SAQ 430 – Paediatrics – exertional syncope with hypertrophic cardiomyopathy

Paediatrics – exertional syncope with hypertrophic cardiomyopathy.

a. ECG findings

• Left ventricular hypertrophy/large voltages.
• Global or widespread ST depression.
• ST elevation in aVR and anterior right precordial leads such as V1-V2.
• P-wave abnormalities.
• Sinus rhythm with no short PR interval.
• Non-specific T-wave abnormalities, particularly laterally.

b. Diagnosis

• Hypertrophic cardiomyopathy presenting with exertional syncope.

c. Complications

• Ventricular arrhythmia and sudden cardiac death.
• Syncope or injury during exertion.
• Genetic transmission and risk to relatives/offspring.
• Myocardial ischaemia from abnormal coronary perfusion may occur.

d. Management priorities

• Admit with continuous cardiac monitoring/telemetry and avoid exertion.
• Echocardiography to assess structure, valve disease, and left ventricular outflow tract obstruction.
• Paediatric cardiology review and counselling for patient and parents, including sports restriction and family screening.
• Consider beta-blocker therapy and electrophysiology/ICD assessment depending on risk.