A case of congenital long QT syndrome, type 8, undergoing laparoscopic hysterectomy with general anesthesia

Abstract

Objective: Patients with Long QT syndrome (LQTS) P may present with torsades de pointes, ventricular tachycardia (VT), or ventricular fibrillation (VF) and are at risk of sudden cardiac death. Materials and methods: A 38 y/o female patient with uterus myoma developed VF during laparoscopic assisted vaginal hysterectomy surgery. Defibrillation was delivered and the electrocardiogram (ECG) returned to sinus rhythm after CPR. Results: Patient survived and implantable cardioverter-defibrillator was implanted and received beta-blocker therapy. ECG obtained in out-patient clinic still showed QT interval prolongation, but revealed no prolongation few months after persistent beta-blocker therapy. LQTS type 8 (CACNA1C E768del mutation) was identified by genetic DNA sequencing study. Conclusions: Patients with concealed LQTS may have normal QT interval unless exposing to stress or specific stimuli. Unexpected ventricular arrhythmia may happen during any medical management. We should avoid triggers of QT prolongation, and get familiar with management of the episode. Keywords: Beta-blockade, Long QT syndrome, Ventricular fibrillation, Gene CACNA1C