Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.

JACC. Clinical Electrophysiology
Jeremy P MooreMichael J Ackerman

Abstract

This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs). LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown. A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death and/or cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest, appropriate shock from implantable cardioverter-defibrillator, or sudden cardiac death. A total of 84 fetuses and/or neonates were identified with LQTS (12 as LQT1, 35 as LQT2, 37 as LQT3). Median gestational age at delivery was 37 weeks (interquartile range: 35 to 39 weeks) and age at hospital discharge was 3 weeks (interquartile range: 2 to 5 weeks). Fetal demise occurred in 2 and pre-discharge death in 1. Over a median of 5.2 years, there were 1 LQT1, 3 LQT2, and 23 LQT3 CEs (13 aborted cardiac arrests, 5 sudden card...Continue Reading

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Citations

Aug 8, 2021·Heart Rhythm : the Official Journal of the Heart Rhythm Society·UNKNOWN Writing Committee MembersPhilip L Wackel
Aug 2, 2021·Indian Pacing and Electrophysiology Journal·UNKNOWN Writing Committee MembersUNKNOWN Document Reviewers
Aug 3, 2021·Cardiology in the Young·Maully J ShahUNKNOWN Document Reviewers: Philip M. Chang, Fabrizio Drago, Anne M. Dubin, Susan P. Etheridge, Apichai Kongpatanayothin, Jose Manue

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