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 tachycardial 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 past-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.