Background/Purpose: Cardiac Neonatal Lupus (NL), characterized by inflammation and fibrosis of the conduction system, endocardium, and myocardium in anti-Ro exposed fetuses, results in significant morbidity and mortality.  There is considerable debate regarding the efficacy of maternal fluorinated steroids (FS) in cardiac NL.  While permanent reversal of complete block has not been reported, less advanced blocks have regressed and 6 month mortality appears decreased if fetal disease was associated with a more global cardiomyopathy.  Long term information regarding the cardiac health of affected children is limited.  Accordingly, substantive data on prenatal exposure to FS and outcomes are critical to decision making. 

Methods: 161 individuals with cardiac NL or their mothers enrolled in the Research Registry for Neonatal Lupus responded to a questionnaire regarding current treatment with cardiac medications (beta blockers, ACE inhibitors, and/or digoxin) and overall cardiac status.  Data were analyzed to determine whether fetal echocardiographic evidence of endocardial fibroelastosis (EFE), dilated cardiomyopathy (DCM) or hydrops, and the use and cumulative dosage (dexamethasone equivalent) of FS during pregnancy associated with long term morbidity.

Results: Cardiac meds were required in 3/14 (21.4%) affected children ages 0-2, 3/25 (12%) ages >2-5, 2/35 (5.7%) ages >5-10, 2/20 (10%) ages >10-15, 4/31 (12.9%) ages >15-20, and 8/36 (22.2%) >20 years.  Digoxin was used by 6 individuals, ACE inhibitors by 18, Beta Blockers by 13, and systemic hypertension was diagnosed in 3.  The age ranges 0-2 and >20 years were significantly associated with taking cardiac meds (p=0.048).  Thirty four (21.1%) had ever been told by a doctor that they had congestive heart failure, and 68 (42.2%) were told of an enlarged heart.  Those with a history of an enlarged heart were significantly older at the time of the questionnaire (15.2 ± 9.75 years vs. 10.8 ± 8.03, p=0.011).  Fetal echo evidence of EFE, DCM, or hydrops vs. 3^rd degree block alone was associated with the future requirement for cardiac meds (p=0.039).  Eighty two (50.9%) were exposed to FS in utero, with cumulative dose ranging from 12 – 693 mg of dexamethasone.  Exposure to FS and cumulative dose did not associate with a decreased future need of cardiac meds, development of CHF, or an enlarged heart.  The absence of an association between in utero exposure to steroids and overall cardiac well being was observed for both those children with isolated 3^rd degree block and those with fetal echo documentation of EFE, DCM, or hydrops.

Conclusion: Medical therapy for cardiac NL is more frequently required shortly after birth, perhaps in order to treat the continuing primary insult which developed in utero.  Increased use of cardiac meds in those over 20 and the reporting of an enlarged heart in older individuals suggests the possibility of decreased heart function as children age, perhaps due to worsening disease or the effects of long term pacing.  FS exposure in utero did not associate with overall better cardiac outcomes. In those with isolated 3^rd degree block, there is no long term benefit to in utero treatment with fluorinated steroids.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prenatal-exposure-to-fluorinated-steroids-does-not-affect-long-term-morbidity-in-cardiac-neonatal-lupus/