Fetal diagnosis of KCNQ1-variant long QT syndrome using fetal echocardiography and magnetocardiography

Pacing Clin Electrophysiol. 2020 Apr;43(4):430-433. doi: 10.1111/pace.13900. Epub 2020 Apr 7.

Abstract

A pregnant woman with KCNQ1 variant long QT syndrome (LQTS) underwent fetal magnetocardiography (fMCG) after atrioventricular (AV) block was noted during fetal echocardiogram-atypical for LQTS type 1. Concern for fetal LQTS on fMCG prompted monitoring of maternal labs, change of maternal beta blocker therapy, and frequent fetal echocardiograms. Collaboration between obstetricians, neonatologists, and pediatric cardiologists ensured safe delivery. Beta blocker therapy was initiated after birth, and postnatal evaluation confirmed genotype and phenotype positive LQTS in the infant. Our experience suggests diagnosis and evaluation of fetal LQTS can alter antenatal management to reduce risk of poor fetal and postnatal outcomes.

Keywords: fetal bradycardia; fetal echocardiography; fetal magnetocardiography; long QT syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Echocardiography*
  • Female
  • Humans
  • KCNQ1 Potassium Channel / genetics
  • Long QT Syndrome / diagnosis*
  • Long QT Syndrome / genetics
  • Magnetocardiography*
  • Pregnancy
  • Prenatal Diagnosis / methods*

Substances

  • KCNQ1 Potassium Channel
  • KCNQ1 protein, human