TY - JOUR
T1 - Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome
AU - Moore, Jeremy P.
AU - Gallotti, Roberto G.
AU - Shannon, Kevin M.
AU - Bos, J. Martijn
AU - Sadeghi, Elham
AU - Strasburger, Janette F.
AU - Wakai, Ronald T.
AU - Horigome, Hitoshi
AU - Clur, Sally Ann
AU - Hill, Allison C.
AU - Shah, Maully J.
AU - Behere, Shashank
AU - Sarquella-Brugada, Georgia
AU - Czosek, Richard
AU - Etheridge, Susan P.
AU - Fischbach, Peter
AU - Kannankeril, Prince J.
AU - Motonaga, Kara
AU - Landstrom, Andrew P.
AU - Williams, Matthew
AU - Patel, Akash
AU - Dagradi, Federica
AU - Tan, Reina B.
AU - Stephenson, Elizabeth
AU - Krishna, Mani Ram
AU - Miyake, Christina Y.
AU - Lee, Michelle E.
AU - Sanatani, Shubhayan
AU - Balaji, Seshadri
AU - Young, Ming Lon
AU - Siddiqui, Saad
AU - Schwartz, Peter J.
AU - Shivkumar, Kalyanam
AU - Ackerman, Michael J.
N1 - Publisher Copyright:
© 2020
PY - 2020/11
Y1 - 2020/11
N2 - Objectives: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs). Background: 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. Methods: 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. Results: 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 cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001). Conclusions: In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs.
AB - Objectives: This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs). Background: 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. Methods: 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. Results: 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 cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001). Conclusions: In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs.
KW - atrioventricular block
KW - cardiac sympathetic denervation
KW - fetal arrhythmia
KW - fetus
KW - genetic testing
KW - implantable cardioverter-defibrillator
KW - long QT syndrome
KW - magnetocardiography
KW - sudden cardiac death
KW - torsades de pointes
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U2 - 10.1016/j.jacep.2020.06.001
DO - 10.1016/j.jacep.2020.06.001
M3 - Article
C2 - 33213816
AN - SCOPUS:85090485030
SN - 2405-500X
VL - 6
SP - 1561
EP - 1570
JO - JACC: Clinical Electrophysiology
JF - JACC: Clinical Electrophysiology
IS - 12
ER -