title: Report of a life-threatening arrhythmia after hospital discharge in a liver transplant recipient with previously unknown congenital long QT syndrome.
abstract: The long QT syndrome affects heart rhythm by prolonging ventricular repolarisation; it is potentially life-threatening since it can evolve into torsades de pointes (a polymorphic ventricular tachycardia) and/or ventricular fibrillation. The case is presented of a 55-year-old liver transplant recipient with a genetically determined long QT syndrome not detected by the standard preoperative cardiological evaluation. It was mild in the immediate post-operative period but developed into torsades de pointes after discharge, probably as a result of therapy. This case was particularly challenging because the first arrhythmic episodes were short and electocardiographically silent, and thus the related faints were thought to have a neurological basis. When the true cause emerged during a monitored episode of torsades de pointes, electric defibrillation was used to restore sinus rhythm and isoproterenol administered to increase heart rate and thus shorten the prolonged QT interval Long-term control was obtained by means of an implantable intracardiac defibrillator. In orthotopic liver transplant recipients with long QT syndrome, particular attention should be given to post-operative therapy as some routinely used drugs can trigger life-threatening ventricular arrhythmias.
authors.affiliation: Anesthesia & Intensive Care, Department No 1, Pisa NHS Trust, Ospedale di Cisanello, Italy. g.biancofiore@med.unipi.it
authors.forename: G
authors.initials: G
authors.lastname: Biancofiore
authors.forename: C
authors.initials: C
authors.lastname: Valentini
authors.forename: F
authors.initials: F
authors.lastname: Cellai
authors.forename: F
authors.initials: F
authors.lastname: Filipponi
authors.forename: F
authors.initials: F
authors.lastname: Mosca
authors.forename: A
authors.initials: A
authors.lastname: Vagelli
mesh_terms: D001145:Arrhythmias, Cardiac / Q000209:etiology*; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008133:Long QT Syndrome / Q000150:complications* / Q000151:congenital* / Q000601:surgery; D008875:Middle Aged