A multidisciplinary approach to prenatal treatment of congenital long QT syndrome. Issue 3 (5th August 2016)

• Record Type: Journal Article
• Authors: Crimmins, Sarah; Vashit, Sudhir; Doyle, Lauren; Harman, Chris; Turan, Ozhan; Turan, Sifa
• Is Part Of: Journal of clinical ultrasound. Volume 45:Issue 3(2017)
• Page Start: 168
• Page End: 170
• Publication Date: 2016-08-05
• Subjects: hydrops -- long QT -- prenatal diagnosis; Ultrasonics in medicine -- Periodicals; 616.07543
• DOI: 10.1002/jcu.22386
• Languages: English
• ISSNs: 0091-2751

Abstract

ABSTRACT: 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