Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management

Alban Elouen Baruteau, Robert H. Pass, Jean Benoit Thambo, Albin Behaghel, Solène Le Pennec, Elodie Perdreau, Nicolas Combes, Leonardo Liberman, Christopher J. McLeod

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Atrioventricular block is classified as congenital if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental passage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive cardiac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe permanent cardiac pacing in almost all patients, including those with structural heart abnormalities. Conclusion: Early diagnosis and appropriate management are critical in many cases in order to prevent sudden death, and this review critically assesses our current understanding of the pathogenetic mechanisms, clinical course, and optimal management of congenital and childhood AV block.(Table presented.)

Original languageEnglish (US)
Pages (from-to)1-14
Number of pages14
JournalEuropean Journal of Pediatrics
DOIs
StateAccepted/In press - Jun 28 2016

Fingerprint

Atrioventricular Block
Congenital Heart Defects
Sudden Death
Early Diagnosis
Mothers
Parturition
Antibodies
Wounds and Injuries
Genes

Keywords

  • Congenital heart disease
  • Heart block
  • Outcomes
  • Pacemaker
  • Pathophysiology

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Baruteau, A. E., Pass, R. H., Thambo, J. B., Behaghel, A., Le Pennec, S., Perdreau, E., ... McLeod, C. J. (Accepted/In press). Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management. European Journal of Pediatrics, 1-14. https://doi.org/10.1007/s00431-016-2748-0

Congenital and childhood atrioventricular blocks : pathophysiology and contemporary management. / Baruteau, Alban Elouen; Pass, Robert H.; Thambo, Jean Benoit; Behaghel, Albin; Le Pennec, Solène; Perdreau, Elodie; Combes, Nicolas; Liberman, Leonardo; McLeod, Christopher J.

In: European Journal of Pediatrics, 28.06.2016, p. 1-14.

Research output: Contribution to journalArticle

Baruteau, AE, Pass, RH, Thambo, JB, Behaghel, A, Le Pennec, S, Perdreau, E, Combes, N, Liberman, L & McLeod, CJ 2016, 'Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management', European Journal of Pediatrics, pp. 1-14. https://doi.org/10.1007/s00431-016-2748-0
Baruteau, Alban Elouen ; Pass, Robert H. ; Thambo, Jean Benoit ; Behaghel, Albin ; Le Pennec, Solène ; Perdreau, Elodie ; Combes, Nicolas ; Liberman, Leonardo ; McLeod, Christopher J. / Congenital and childhood atrioventricular blocks : pathophysiology and contemporary management. In: European Journal of Pediatrics. 2016 ; pp. 1-14.
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