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

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.