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

Desai, L.; Wakai, R.; Tsao, S.; Strasburger, J.; Gotteiner, N.; Patel, A.

Pacing Clin Electrophysiol. 2020 Mar 14; 43(4):430-433

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.

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