Background/Purpose: Life-threatening cardiac manifestations of neonatal lupus (cardiac-NL) include complete block, endocardial fibroelastosis (EFE) and dilated cardiomyopathy (DCM), all supportive of intense fibrosis at the AV node and beyond. The overall case fatality rate is 17.5% but ranges from 7.8% for isolated block to 46% when the block is accompanied by more extensive disease. Both prevention and treatment with fluorinated steroids (FS) have been considered but results are inconclusive and even conflicting, a disconcerting situation given the potential for maternal toxicity. Accordingly, we reviewed the records from the U.S. Research Registry for Neonatal Lupus (RRNL), and the French and U.K. Registries to ascertain whether the use of FS conferred a survival benefit for fetuses with cardiac-NL or prevented the recurrence of cardiac-NL.

Methods: Data from the RRNL were analyzed to determine whether the use of FS affected survival at six months. Isolated third degree block and risk factors associated with a poor prognosis for cardiac-NL (HR<50, DCM and EFE) were evaluated individually or in combination given the likelihood of more than one poor prognostic factor being present. The effect of FS on survival once hydrops was detected was also addressed. The efficacy of prophylactic FS was assessed in an international historical cohort.

Results: In 276 cases of cardiac-NL, sufficient data were available regarding medications used during pregnancy; 150 were treated with FS. Not unexpectedly, FS were most often used when disease extended beyond the AV node. Neither maternal race/ethnicity nor health status influenced the use of FS. In isolated 3^rddegree block, 2.6% (2/78) died by 6 months postpartum despite the use of FS compared to 0/74, in those never given FS, p=.50. In those with HR < 50 bpm, 0 of 25 exposed to FS died compared to 0 of 18 unexposed to FS. Of the 12 cases with EFE (present or absent block) 11 received FS and only 1 died (9.9%) which was an elective termination. In cases where DCM was present (with or without block) 25% (1/4) died by 6 months postpartum despite the use of FS compared to 20.0% (1/5) never given FS. In cases with ≥ 2 risk factors, 16.7% (1/6) exposed to FS died by 6 months postpartum compared to 33.3% (2/6) unexposed. In fetuses with hydrops, 55.6% (15/27) receiving FS died compared to 81.8% (9/11) who did not receive FS, p=.16. When terbutaline was added to FS for hydrops, 50.0% (5/10) died compared to 58.8% (10/17) not receiving terbutaline. With regard to prophylaxis, recurrent cardiac-NL occurred in 14.3% (2/14) of pregnancies of mothers given FS compared to 19.3% (47/257) in those not treated with FS, p=.58.

Conclusion: These data suggest that fetuses with isolated 3^rd degree block, even those who develop severe bradycardia in absence of other risk factors, do well and the addition of FS does not improve the 6 month survival rate. FS may be beneficial in cardiac-NL cases which develop hydrops or have multiple poor prognostic factors. Available evidence does not support the use of FS to reduce the recurrence rate of cardiac-NL.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/critical-management-decisions-in-cardiac-neonatal-lupus-the-role-of-fluorinated-steroids/