Arrhythmias & electrophysiologyCohort study

Effects of cohort, genotype, variant, and maternal β-blocker treatment on foetal heart rate predictors of inherited long QT syndrome

EuropacePublished

  • Cohort study
  • international

Authors' conclusion

Genotype, LQT1 variant, and maternal β-blocker therapy affect FHR. A predictive threshold of FHR/GA significantly improves the accuracy, sensitivity, and specificity for LQT1 and LQT2, above the infant's a priori 50% probability.

Study context

Population
Pregnancies in which one parent had long QT syndrome (LQTS)164 LQTS families
Participants
267 pregnancies
Exposure
Foetal heart rate (FHR) by LQTS genotype, variant and maternal β-blocker therapy
  • LQT1n = 220
  • LQT2n = 35
  • LQT3n = 12
Outcome
FHR by genotype, variant and maternal β-blocker therapy; FHR/gestational age (GA) threshold for LQTS
Follow-up
7-42 weeks' GA

Main finding

As reported in the abstract

FHR
  • FHR significantly lower in LQT1 and LQT2 but not LQT3 or LQTS negative
  • LQT1 variants with non-nonsense and severe function loss had lower FHR
  • Maternal β-blockers potentiated bradycardia in LQT1 and LQT2 but did not affect FHR in LQTS negative

Observational data: associations, not effects.

Further findings

OutcomeValues
FHR/GA threshold for predicting LQT1 and LQT2FHR/GA threshold accuracy 74.9% · sensitivity 71% · specificity 81%

Original publication

Effects of cohort, genotype, variant, and maternal β-blocker treatment on foetal heart rate predictors of inherited long QT syndrome. Europace. Published 17 November 2023. doi:10.1093/europace/euad319

Summarised from the published abstract; every number is checked against it