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Fetal diagnosis of KCNQ1-variant long QT syndrome using fetal echocardiography and magnetocardiography.
Pacing and Clinical Electrophysiology ( IF 1.8 ) Pub Date : 2020-04-07 , DOI: 10.1111/pace.13900
Lajja Desai 1 , Ron Wakai 2 , Sabrina Tsao 3 , Janette Strasburger 4 , Nina Gotteiner 1 , Angira Patel 1
Affiliation  

A pregnant woman with KCNQ1 variant long QT syndrome (LQTS) underwent fetal magnetocardiography (fMCG) after atrioventricular (AV) block was noted during fetal echocardiogram-atypical for LQTS type 1. Concern for fetal LQTS on fMCG prompted monitoring of maternal labs, change of maternal beta blocker therapy, and frequent fetal echocardiograms. Collaboration between obstetricians, neonatologists, and pediatric cardiologists ensured safe delivery. Beta blocker therapy was initiated after birth, and postnatal evaluation confirmed genotype and phenotype positive LQTS in the infant. Our experience suggests diagnosis and evaluation of fetal LQTS can alter antenatal management to reduce risk of poor fetal and postnatal outcomes.

中文翻译:

使用胎儿超声心动图和心动图对胎儿进行KCNQ1变异长QT综合征的胎儿诊断。

患有超声心动图检查的非典型LQTS类型1患者,在房室传导阻滞后,患有KCNQ1变异长QT综合征(LQTS)的孕妇接受了胎儿心动描记术(fMCG),对fQG的胎儿LQTS的关注提示监测母体检查,孕产妇β受体阻滞剂治疗和频繁的胎儿超声心动图检查。产科医生,新生儿科医生和儿科心脏病专家之间的合作确保了安全分娩。出生后开始β受体阻滞剂治疗,产后评估证实婴儿的LQTS基因型和表型为阳性。我们的经验表明,对胎儿LQTS的诊断和评估可以改变产前管理,以降低胎儿和产后预后不良的风险。
更新日期:2020-04-23
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