窦性心动过缓
医学
心动过缓
长QT综合征
内科学
尖端扭转
心脏病学
QT间期
房室传导阻滞
心脏传导阻滞
心率
胎儿
窦性心律
胎龄
胎儿超声心动图
麻醉
心电图
产前诊断
怀孕
心房颤动
生物
血压
遗传学
作者
Bettina F. Cuneo,Susan P. Etheridge,Hitoshi Horigome,Denver Sallee,Anita J. Moon-Grady,Hsin‐Yi Weng,Michael J. Ackerman,D. Woodrow Benson
出处
期刊:Circulation-arrhythmia and Electrophysiology
[Ovid Technologies (Wolters Kluwer)]
日期:2013-10-01
卷期号:6 (5): 946-951
被引量:55
标识
DOI:10.1161/circep.113.000618
摘要
Background— Fetal arrhythmias characteristic of long QT syndrome (LQTS) include torsades de pointes (TdP) and/or 2° atrioventricular block, but sinus bradycardia, defined as fetal heart rate <3% for gestational age, is most common. We hypothesized that prenatal rhythm phenotype might predict LQTS genotype and facilitate improved risk stratification and management. Method and Results— Records of subjects exhibiting fetal LQTS arrhythmias were reviewed. Fetal echocardiograms, neonatal ECG, and genetic testing were evaluated. We studied 43 subjects exhibiting fetal LQTS arrhythmias: TdP±2° atrioventricular block (group 1, n=7), isolated 2° atrioventricular block (group 2, n=4), and sinus bradycardia (group 3, n=32). Mutations in known LQTS genes were found in 95% of subjects tested. SCN5A mutations occurred in 71% of group 1, whereas 91% of subjects with KCNQ1 mutations were in group 3. Small numbers of subjects with KCNH2 mutations (n=4) were scattered in all 3 groups. Age at presentation did not differ among groups, and most subjects (n=42) were live-born with gestational ages of 37.5±2.8 weeks (mean±SD). However, those with TdP were typically delivered earlier. Prenatal treatment in group 1 terminated (n=2) or improved (n=4) TdP. The neonatal heart rate–corrected QT interval (mean±SE) of group 1 (664.7±24.9) was longer than neonatal heart rate–corrected QT interval in both group 2 (491.2±27.6; P =0.004) and group 3 (483.1±13.7; P <0.001). Despite medical and pacemaker therapy, postnatal cardiac arrest (n=4) or sudden death (n=1) was common among subjects with fetal/neonatal TdP. Conclusions— Rhythm phenotypes of fetal LQTS have genotype-suggestive features that, along with heart rate–corrected QT interval duration, may risk stratify perinatal management.
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