Epinephrine-Induced Polymorphic Ventricular Tachycardia in a Patient With Congenital Long QT Syndrome

A 24-year-old woman presented to the department of plastic surgery for surgical excision of a nevus on her nose. Although her history failed to reveal any cardiac disease, her pre-operative electrocardiogram (ECG) showed an extremely prolonged QT interval of up to 528 msec. Repeated history-taking after admission revealed three syncopal episodes associated with both physical and emotional stress, and because the two-dimensional echocardiography and exercise ECG test were normal except for the prolonged QT interval, an epinephrine test was done to assess QT interval changes after an epinephrine infusion. Immediately after a bolus injection of epinephrine (0.1 microg/kg), marked prolongation of the QT interval developed, followed by polymorphic ventricular tachycardia which was immediately terminated with direct current shock, resulting in the diagnosis of a long QT syndrome (LQTS), probably type 1. Gene studies were recommended, but declined by the patient and her family. She was instructed to avoid competitive sports, and a beta-blocker was prescribed after which she remained symptom-free.