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Related Experiment Videos

Idiopathic long q-t syndrome: brief case report and discussion

W E Noble1, P Chaudhuri, M A Qazi

  • 1Department of Medicine, Robert C. Byrd Health Sciences Center of WVU, Morgantown.

The West Virginia Medical Journal
|April 1, 1994
PubMed
Summary

Idiopathic Long QT Syndrome (LQTS) is a rare genetic disorder causing prolonged QTc intervals, syncope, and arrhythmias. This case highlights a late-onset presentation in a 54-year-old female, emphasizing the importance of recognizing LQTS in adults.

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Area of Science:

  • Cardiology
  • Genetics
  • Clinical Medicine

Background:

  • Idiopathic Long QT Syndrome (LQTS) is a rare familial disorder characterized by a prolonged QTc interval, predisposing individuals to syncope, ventricular arrhythmias, and sudden cardiac death.
  • The syndrome typically presents in childhood or adolescence, often with a history of syncope or documented arrhythmias.

Observation:

  • A 54-year-old female presented with syncope, exhibiting a prolonged QTc interval (0.50 ms) on electrocardiogram.
  • She experienced recurrent episodes of Torsades de Pointes during hospitalization, which were successfully managed with beta-blockers.
  • Electrocardiograms revealed asymptomatic prolonged QTc intervals in her mother and daughter, suggesting an autosomal dominant inheritance pattern.

Findings:

  • The case demonstrates a rare late-onset presentation of Idiopathic LQTS, diagnosed at age 54.

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  • The patient's symptoms and electrocardiographic findings were consistent with LQTS, despite the absence of congenital hearing impairment, as initially described by Romano and Ward.
  • Genetic transmission was evident through affected maternal and filial relatives.

    • Implications:

    • This case underscores the importance of considering LQTS in adult patients presenting with syncope and unexplained prolonged QTc intervals, even in the absence of typical early-onset symptoms.
    • Late diagnosis and management of LQTS can prevent potentially life-threatening cardiac events.
    • Further research into the genetic and clinical variability of LQTS may improve diagnostic strategies and therapeutic approaches for late-onset presentations.