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Mothers with long QT syndrome are at increased risk for fetal death: Findings from a multicenter international study

American Journal of Obstetrics and Gynecology Dec 07, 2019

Cuneo BF, et al. - Researchers examined the fetal death risk in familial long QT syndrome, in which the mother or father carries the long QT syndrome genotype and ascertained if risk differed in case of inheriting the long QT syndrome genotype from the mother or father. They conducted a multicenter observational study of case series of 148 pregnancies from 103 families (80 mothers, 23 fathers) with familial long QT syndrome [60 with long QT syndrome genotypes in KCNQ1, 29 with long QT syndrome genotypes in KCNH2, 14 with long QT syndrome genotypes in SCN5A] recruited from 11 international centers with expertise in hereditary heart rhythm diseases, pediatric and/or adult electrophysiology, and high-risk pregnancies. Most offspring (80%; 118/148) were liveborn at term; long QT syndrome was reported in 66% of offspring (73/110). In this report, for the first time, it was inscribed that mothers with long QT syndrome are at heightened risk of fetal death and uncovered a previously unreported cause of stillbirth. Results here infer that maternal effects of long QT syndrome channelopathy may lead to placental or myometrial dysfunction that confers heightened susceptibility to fetal death and growth restriction in newborn survivors; this was observed irrespective of long QT syndrome status.
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