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Pediatric Congenital Atrioventricular Block Workup

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

Approach Considerations

After birth, electrocardiography (ECG) is recommended to assess for CAVB and to assess the QT interval that can be prolonged. ECG and Holter ambulatory ECG monitoring are routinely performed initially and periodically in patients with complete CAVB.

Holter monitoring (ambulatory ECG) is recommended to determine if the AV block is intermittent or persistent and to evaluate for associated arrhythmias.

Exercise testing is performed on a regular basis in patients who are capable, usually in patients older than age 7 years. Electrophysiologic testing is not routinely performed in patients with CAVB, although it provides information regarding pathophysiology and prognosis in certain cases.

Chest radiography may reveal cardiomegaly and pleural effusions. Heterotaxia may be suspected in presence of visceroatrial discordance. One should look for a midline liver, rightward stomach bubble, and dextrocardia. Looking at lead placement in temporary and permanent pacemakers is helpful.

Consultations

The mother should consult with a rheumatologist to begin monitoring for possible autoimmune disease. Consultation with a rheumatologist is also advised for the infant, particularly if other manifestations of neonatal lupus erythematosus are present.

Genetic consultation is recommended for children with first-degree relatives with structural heart disease or those with storage disorder or cardiomyopathy.

Pediatric cardiology consultation is necessary for every case.

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Laboratory Studies

In patients with CAVB, routine electrolyte assays should be performed to assess for metabolic derangements (especially hyperkalemia), and a complete blood count (CBC) should be obtained to assess for anemia, neutropenia, and thrombocytopenia.

Neonatal assessment should include a measurement of anti-Ro and anti-La antibody levels, preferably using a line immunoassay[3] in the mother. Assessment for other organ and/or tissue damage should include a platelet assessment to rule out thrombocytopenia and an assessment of liver enzymes to rule out alloimmune hepatitis.

If inflammatory disease is suspected, workup for various infectious etiologies (eg, human immunodeficiency virus [HIV]) should be performed. Cardiac troponins and pro-brain natriuretic peptide (BNP) levels can be helpful in assessing the severity of the disease.

If hypertrophic cardiomyopathy is suspected, evaluation for lysosomal storage diseases should be included.

Preductal and post-ductal saturations by pulse oximetry should be performed. Check for hypoxemia and acidosis using ABG findings. Also, check lactic acid for perfusion. A hyperoxia test is very helpful in differentiating lung disease–related hypoxemia versus hypoxemia due to a cyanotic heart disease. One can evaluate for Howell-Jolly/Heinz bodies on peripheral smear for evaluation of asplenia that is seen with heterotaxia syndromes.

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Ultrasonography

Echocardiography should be performed initially and in periodic follow-up care in affected fetuses, infants, and children to assess ventricular function and size and to rule out congenital or acquired cardiac malformations or valve dysfunction.[13]

Children of mothers with the anti-Ro and anti-La antibodies should undergo regular fetal ultrasonographic assessments, including detailed fetal echocardiography to identify conduction delay, bradycardia, and ventricular function. All children, regardless of fetal findings, should undergo an electrocardiogram after birth to check the conduction intervals. Children with first-degree or second-degree AV block have been known to progress to third-degree CAVB.

Mothers with the autoimmune antibodies or those with one affected child should undergo regular fetal ultrasonographic assessments, including detailed fetal echocardiography, to identify subsequent affected pregnancies starting early, at 16 weeks' gestation. Fetal echocardiography may reveal varying degrees of AV block. Fetal monitoring should be performed to look for bradycardia, fetal distress, and hydrops fetalis.

Fetal echocardiography is geared towards looking for early heart block, bradycardia, arrhythmias, pericardial effusion, cardiomegaly, valvular insufficiency (especially tricuspid regurgitation), decreasing contractile function, and abnormal venous and arterial pulsations.

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[#WorkupHistologicFindings]Histologic Findings

Myocardial biopsies are not routinely performed in patients with CAVB. However, histologic findings have been reported from experimental studies and autopsy specimens; these findings demonstrated various stages of fibrosis and calcification of the AV conduction area, depending on the timing of the specimen.

Immune deposition is also a frequent finding, although whether this is specific for the conduction system or occurs throughout the myocardium in general is unclear. The mechanisms of cell death and fibrosis are unclear. Hypotheses include alloimmune-mediated inflammatory responses and immune-triggered apoptosis.

In some cases, the sinoatrial node has also been found to be affected and may be hypoplastic, fibrotic, or completely absent.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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.

Acknowledgements

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.

References
  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].

 
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