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


CONCLUSIONS
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.
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
Kateri
K
Levesque
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
Nathalie
N
Morel
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France.
Alice
A
Maltret
AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
Gabriel
G
Baron
Service de Médecine Interne, CHU Nantes, France.
Agathe
A
Masseau
CHU de Reims, Centre de compétence maladies auto-immunes et systémiques rares, Reims, France.
Pauline
P
Orquevaux
UPMC; AP-HP, Hôpital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
Jean-Charles
JC
Piette
Réanimation Pédiatrique AP-HM, CHU Timone Enfants, Marseille, France.
Francois
F
Barriere
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France.
Jérome
J
Le Bidois
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France.
Laurent
L
Fermont
UPMC; AP-HP, Hôpital Saint Antoine, Paris, France.
Olivier
O
Fain
Rhumatologie, Centre de référence maladies auto-immunes et systémiques rares, Strasbourg, France.
Arnaud
A
Theulin
Hôpital Louis Pradel, Lyon, France.
Francois
F
Sassolas
CHU, Angers, France.
Philippe
P
Pezard
UPMC; AP-HP, Hôpital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
Zahir
Z
Amoura
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
Gaëlle
G
Guettrot-Imbert
AP-HP, Gynécologie, Hôpital Necker, Paris, France.
Delphine
D
Le Mercier
UPMC; Hôpital Tenon, Paris, France.
Sophie
S
Georgin-Lavialle
CHU Fort de France, Martinique, France.
Christophe
C
Deligny
Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Université de Lille 2, Lille, France.
Eric
E
Hachulla
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
Luc
L
Mouthon
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.
Philippe
P
Ravaud
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France.
Elisabeth
E
Villain
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
Damien
D
Bonnet
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: nathalie.costedoat@gmail.com.
Nathalie
N
Costedoat-Chalumeau
D006327:Heart Block / Q000150:complications / Q000151:congenital* / Q000276:immunology / Q000601:surgery; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000151:congenital* / Q000276:immunology; D010138:Pacemaker, Artificial; D019919:Prosthesis Implantation; D016896:Treatment Outcome
Anti-SSA/Ro antibodies; Congenital heart block; Dilated cardiomyopathy; Neonatal lupus erythematosus syndrome; Pregnancy