A Multidisciplinary Approach to Prenatal Treatment of Congenital Long QT Syndrome

被引:5
作者
Crimmins, Sarah [1 ]
Vashit, Sudhir [2 ]
Doyle, Lauren [1 ]
Harman, Chris [1 ]
Turan, Ozhan [1 ]
Turan, Sifa [1 ]
机构
[1] Univ Maryland, Sch Med, Dept Obstet, Ctr Adv Fetal Care, Baltimore, MD 21201 USA
[2] Univ Maryland, Sch Med, Dept Pediat, Baltimore, MD 21201 USA
关键词
hydrops; long QT; prenatal diagnosis; CARDIAC-ARRHYTHMIA; CHANNEL DYSFUNCTION; FETAL;
D O I
10.1002/jcu.22386
中图分类号
O42 [声学];
学科分类号
070206 ; 082403 ;
摘要
A 27-week fetus evaluated for bradycardia and hydrops was found to have anti-SSA-negative 28 atrioventricular block and ventricular tachycardia. A presumptive diagnosis of fetal long QT syndrome was made. Transplacental pharmacotherapy with intravenous magnesium and lidocaine restored sinus rhythm. At 30 6/7 weeks, the infant was delivered due to premature labor. Despite postnatal treatment with mexiletine and propranolol, she developed torsades de pointes. Ultimately, a de novo KCNH2 G628S mutation was diagnosed. She received an implantable cardiac defibrillator at 5 months of age. Early diagnosis and a multidisciplinary approach allowed successful in utero treatment and anticipatory postnatal management. (C) 2016 Wiley Periodicals, Inc.
引用
收藏
页码:168 / 170
页数:3
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