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Concealed congenital long QT syndrome during velopharyngeal dysfunction correction: a case report

Soeun Jeon1,2, Hyeon-Jeong Lee1,2, Young-Hoon Jung1

  • 1Department of Anesthesia and Pain Medicine, School of Medicine, Pusan National University, Busan, Korea.

Journal of Dental Anesthesia and Pain Medicine
|July 4, 2020
PubMed

Insights

Congenital long QT syndrome (LQTS) can be concealed, presenting during anesthesia with dangerous arrhythmias. This case highlights the importance of recognizing and managing LQTS, even when symptoms are transient.

Area of Science:

  • Cardiology
  • Genetics
  • Anesthesiology

Background:

  • Congenital long QT syndrome (LQTS) is a genetic disorder affecting heart rhythm.
  • LQTS increases the risk of ventricular tachycardia, leading to syncope or sudden cardiac death.
  • Diagnosis often relies on electrocardiographic findings and family history.

Observation:

  • A 4-year-old female experienced Torsades de pointes during general anesthesia for surgery.
  • The arrhythmia resolved spontaneously, but QT prolongation persisted post-event.
  • This presentation suggests a previously undiagnosed, concealed form of LQTS.

Findings:

  • The case demonstrates a rare presentation of concealed congenital long QT syndrome.
  • Anesthesia can unmask underlying cardiac channelopathies.
  • Persistent QT prolongation confirms the diagnosis despite transient arrhythmia.

Implications:

  • Early identification and management of concealed LQTS are crucial for preventing adverse cardiac events.
  • Awareness among anesthesiologists and cardiologists is vital for at-risk pediatric patients.
  • Genetic counseling and long-term cardiac monitoring are recommended for affected individuals and families.

Keywords:
ElectrocardiogramLong QT SyndromeTorsades de Pointes

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