ArticleCirculation. Arrhythmia and electrophysiology2025
Fetal Bradycardia Prompting the Diagnosis and Management of Parental Long QT Syndrome.
Article in Circulation. Arrhythmia and electrophysiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundLong QT syndrome (LQTS) is primarily an inherited condition associated with the risk of sudden cardiac death. Due to variable phenotypic expression, a prolonged QT interval on a 12-lead ECG is not always present. LQTS may present in the fetus with persistent bradycardia, including sinus bradycardia or functional 2:1 atrioventricular block. We report our experience of persistent fetal bradycardia prompting parental assessment for congenital LQTS.
methodsFrom January 1, 2018 to November 1, 2023, 20 parents (20 mothers; 20 fathers) of fetuses presenting with persistent bradycardia and suspected congenital LQTS were assessed. Autoimmune-mediated atrioventricular block, diagnosed in the presence of maternal anti-Ro/anti-La antibodies, and fetuses with ventricular tachycardia were excluded. Parental ECGs were acquired in the remainder, with comprehensive evaluation, including genomic testing, performed in 12 mothers and 11 fathers.
resultsAmong 20 fetuses, 16 had sinus bradycardia and 4 had 2:1 atrioventricular block (intermittent=2; persistent=2). Pathogenic LQTS genetic variants were found in 11 fetuses (
conclusionsThe first indication of parental LQTS may be persistent fetal bradycardia. This should prompt consideration of this diagnosis even with a normal maternal corrected QT interval and lead to the initiation of specific management strategies for pregnancy, delivery, and the postpartum period before the results of genomic testing are available.
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