Format

Send to

Choose Destination
Autoimmun Rev. 2015 Dec;14(12):1154-60. doi: 10.1016/j.autrev.2015.08.005. Epub 2015 Aug 15.

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

Author information

1
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
2
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France.
3
AP-HP, Hôpital Hôtel-Dieu, Centre d'Epidémiologie Clinique, Paris; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
4
Service de Médecine Interne, CHU Nantes, France.
5
CHU de Reims, Centre de compétence maladies auto-immunes et systémiques rares, Reims, France.
6
UPMC; AP-HP, Hôpital Pitié-Salpêtrière, Centre de référence maladies auto-immunes et systémiques rares, Paris, France.
7
Réanimation Pédiatrique AP-HM, CHU Timone Enfants, Marseille, France.
8
UPMC; AP-HP, Hôpital Saint Antoine, Paris, France.
9
Rhumatologie, Centre de référence maladies auto-immunes et systémiques rares, Strasbourg, France.
10
Hôpital Louis Pradel, Lyon, France.
11
CHU, Angers, France.
12
AP-HP, Gynécologie, Hôpital Necker, Paris, France.
13
UPMC; Hôpital Tenon, Paris, France.
14
CHU Fort de France, Martinique, France.
15
Centre de référence maladies auto-immunes et systémiques rares, Service de Médecine Interne, Université de Lille 2, Lille, France.
16
AP-HP, Hôpital Cochin, Centre de référence maladies auto-immunes et systémiques rares, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
17
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.
18
APHP, Centre de référence Malformations Cardiaques Congénitales Complexes-M3C, Hôpital Necker, Paris, France; Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
19
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.

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.

KEYWORDS:

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

PMID:
26284740
DOI:
10.1016/j.autrev.2015.08.005
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center