A rare case with fetal autoimmune heart block and KNCH2 variant-induced long QT syndrome: a controversial opinion on prenatal management strategy.

ABSTRACT:

Background

Among all fetal heart block patients, > 50% cases are associated with maternal autoimmune diseases, and such patients should receive treatment. However, nearly half of fetal heart block cases involve a mother with negative results following autoimmune antibody screening. A few studies have reported long QT syndrome (LQTS) can also present as a severe fetal bradycardia, which does not respond to fetal treatment. Herein, we reported a rare case of an infant who presented with high-degree autoimmune-mediated fetal atrioventricular block (AVB) with LQTS induced by a novel KCNH2 variant. This case led us to review our prenatal therapeutic strategy.

Case presentation

A 1-year-old boy presented to our heart center having experienced syncope 5 times in the past year. He h

SUBMITTER: Wei L 

PROVIDER: S-EPMC10044358 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature