Return of the Living Dead: Unique Case of Spontaneous Asystole after Torsades Pointes Ventricular Tachycardia Caught on Holter in a Congenital Long QT Syndrome Patient

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Congenital long QT syndrome is characterized by prolonged QT interval, associated with polymorphic ventricular tachycardia (Torsades pointes), related to repetitive episodes of syncope, and even sudden cardiac arrest. A 40-year-old female presented with palpitations, chest pain, dizziness, and recurrent, progressive episodes of syncope. Family history revealed no incidence of sudden cardiac death. Baseline determinations of complete blood count, thyroid function tests, and electrolytes were normal. 12-lead ECG showed prolonged QT interval (QTa=480 msec; QTc=490 msec). 24-hour Holter monitor revealed predominantly sinus bradycardia, QT of 540 msec. While on Holter, the patient went into syncope followed by cardiac arrest, and was resuscitated after less than 5 minutes. She was awake and stable immediately after. Review of Holter showed premature ventricular complexes in bigeminy, followed by long episode of Torsades pointes and ventricular tachycardia lasting for 48.9 seconds, which was spontaneously followed by asystole lasting 48.9 seconds, during which she was noted to be in cardiac arrest. Post-resuscitation rhythm was sinus rhythm. Coronary angiography was normal. Electrophysiology studies revealed no sinus node dysfunction. Implantation of cardiac defibrillator was done. She was started on propranolol, and was improved on follow up after 6 months. Recognition of congenital long QT syndrome is vital for proper intervention to be instituted.