title: Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study.
abstract: OBJECTIVE
  The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined.
  
  
  RESULTS
  We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%).
  
  
  CONCLUSIONS
  In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patient's age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.
authors.affiliation: INSERM, U915, l'Institut du Thorax, Nantes F-44000, France. alban.baruteau@chu-rennes.fr
authors.forename: Alban-Elouen
authors.initials: AE
authors.lastname: Baruteau
authors.forename: Swanny
authors.initials: S
authors.lastname: Fouchard
authors.forename: Albin
authors.initials: A
authors.lastname: Behaghel
authors.forename: Philippe
authors.initials: P
authors.lastname: Mabo
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Villain
authors.forename: Jean-Benoit
authors.initials: JB
authors.lastname: Thambo
authors.forename: François
authors.initials: F
authors.lastname: Marçon
authors.forename: Véronique
authors.initials: V
authors.lastname: Gournay
authors.forename: Francis
authors.initials: F
authors.lastname: Rouault
authors.forename: Alain
authors.initials: A
authors.lastname: Chantepie
authors.forename: Sophie
authors.initials: S
authors.lastname: Guillaumont
authors.forename: François
authors.initials: F
authors.lastname: Godart
authors.forename: Caroline
authors.initials: C
authors.lastname: Bonnet
authors.forename: Alain
authors.initials: A
authors.lastname: Fraisse
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Schleich
authors.forename: Jean-René
authors.initials: JR
authors.lastname: Lusson
authors.forename: Yves
authors.initials: Y
authors.lastname: Dulac
authors.forename: Christophe
authors.initials: C
authors.lastname: Leclercq
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Daubert
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Schott
authors.forename: Hervé
authors.initials: H
authors.lastname: Le Marec
authors.forename: Vincent
authors.initials: V
authors.lastname: Probst
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D054537:Atrioventricular Block / Q000151:congenital / Q000175:diagnosis / Q000628:therapy*; D002037:Bundle-Branch Block / Q000175:diagnosis / Q000209:etiology; D002304:Cardiac Pacing, Artificial / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D018572:Disease-Free Survival; D004562:Electrocardiography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010138:Pacemaker, Artificial; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult