An asymptomatic 6-year-old is found to have a corrected QT interval of 490 ms with T wave notching on a school screening ECG. Genetic testing confirms type 1 long QT syndrome. No family member has had syncope. What is the appropriate first-line therapy?
- A Beta blocker therapy with nadolol or propranolol plus avoidance of QT-prolonging drugs ✓
- B Implantable cardioverter defibrillator implantation
- C Left cardiac sympathetic denervation
- D Flecainide monotherapy
Correct answer: A. Beta blocker therapy with nadolol or propranolol plus avoidance of QT-prolonging drugs
Explanation
Even asymptomatic genotype-positive children merit beta blockade because the first event can be fatal, and nadolol or propranolol are preferred agents with consistent efficacy across genotypes. Counseling must cover avoidance of competitive swimming in type 1, dehydration, and all QT-prolonging drugs. An ICD is reserved for survivors of cardiac arrest or breakthrough syncope despite therapy, and sympathetic denervation is third-line. Flecainide is an adjunct in selected type 3 patients, not monotherapy.
Reference: Nelson Textbook of Pediatrics, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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