Systematic review of long QT syndrome identified during fetal life

Sian Chivers, Caroline Ovadia, William Regan, Vita Zidere, Trisha Vigneswaran, Gurleen Sharland, Eric Rosenthal, Paul T. Seed, John M. Simpson*, Catherine Williamson

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Pages (from-to)596-606
Number of pages11
JournalHeart Rhythm
Issue number4
Publication statusPublished - Apr 2023


  • Fetal
  • Fetal arrhythmia
  • Long QT syndrome
  • Outcomes
  • Risk factors


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