title: Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.
abstract: BACKGROUND
  Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.
  
  
  RESULTS
  We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting. In 80% of 162 pregnancies with documented antibody status, atrioventricular block was associated with maternal anti-Ro/SSA antibodies. Sixty-seven cases (38%) were treated with fluorinated corticosteroids for a median of 10 weeks (1-21 weeks). Ninety-one percent were alive at birth, and survival in the neonatal period was 93%, similar in steroid-treated and untreated fetuses, regardless of degree of block and/or presence of anti-Ro/SSA. Variables associated with death were gestational age < 20 weeks, ventricular rate ≤ 50 bpm, fetal hydrops, and impaired left ventricular function at diagnosis. The presence of ≥ 1 of these variables was associated with a 10-fold increase in mortality before birth and a 6-fold increase in the neonatal period independently of treatment. Except for a lower gestational age at diagnosis in treated than untreated (23.4 ± 2.9 versus 24.9 ± 4.9 weeks; P=0.02), risk factors were distributed equally between treatment groups. Two-thirds of survivors had a pacemaker by 1 year of age; 8 children developed cardiomyopathy.
  
  
  CONCLUSIONS
  Risk factors associated with a poor outcome were gestation < 20 weeks, ventricular rate ≤ 50 bpm, hydrops, and impaired left ventricular function. No significant effect of treatment with fluorinated corticosteroids was seen.
authors: Pediatric Cardiology Q1:03, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden. hakan.eliasson@karolinska.se
authors: Håkan
authors: H
authors: Eliasson
authors: Sven-Erik
authors: SE
authors: Sonesson
authors: Gurleen
authors: G
authors: Sharland
authors: Fredrik
authors: F
authors: Granath
authors: John M
authors: JM
authors: Simpson
authors: Julene S
authors: JS
authors: Carvalho
authors: Hana
authors: H
authors: Jicinska
authors: Viktor
authors: V
authors: Tomek
authors: Joanna
authors: J
authors: Dangel
authors: Paulo
authors: P
authors: Zielinsky
authors: Maria
authors: M
authors: Respondek-Liberska
authors: Matthias W
authors: MW
authors: Freund
authors: Mats
authors: M
authors: Mellander
authors: Joaquim
authors: J
authors: Bartrons
authors: Helena M
authors: HM
authors: Gardiner
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D054537:Atrioventricular Block / Q000188:drug therapy / Q000276:immunology / Q000401:mortality*; D005260:Female; D005313:Fetal Death / Q000453:epidemiology*; D005315:Fetal Diseases / Q000188:drug therapy / Q000276:immunology / Q000401:mortality*; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D010956:Plasmapheresis; D011247:Pregnancy; D011256:Pregnancy Outcome / Q000453:epidemiology; D012189:Retrospective Studies; D012307:Risk Factors; D013256:Steroids / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000974:Antibodies, Antinuclear; C035356:SS-A antibodies; C035357:SS-B antibodies; D013256:Steroids