A Case of Long QT Syndrome

Sudden death of a family member is an emotionally devastating event and unfortunately an all-too-common presentation of genetic arrhythmia syndromes. The most common of these syndromes is the long-QT syndrome, which is characterized by an abnormal QT-interval prolongation on the surface ECG (＞440 msec in men, ＞460 msec in women) and an increased risk of sudden death, usually due to polymorphic ventricular arrythmia (Torsades de pointes) leading to ventricular fibrillation.A 44 year old man with hypertension, HCVD, Type 2 DM, legal blindness with ESRD on regular HD, and a recent history of syncope and VT during the previous week, presented to our Taichung Cheng Ching Hospital for persistent intermittent chest tightness and shortness of breath. He was found to have symptomatic bradycardia (HR 40-50), left lower lobe pneumonia, and right sided pleural effusion. He was admitted to MICU for close monitoring. Initially, a transvenous pacemaker was placed due to persistent bradycardia. Telemetric monitoring showed prolonged QT (QTc 536 msec), recurrent runs of Torsades and ventricular fibrillation in the absence of electrolyte imbalance. He was also treated with antiarrythmic agents intravenously, followed by mexiletine and Xanthium orally, and his symptoms were controlled. The hospital course was complicated by hospital-acquired pneumonia and ORSA bacteremia from catheter -related infections and he was treated with vancomycin and ceftazidime intravenously. He was discharged home after VVIR pacemaker placement on oral medications. On further testing, he was found to have late onset congenital LQTS type 2 proven by DNA sequence testing.