Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system

Abstract

Objectives: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit.

Methods: In this retrospective study of cases enrolled in the Research Registry for NL, inclusion was restricted to anti-SSA/Ro-exposed cases presenting with isolated advanced heart block in utero who either received fluorinated steroids within 1 week of detection (N=71) or no treatment (N=85). Outcomes evaluated were: development of endocardial fibroelastosis, dilated cardiomyopathy and/or hydrops fetalis; mortality and pacemaker implantation.

Results: In Cox proportional hazards regression analyses, fluorinated steroids did not significantly prevent development of disease beyond the AV node (adjusted HR=0.90; 95% CI 0.43 to 1.85; p=0.77), reduce mortality (HR=1.63; 95% CI 0.43 to 6.14; p=0.47) or forestall/prevent pacemaker implantation (HR=0.87; 95% CI 0.57 to 1.33; p=0.53). No risk factors for development of disease beyond the AV node were identified.

Conclusions: These data do not provide evidence to support the use of fluorinated steroids to prevent disease progression or death in cases presenting with isolated heart block.

Keywords: Autoantibodies; Cardiovascular Disease; Corticosteroids; Outcomes research.

Figure 1

Flow diagram of assessment, inclusion, exclusion and analysis of study subjects. FS, fluorinated steroids; NL, neonatal lupus; RRNL, Research Registry for Neonatal Lupus.

Figure 2

Kaplan–Meier curves reflecting the probability of: (A) Developing disease beyond the atrioventricular (AV) node in cases that presented with isolated advanced block. Solid line=no fluorinated steroid treatment (N=85); dashed line=fluorinated steroid treatment (N=71). The X axis represents weeks from conception. (B) Survival for cases presenting with isolated advanced block. Solid line=no fluorinated steroid treatment (N=80); dashed line=fluorinated steroid treatment (N=71). The X axis represents weeks from conception. Of note, the five cases who received fluorinated steroids at the time of diagnosis of extranodal disease were excluded from this analysis. (C) Pacemaker implantation. Solid line=no fluorinated steroid treatment (N=78); dashed line=fluorinated steroid treatment (N=64). The X axis represents weeks from birth. Of note, the five cases who received fluorinated steroids at the time of diagnosis of extranodal disease were excluded from this analysis. Additional four cases that died in utero and five cases in which pacing data were either unavailable (N=2) or the exact timing of pacemaker implantation was unknown (N=3) were also removed.