WG, Hamilton RH, et al. Trends in pediatric cardiac pacing. Can J Cardiol. Dec 1995;11(11):993-9. [Medline].

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Further Reading

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Keywords

third degree congenital atrioventricular block, third-degree congenital atrioventricular block, CAVB, congenital heart block, congenital complete heart block, congenital complete atrioventricular block, atrioventricular, AV, congential complete AV block, congential complete A-V block, autoimmune complete heart block, 3° atrioventricular block, 3° AV block, 3° A-V block, collagen vascular disease, systemic lupus erythematosus, Sjogren syndrome, Hunter syndrome, Hurler syndrome, myocarditis, hydrops fetalis, endocardial fibroelastosis, L-transposition of the great arteries, ventricular septal defect

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Contributor Information and Disclosures

Author

Monesha Gupta, MD, MBBS, FAAP, FACC, Assistant Professor, Division of Pediatric Cardiology, University of Texas Medical School, Children's Memorial Hermann Hospital
Monesha Gupta, MD, MBBS, FAAP, FACC is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Society of Echocardiography, Medical Council of India, and Society of Pediatric Echocardiography
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 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.

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 financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
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

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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