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DOI: 10.1007/s40556-022-00351-9
Fetal Endocardial Valvular and Supravalvular Calcification as a Manifestation of Anti-Ro/SSA-La/SSB Antibody Mediated Cardiac Phenotype

Abstract
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.
Keywords
Prenatal diagnosis - Fetal calcification - Fetal echocardiography - Neonatal lupus - Anti-Ro and anti-La antibodiesPublication History
Received: 11 February 2022
Accepted: 13 July 2022
Article published online:
04 May 2023
© 2022. Society of Fetal Medicine. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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