Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome.

Abstract

BACKGROUND:

Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB.

METHODS:

Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies.

RESULTS:

214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p<0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7-32.7]) and prematurity (p=0.002; HR 17.1 [95%CI 2.8-103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p=0.0157; HR 6.37 [95%CI: 1.25-32.44]), postnatal DCM (p<0.0001; HR 227.58[95%CI: 24.33-2128.46]) and pacemaker implantation (p=0.0035; HR 0.11[95%CI: 0.02-0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB.

CONCLUSION:

In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids.

• Authors: Kateri Levesque, Nathalie Morel, Alice Maltret, Gabriel Baron, Agathe Masseau, Pauline Orquevaux, Jean-Charles Piette, Francois Barriere, Jérome Le Bidois, Laurent Fermont, Olivier Fain, Arnaud Theulin, Francois Sassolas, Philippe Pezard, Zahir Amoura, Gaëlle Guettrot-Imbert, Delphine Le Mercier, Sophie Georgin-Lavialle, Christophe Deligny, Eric Hachulla, Luc Mouthon, Philippe Ravaud, Elisabeth Villain, Damien Bonnet, Nathalie Costedoat-Chalumeau, “Lupus néonatal” group, Group of collaborators
• Journal: Autoimmunity reviews (Autoimmun Rev) Vol. 14 Issue 12 Pg. 1154-60 (Dec 2015) ISSN: 1873-0183 [Electronic] Netherlands
• PMID: 26284740 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2015 Elsevier B.V. All rights reserved.

Topics

• Heart Block (complications, congenital, immunology, surgery)
• Humans
• Lupus Erythematosus, Systemic (complications, congenital, immunology)
• Pacemaker, Artificial
• Prosthesis Implantation
• Treatment Outcome