Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose:
Cardiac neonatal lupus manifestations mainly include congenital heart block (CHB), endocardial fibroelastosis and dilated cardiomyopathy. We report the preliminary results of the French registry of neonatal lupus.
Methods:
This French registry was established in 2000 and includes foetuses or children with neonatal lupus, born to mothers with anti-SSA or/and anti-SSB antibodies. This database has Institutional Review Board approval. Here, we report data on CHB.
Results:
141 cases of CHB born to 124 mothers were included. When the first CHB was diagnosed, 45 mothers (36 %) had an autoimmune disease: 16 had systemic lupus erythematosus (1 with an antiphospholipid syndrome), 15 had Sjögren syndrome, and 14 had another connective tissue disease (CTD). After a median follow-up period of 5.6 years, [0.03-36.5], 85 women (60%) had a diagnosis of autoimmune disease (Sjögren syndrome in 33, systemic lupus erythematosus in 32, and other CTD in 20 ).
At the time of the CHB diagnosis, 24 (17%) of the pregnant women were treated with corticosteroids, 13 (9.2%) with hydroxychloroquine, and 21 (14.9%) with acetylsalicylic acid. The median term at diagnosis of CHB was 22 WG [16-37 WG]. Twenty-two foetuses (15.6%) were also diagnosed with endocardial fibroelastosis. Among the 141 fetuses with CHB, there were 9 intrauterine deaths, 10 elective terminations of pregnancy, and 122 (86.5%) children born alive at a median term of 37 WG [28-40]. After a median follow-up period of 5.6 years, [0.03-36.5], 11 children (9%) had died. Three died in the neonatal period (2 from complication of CHB and one from prematurity) and 8 later on at a median age of 10 months [2-60]. Of those 8 children, 7 deaths were attributed to a cardiomyopathy associated with CHB, and one to a nosocomial infection.
Ninety five children (77.8 %) had a pacemaker, implanted at a median age of 3.7 months [0.01-14.4]. Fifteen children (12.3%) developed a cardiomyopathy requiring a medical treatment and 9 of those 15 children died from complications of this cardiomyopathy. There was no cardiac transplantation.
After a first pregnancy complicated with a CHB, 57 women had a total of 84 subsequent pregnancies. The following pregnancies were complicated by a CHB in 20.2% of cases (n=17). There were 14 cases of CHB in the 52 pregnancies non-exposed to hydroxychloroquine (26.9%) versus 3 cases in the 32 pregnancies exposed to hydroxychloroquine (9.4%; p=0.052).
Conclusion:
87% of foetuses diagnosed with CHB were alive at birth, and 9% died during a median follow up of 5.6 years. A pacemaker was inserted in 77.8% of the cases. Our data confirm that the use of hydroxychloroquine may protect against recurrence of CHB in a subsequent pregnancy (Izmirly et al, Circulation. 2012 May 24. [Epub ahead of print]*). An international prospective study is ongoing to confirm this point (PATCH study; ClinicalTrials.gov Identifier: NCT01379573).
Disclosure:
K. Levesque,
None;
A. Maltret,
None;
M. Hamidou,
None;
M. Jallouli,
None;
J. L. Pennaforte,
None;
P. Orquevaux,
None;
J. C. Piette,
None;
Z. Amoura,
None;
F. Barriere,
None;
J. Le Bidois,
None;
L. Fermont,
None;
L. Cohen,
None;
O. Meyer,
None;
O. Fain,
None;
A. Theulin,
None;
H. Lucron,
None;
F. Sassolas,
None;
H. Bezanahary,
None;
G. Guettrot-Imbert,
None;
P. Seve,
None;
E. Diot,
None;
N. Morel,
None;
C. Deligny,
None;
E. Villain,
None;
N. Costedoat-Chalumeau,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/french-cohort-study-of-141-cases-of-autoimmune-congenital-heart-block/