Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Congenital Atrioventricular Block Clinical Presentation

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


Congenital atrioventricular block (CAVB) may be identified during prenatal examinations, in the perinatal period, or during childhood or adulthood. Historical and other features widely vary and particularly depend on the timing of presentation and accompanying structural heart disease.

Isolated CAVB

Many of the mothers confirmed to have a fetus with autoimmune AV block may have no symptoms. The fetus in whom isolated CAVB is identified during the perinatal period usually presents with an incidental finding of bradycardia or hydrops fetalis.

Occasionally, these mothers may have a history of recurrent fetal loss. Fetuses of such mothers should be routinely evaluated for CAVB.

The mother is often completely asymptomatic. In addition to fetuses who are incidentally identified, siblings of children with known isolated heart block are at a higher risk with a recurrence rate for heart block of 17-22%.

In the perinatal period, fetal bradycardia may have myriad causes. 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.

Newborns with congenital heart block may present with a hydropic appearance secondary to fetal heart failure or may develop signs of low cardiac output within hours to days after birth. However, affected newborns often appear asymptomatic and may have accelerated ventricular rates approaching those of healthy newborns. Congenital heart block is not likely to be identified in many such infants until well after birth. An associated finding of neonatal lupus may be present.

In older infants or children, signs of low cardiac output due to bradycardia, such as pallor, mottling, lethargy, exercise intolerance, palpitations, dizziness, or syncope, may occur. Children may have sleep disturbances or be asymptomatic.

CAVB with structural heart disease

The diagnosis of a congenital heart defect in one child or first-degree relative is an indication for fetal echocardiography to check for cardiac malformations.

Children with structural heart defects may present with cyanosis, murmur, failure to thrive, or recurrent pneumonias or may be completely asymptomatic in childhood (such as children with L-transposition of the great arteries and intact ventricular septum).


Physical Examination

The fetus may be monitored with ultrasonography for varying degrees of heart block and hydrops fetalis. In the newborn, the findings may range from asymptomatic to signs of congestive heart failure and low cardiac output.

The most typical physical finding is a low heart rate for age. Because the block is usually complete and the escape rhythm is usually junctional in origin, a regular rhythm at 60-80 beats per minute (bpm) is often found. Auscultation demonstrates a variable first heart sound caused by the AV asynchrony.

Congenital AV block may be associated with findings of low cardiac output or congestive heart failure. Low cardiac output may manifest with physical findings of irritability or lethargy, cool skin, mottling, or cyanosis.

Congestive heart failure may manifest with tachypnea and hepatomegaly. If congestive heart failure has been present prenatally, marked edema may be part of the complex of hydrops fetalis.

Children with neonatal lupus can present with rash, as well as with neurologic and hepatic manifestations. The rash can occur some days after birth and is worsened with sun exposure. Annular or elliptical erythematous plaques can be present on the skin of face, scalp, and extremities. It usually resolves without scarring but can be associated with residual hypopigmentation or, rarely, telangiectasias.

Children with structural heart disease may manifest symptoms and signs related to the specific congenital heart defect. Patients with heterotaxia syndrome may be cyanotic. Patients with L-transposition of the great arteries may be completely asymptomatic or may demonstrate a murmur.

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.

  1. 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. 2006 Aug. 26(10):886-95. [Medline].

  2. 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].

  3. 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].

  4. Ambrosi A, Sonesson SE, Wahren-Herlenius M. Molecular mechanisms of congenital heart block. Exp Cell Res. 2014 Jul 1. 325 (1):2-9. [Medline].

  5. Skog A, Lagnefeldt L, Conner P, Wahren-Herlenius M, Sonesson SE. Outcome in 212 anti-Ro/SSA-positive pregnancies and population-based incidence of congenital heart block. Acta Obstet Gynecol Scand. 2016 Jan. 95 (1):98-105. [Medline].

  6. Abadir S, Fournier A, Vobecky SJ, Rohlicek CV, Romeo P, Khairy P. Left Atrial Inexcitability in Children With Congenital Lupus-Induced Complete Atrioventricular Block. J Am Heart Assoc. 2015 Dec 16. 4 (12):[Medline].

  7. Cozzani E, Agnoletti AF, Pappalardo F, Schiavetti I, Torino A, Parodi A. The high incidence of anti-Ro/SSA and anti-p200 antibodies in female patients with connective tissue diseases confirms the importance of screening for congenital heart block-associated autoantibodies during pregnancy. Arch Dermatol Res. 2016 Mar. 308 (2):139-43. [Medline].

  8. Yan J, Varma SK, Malhotra A, Menahem S. Congenital complete heart block: single tertiary centre experience. Heart Lung Circ. 2012 Nov. 21(11):666-70. [Medline].

  9. Weng KP, Chiou CW, Huang SH, et al. The long-term outcome of children with isolated congenital complete atrioventricular block. Acta Paediatr Taiwan. 2005 Sep-Oct. 46(5):260-7. [Medline].

  10. Miyoshi T, Maeno Y, Sago H, et al. Fetal bradyarrhythmia associated with congenital heart defects - nationwide survey in Japan. Circ J. 2015. 79 (4):854-61. [Medline].

  11. Kuleva M, Le Bidois J, Decaudin A, et al. Clinical course and outcome of antenatally detected atrioventricular block: experience of a single tertiary centre and review of the literature. Prenat Diagn. 2015 Apr. 35 (4):354-61. [Medline].

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

  13. 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. 2005 Jul. 26(1):16-21. [Medline].

  14. 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].

  15. Martin TA. Congenital heart block: current thoughts on management, morphologic spectrum, and role of intervention. Cardiol Young. 2014 Oct. 24 Suppl 2:41-6. [Medline].

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

  17. Doti PI, Escoda O, Cesar-Diaz S, et al. Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center. Clin Rheumatol. 2016 Feb. 35 (2):351-6. [Medline].

All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.