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

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

Journal: Autoimmunity reviews 2016;14(12):1154-60

PMID: 26284740

Full Text on Elsevier Science View on PubMed

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.

Address: AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.; APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France.; AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.; Service de Médecine Interne, CHU Nantes, France.; CHU de Reims, Centre de compétence maladies auto-immunes et systémiques rares, Reims, France.; UPMC; AP-HP, Hôpital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.; Réanimation Pédiatrique AP-HM, CHU Timone Enfants, Marseille, France.; UPMC; AP-HP, Hôpital Saint Antoine, Paris, France.; Rhumatologie, Centre de référence maladies auto-immunes et systémiques rares, Strasbourg, France.; Hôpital Louis Pradel, Lyon, France.; CHU, Angers, France.; AP-HP, Gynécologie, Hôpital Necker, Paris, France.; UPMC; Hôpital Tenon, Paris, France.; CHU Fort de France, Martinique, France.; Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Université de Lille 2, Lille, France.; AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.; AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris, France; Department of Epidemiology, Mailman School of Public Health, Columbia University, NY, USA; French Cochrane Centre, Paris.; APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.; AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France. Electronic address: [email protected].

DOI: 10.1016/j.autrev.2015.08.005

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MeSH Terms: Heart Block, Humans, Lupus Erythematosus, Systemic, Pacemaker, Artificial, Prosthesis Implantation, Treatment Outcome