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A multidisciplinary approach to prenatal treatment of congenital long QT syndrome
Author(s) -
Crimmins Sarah,
Vashit Sudhir,
Doyle Lauren,
Harman Chris,
Turan Ozhan,
Turan Sifa
Publication year - 2016
Publication title -
journal of clinical ultrasound
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.272
H-Index - 61
eISSN - 1097-0096
pISSN - 0091-2751
DOI - 10.1002/jcu.22386
Subject(s) - medicine , long qt syndrome , torsades de pointes , mexiletine , bradycardia , lidocaine , supraventricular tachycardia , sick sinus syndrome , flecainide , cardiology , proarrhythmia , fetus , atrioventricular block , in utero , ventricular tachycardia , transplacental , sinus rhythm , sinus bradycardia , heart disease , anesthesia , qt interval , tachycardia , pregnancy , heart rate , atrial fibrillation , biology , placenta , blood pressure , genetics
A 27‐week fetus evaluated for bradycardia and hydrops was found to have anti‐SSA‐negative 2° 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. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45 :168–170, 2017