@article{7065597ad94c42af85e8eeab3357d3c9,
title = "Systematic review of long QT syndrome identified during fetal life",
abstract = "Fetal long QT syndrome (LQTS) may present with sinus bradycardia, functional 2:1 atrioventricular block (AVB), and ventricular arrhythmias (ventricular tachycardia [VT]/torsades de pointes [TdP]) and lead to fetal or postnatal death. We performed a systematic review and individual participant data meta-analysis of 83 studies reporting outcomes of 265 fetuses for which suspected LQTS was confirmed postnatally and determined risk of adverse perinatal and postnatal outcomes using logistic and stepwise logistic regression. A longer fetal QTc was more predictive of death than any other antenatal factor (receiver operating characteristic [ROC] area under the curve [AUC] 0.85; 95% confidence interval [CI] 0.66–1.00). Risk of death was significantly increased with fetal QTc >600 ms. Neither fetal heart rate nor heart rate z-score predicted death (ROC AUC 0.51; 95% CI 0.31–0.71; and ROC AUC 0.59; 95% CI 0.37–0.80, respectively). The combination of antenatal VT/TdP or functional 2:1 AVB and lack of family history of LQTS was also highly predictive of death (ROC AUC 0.82; 95% CI 0.76–0.88). Our data provide clinical screening tools to enable prediction and intervention for fetuses with LQTS at risk of death.",
keywords = "Fetal, Fetal arrhythmia, Long QT syndrome, Outcomes, Risk factors",
author = "Sian Chivers and Caroline Ovadia and William Regan and Vita Zidere and Trisha Vigneswaran and Gurleen Sharland and Eric Rosenthal and Seed, {Paul T.} and Simpson, {John M.} and Catherine Williamson",
note = "Funding Information: Funding Sources: Dr Chivers was funded by Tommy's Charity and by the NIHR Biomedical Research Centre at Guy's and St Thomas{\textquoteright} NHS Foundation Trust and King's College London. Dr Ovadia was funded by the NIHR Biomedical Research Centre at Guy's and St Thomas{\textquoteright} NHS Foundation Trust and King's College London. Dr Williamson was funded by the NIHR Biomedical Research Centre at Guy's and St Thomas{\textquoteright} NHS Foundation Trust and King's College London. The funders had no role in study design, data collection, data analysis, data interpretation, or writing of the manuscript. Disclosures: Dr Rosenthal reports travel support from BVM Medical. Dr Williamson reports being a consultant to GSK and Mirum Pharmaceuticals (payment made to King's College London). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Funding Information: Funding Sources: Dr Chivers was funded by Tommy{\textquoteright}s Charity and by the NIHR Biomedical Research Centre at Guy{\textquoteright}s and St Thomas{\textquoteright} NHS Foundation Trust and King{\textquoteright}s College London. Dr Ovadia was funded by the NIHR Biomedical Research Centre at Guy{\textquoteright}s and St Thomas{\textquoteright} NHS Foundation Trust and King{\textquoteright}s College London. Dr Williamson was funded by the NIHR Biomedical Research Centre at Guy{\textquoteright}s and St Thomas{\textquoteright} NHS Foundation Trust and King{\textquoteright}s College London. The funders had no role in study design, data collection, data analysis, data interpretation, or writing of the manuscript. Disclosures: Dr Rosenthal reports travel support from BVM Medical. Dr Williamson reports being a consultant to GSK and Mirum Pharmaceuticals (payment made to King{\textquoteright}s College London). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Publisher Copyright: {\textcopyright} 2022 Heart Rhythm Society",
year = "2023",
month = apr,
doi = "10.1016/j.hrthm.2022.12.026",
language = "English",
volume = "20",
pages = "596--606",
journal = "Heart Rhythm",
issn = "1547-5271",
publisher = "Elsevier",
number = "4",
}