TY - JOUR
T1 - Assessment of Severity of Long QT Syndrome Phenotype and Risk of Fetal Death
AU - Albertini, Lisa
AU - Ezekian, Jordan
AU - Care, Melanie
AU - Silversides, Candice
AU - Sermer, Mathew
AU - Gollob, Michael H.
AU - Spears, Danna
N1 - Publisher Copyright:
© 2023 The Authors.
PY - 2023
Y1 - 2023
N2 - BACKGROUND: It has been postulated that long QT syndrome (LQTS) can cause fetal loss through putative adverse effects of the channelopathy on placenta and myometrial function. The authors aimed to describe the fetal death rate in a population of pregnant women with long QT syndrome and investigate whether women with more severe phenotype had worse fetal outcomes. METHODS AND RESULTS: The authors retrospectively evaluated fetal outcomes of 64 pregnancies from 23 women with long QT syndrome followed during pregnancy in a tertiary pregnancy and heart disease program. Thirteen of 64 pregnancies (20%) resulted in a fetal loss, 12 miscarriages (19%), and 1 stillbirth (1.6%). Baseline maternal characteristics, including age and use of β-blockers, did not differ between women who experienced a fetal death or not. Maternal corrected QT interval (QTc) was significantly longer in pregnancies that resulted in fetal death compared with live births (median, 518 ms [interquartile range (IQR), 482-519 ms] versus 479 ms [IQR, 454–496 ms], P<0.001). Mothers treated with β-blockers had babies born at term with lower birth weight compared with untreated women (2973±298 g versus 3470±338 g, P=0.002). In addition, the birth weight of babies born at term to treated women with QTc >500 ms was significantly lower compared with women with QTc <500 ms (2783±283 g versus 3084±256 g, P=0.029). CONCLUSIONS: Women with long QT syndrome with more severe phenotypes have a higher incidence of fetal death. Maternal QTc is longer in pregnancies that result in fetal loss, and the birth weight of babies born to patients taking β-blockers with a QTc >500 ms is lower, suggesting that patients with more marked phenotype may experience worse fetal outcomes.
AB - BACKGROUND: It has been postulated that long QT syndrome (LQTS) can cause fetal loss through putative adverse effects of the channelopathy on placenta and myometrial function. The authors aimed to describe the fetal death rate in a population of pregnant women with long QT syndrome and investigate whether women with more severe phenotype had worse fetal outcomes. METHODS AND RESULTS: The authors retrospectively evaluated fetal outcomes of 64 pregnancies from 23 women with long QT syndrome followed during pregnancy in a tertiary pregnancy and heart disease program. Thirteen of 64 pregnancies (20%) resulted in a fetal loss, 12 miscarriages (19%), and 1 stillbirth (1.6%). Baseline maternal characteristics, including age and use of β-blockers, did not differ between women who experienced a fetal death or not. Maternal corrected QT interval (QTc) was significantly longer in pregnancies that resulted in fetal death compared with live births (median, 518 ms [interquartile range (IQR), 482-519 ms] versus 479 ms [IQR, 454–496 ms], P<0.001). Mothers treated with β-blockers had babies born at term with lower birth weight compared with untreated women (2973±298 g versus 3470±338 g, P=0.002). In addition, the birth weight of babies born at term to treated women with QTc >500 ms was significantly lower compared with women with QTc <500 ms (2783±283 g versus 3084±256 g, P=0.029). CONCLUSIONS: Women with long QT syndrome with more severe phenotypes have a higher incidence of fetal death. Maternal QTc is longer in pregnancies that result in fetal loss, and the birth weight of babies born to patients taking β-blockers with a QTc >500 ms is lower, suggesting that patients with more marked phenotype may experience worse fetal outcomes.
KW - fetal death
KW - long QT
KW - long QT syndrome
KW - miscarriage
KW - stillbirth
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U2 - 10.1161/JAHA.122.029407
DO - 10.1161/JAHA.122.029407
M3 - Article
C2 - 38014677
AN - SCOPUS:85179004471
SN - 2047-9980
VL - 12
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
IS - 23
M1 - e029407
ER -