Fetal Endocardial Valvular and Supravalvular Calcification as a Manifestation of Anti-Ro/SSA-La/SSB Antibody Mediated Cardiac Phenotype

Neonatal Lupus syndrome is caused by the placental transfer of maternal autoantibodies to the fetus. Maternal anti-SSA/Ro-SSB/La antibodies may result in congenital heart block in 1–2% of exposed fetuses. However, fetal valvular calcification is rarely reported. We report a 20 weeks fetus with multiple intracardiac calcifications, valvular calcification in the pulmonary valve and supravalvular calcification at the origin of the main pulmonary artery along with calcification in the chordae of mitral and tricuspid valve in a structurally normal heart. The mother was asymptomatic but strongly positive for anti-SSA/Ro-SSB/La antibodies. Fetal cardiac valvular calcification is an uncommon phenotype of neonatal lupus syndrome. It is important to suspect, recognize and screen for maternal autoimmune disorders even in the absence of congenital heart block.

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