Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.
Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.
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基因型预测胎儿和新生儿的结局，患有严重的先天性QT综合征。
DOI：
10.1016/j.jacep.2020.06.001
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发表时间：
2020-11
期刊：
影响因子：
--
通讯作者：
Ackerman MJ
中科院分区：
文献类型：
--
作者：
Moore JP;Gallotti RG;Shannon KM;Bos JM;Sadeghi E;Strasburger JF;Wakai RT;Horigome H;Clur SA;Hill AC;Shah MJ;Behere S;Sarquella-Brugada G;Czosek R;Etheridge SP;Fischbach P;Kannankeril PJ;Motonaga K;Landstrom AP;Williams M;Patel A;Dagradi F;Tan RB;Stephenson E;Krishna MR;Miyake CY;Lee ME;Sanatani S;Balaji S;Young ML;Siddiqui S;Schwartz PJ;Shivkumar K;Ackerman MJ
探讨LQTS亚型与出生后心脏事件(CES)的关系。在胎儿/新生儿出现2：1房室传导阻滞或尖端扭矩(TDP)的LQTS与重大CES的风险相关，但总体结果和预测因素尚不清楚。一项涉及25个国际中心的回顾性研究评估了被诊断为先天性LQTS和2：1房室传导阻滞或TDP的胎儿/新生儿的病程。主要结果是新生儿出院后首次CE的年龄和随访期间的死亡/心脏移植。CE定义为心脏骤停(ACA)、适当ICD休克(AS)或心脏性猝死(SCD)。共检出84个胎儿/新生儿，其中LQT1 12例，LQT2 35例，LQT3 37例。分娩时胎龄中位数为37周(IQR35-39)，出院时胎龄中位数为3周(IQR2-5)。死亡2例，出院前死亡1例。中位时间5.2年，LQT1 1例，LQT2 3例，LQT3CE23例(ACA 13例，SCD 5例，AS 9例)。1例LQT1患者和11例LQT3患者在随访期间死亡或接受心脏移植。出院后CES的唯一多变量预测因子是LQT3状态(LQT3vs LQT2，HR8.4[CI2.6-38.9]，p<；0.001)，LQT3型相对于LQT2预测死亡/心脏移植(p<；0.001)。在这项大型多中心研究中，LQT3而不是LQT1或LQT2出现严重心律失常的胎儿/新生儿不仅频繁，而且致命的CES的风险很高。表现为2：1房室传导阻滞或尖端扭转肌(TDP)的长QT综合征(LQTS)的胎儿和新生儿的预后尚不清楚。来自25个国际中心的84个受影响的胎儿/新生儿被确认。在中位数为5.2年的时间里，有27例出院后CES。CES的唯一多变量预测因子是LQT3状态(p<；0.001)。此外，在最后一次随访时，LQT3型可预测死亡/心脏移植(p<；0.001)。这些数据表明，患有2：1房室传导阻滞或TDP的LQT3胎儿/新生儿是未来CES的最高风险者，他们不仅经常发生CES，而且还会发生致命的CES。
To determine the relationship between LQTS subtype and postnatal cardiac events (CEs). LQTS presenting with 2:1 atrioventricular (AV) block or torsades de pointes (TdP) in the fetus/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 AV block or TdP. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death/cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest (ACA), appropriate ICD shock (AS), or sudden cardiac death (SCD). Eighty-four fetuses/neonates were identified (12 LQT1, 35 LQT2, 37 LQT3). Median gestational age at delivery was 37 weeks (IQR 35 – 39) and age at hospital discharge was 3 weeks (IQR 2 – 5). 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 ACA, 5 SCD, and 9 AS). One LQT1 patient and 11 LQT3 patients died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs LQT2, HR 8.4 [CI 2.6 – 38.9], p<0.001) and LQT3 genotype predicted death/cardiac transplant relative to LQT2 (p<0.001). In this large multicenter study, LQT3 but not LQT1 or LQT2 fetuses/neonates presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs. Outcomes of fetuses and neonates with long QT syndrome (LQTS) presenting with 2:1 atrioventricular (AV) block or torsades de pointes (TdP) are unknown. Eighty-four affected fetuses/neonates were identified from 25 international centers. Over a median of 5.2 years, there were 27 post-discharge CEs. The only multivariate predictor of CEs was LQT3 status (p<0.001). Moreover, LQT3 genotype predicted death/cardiac transplant at last follow-up (p<0.001). These data suggest LQT3 fetuses/neonates with 2:1 AV block or TdP are at the highest risk for future CEs and experience not only frequent, but lethal CEs.
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影响因子：
24
作者：
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