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Summary:A well-studied case of intermittent long QT syndrome in a 21 -year-old female is presented. Electrophysiologic investigation repeated three times revealed changing sinoatrial and atrioventricular dysfunction and nonsustained ventricular tachycardia. The patient died 29 months after first hospitalization in a stage of electromechanical dissociation after runs of torsade de pointes although she had been treated with repeated anti-inflammatory therapy as well as high doses of propranolol. Postmortem examination demonstrated active inflammation of stellate ganglia. Myocardium appeared normal.

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