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

作者：Levesque Kateri; Morel Nathalie; Maltret Alice; Baron Gabriel; Masseau Agathe; Orquevaux Pauline; Piette Jean Charles; Barriere Francois; Le Bidois Jerome; Fermont Laurent; Fain Olivier; Theulin Arnaud; Sassolas Francois; Pezard Philippe; Amoura Zahir; Guettrot Imbert Gaelle; Le Mercier Delphine; Georgin Lavialle Sophie; Deligny Christophe; Hachulla Eric; Mouthon Luc; Ravaud Philippe; Villain Elisabeth; Bonnet Damien; Costedoat Chalumeau Nathalie^*

来源：Autoimmunity Reviews, 2015, 14(12): 1154-1160.

DOI：10.1016/j.autrev.2015.08.005

• Congenital heart block
• Neonatal lupus erythematosus syndrome
• Dilated cardiomyopathy
• Anti-SSA/Ro antibodies
• Pregnancy

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 [HA] 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.

• 出版日期2015-12