Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.
| dc.contributor.author | Moore, Jeremy P | |
| dc.contributor.author | Gallotti, Roberto G | |
| dc.contributor.author | Shannon, Kevin M | |
| dc.contributor.author | Bos, J Martijn | |
| dc.contributor.author | Sadeghi, Elham | |
| dc.contributor.author | Strasburger, Janette F | |
| dc.contributor.author | Wakai, Ronald T | |
| dc.contributor.author | Horigome, Hitoshi | |
| dc.contributor.author | Clur, Sally-Ann | |
| dc.contributor.author | Hill, Allison C | |
| dc.contributor.author | Shah, Maully J | |
| dc.contributor.author | Behere, Shashank | |
| dc.contributor.author | Sarquella-Brugada, Georgia | |
| dc.contributor.author | Czosek, Richard | |
| dc.contributor.author | Etheridge, Susan P | |
| dc.contributor.author | Fischbach, Peter | |
| dc.contributor.author | Kannankeril, Prince J | |
| dc.contributor.author | Motonaga, Kara | |
| dc.contributor.author | Landstrom, Andrew P | |
| dc.contributor.author | Williams, Matthew | |
| dc.contributor.author | Patel, Akash | |
| dc.contributor.author | Dagradi, Federica | |
| dc.contributor.author | Tan, Reina B | |
| dc.contributor.author | Stephenson, Elizabeth | |
| dc.contributor.author | Krishna, Mani Ram | |
| dc.contributor.author | Miyake, Christina Y | |
| dc.contributor.author | Lee, Michelle E | |
| dc.contributor.author | Sanatani, Shubhayan | |
| dc.contributor.author | Balaji, Seshadri | |
| dc.contributor.author | Young, Ming-Lon | |
| dc.contributor.author | Siddiqui, Saad | |
| dc.contributor.author | Schwartz, Peter J | |
| dc.contributor.author | Shivkumar, Kalyanam | |
| dc.contributor.author | Ackerman, Michael J | |
| dc.date.accessioned | 2021-07-01T18:16:20Z | |
| dc.date.available | 2021-07-01T18:16:20Z | |
| dc.date.issued | 2020-11 | |
| dc.date.updated | 2021-07-01T18:16:20Z | |
| dc.description.abstract | ObjectivesThis study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs).BackgroundLQTS 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.MethodsA 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.ResultsA 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).ConclusionsIn 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. | |
| dc.identifier | S2405-500X(20)30482-5 | |
| dc.identifier.issn | 2405-500X | |
| dc.identifier.issn | 2405-5018 | |
| dc.identifier.uri | ||
| dc.language | eng | |
| dc.publisher | Elsevier BV | |
| dc.relation.ispartof | JACC. Clinical electrophysiology | |
| dc.relation.isversionof | 10.1016/j.jacep.2020.06.001 | |
| dc.subject | atrioventricular block | |
| dc.subject | cardiac sympathetic denervation | |
| dc.subject | fetal arrhythmia | |
| dc.subject | fetus | |
| dc.subject | genetic testing | |
| dc.subject | implantable cardioverter-defibrillator | |
| dc.subject | long QT syndrome | |
| dc.subject | magnetocardiography | |
| dc.subject | sudden cardiac death | |
| dc.subject | torsades de pointes | |
| dc.title | Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome. | |
| dc.type | Journal article | |
| duke.contributor.orcid | Landstrom, Andrew P|0000-0002-1878-9631 | |
| pubs.begin-page | 1561 | |
| pubs.end-page | 1570 | |
| pubs.issue | 12 | |
| pubs.organisational-group | School of Medicine | |
| pubs.organisational-group | Cell Biology | |
| pubs.organisational-group | Pediatrics, Cardiology | |
| pubs.organisational-group | Duke | |
| pubs.organisational-group | Basic Science Departments | |
| pubs.organisational-group | Pediatrics | |
| pubs.organisational-group | Clinical Science Departments | |
| pubs.publication-status | Published | |
| pubs.volume | 6 |
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