Key result
A 21-year-old female with intermittent long QT syndrome died 29 months after hospitalization despite therapy, with postmortem showing active inflammation of stellate ganglia.
Case Report (n=1)
Demonstrates a potential link between inflammatory degeneration of the stellate ganglia and fatal intermittent long QT syndrome.
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Does not support practice change in long QT syndrome; leaves open stellate ganglia inflammation as a hypothesis in fatal cases.
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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Pfeiffer et al. (1989) conducted a case report in Intermittent long QT syndrome (n=1). Anti-inflammatory therapy and propranolol was evaluated on Death. A 21-year-old female with intermittent long QT syndrome died 29 months after hospitalization despite therapy, with postmortem showing active inflammation of stellate ganglia.
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