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LQTS Treatment — RACP Paediatrics MCQ

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ModerateCardiologyLQTS TreatmentRACP Paediatrics

A 14-year-old girl presents with syncope during exercise. Her ECG shows a corrected QT interval (QTc) of 520 ms. Her family history includes sudden death of a sibling at age 16. What is the first-line treatment?

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Correct answer: D — Beta-blocker (nadolol or propranolol)

Long QT syndrome with QTc >500 ms and symptoms (syncope) requires beta-blocker therapy (nadolol preferred). ICD is considered for cardiac arrest survivors, syncope despite beta-blockers, or very high-risk genotypes (LQTS3). Avoidance of QT-prolonging drugs is essential for all patients.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Long QT Syndrome