title: Foetal presentation of long QT syndrome.
abstract: Long-QT syndrome is a rare, inherited cardiac channelopathy that is characterized by arrhythmia, syncope and sudden cardiac death. Foetal symptoms are very rare and prenatal diagnosis is difficult. We report on a foetal presentation of long-QT syndrome with severe hydrops and a chaotic heart rhythm at 32 weeks of gestation. Postnatal electrocardiography showed runs of polymorphic ventricular tachycardia and an extremely prolonged-QT segment (QTc of 640 ms). The initial approach of overdrive pacing, followed by the combined therapy of a beta blocker, a sodium channel blocker (mexiletine) and potassium suppletion proved successful in maintaining a stable sinus rhythm. The girl was doing well at eight months of followup. In this patient a timely diagnosis and effective management after birth have been life-saving.The intrauterine manifestation of foetal atrioventricular dissociation and ventricular arrhythmia should raise suspicion of congenital long-QT syndrome.
authors.affiliation: Dept. of Pediatrics, University Hospitals Leuven, Leuven, Belgium. .
authors.forename: Chloe
authors.initials: C
authors.lastname: Theeuws
authors.forename: Dieter
authors.initials: D
authors.lastname: Nuyens
authors.forename: Marc
authors.initials: M
authors.lastname: Gewillig
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis*; D006801:Humans; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000175:diagnosis / Q000196:embryology*; D011247:Pregnancy; D016216:Ultrasonography, Prenatal / Q000379:methods*