An expanded phenotype of maternal SSA/SSB antibody-associated fetal cardiac disease.

OBJECTIVES

Conventional manifestations of fetal Sjogren's antibodies (SSA/SSB) associated cardiac disease include atrioventricular block (AVB), transient sinus bradycardia, endocardial fibroelastosis (EFE) and dilated cardiomyopathy. We describe other manifestations of cardiac disease.

METHODS

We describe three fetuses with unique myocardial and conduction system disease.

RESULTS

One had isolated EFE with subsequent mitral and tricuspid valve chordal avulsion, the second had sinoatrial and infrahissian conduction system disease, and in both, neonatal progression to life threatening disease occurred. The third had sinus node dysfunction and atrial flutter.

CONCLUSION

These findings expand the clinical phenotype of maternal SSA/SSB antibody associated fetal cardiac disease.