Ventricular arrhythmias and sudden cardiac death — branching viva

Examiner opening (Examiner)

You are the general paediatric registrar in the emergency department. A fifteen-year-old boy has been brought in by paramedics after collapsing during a school football match. He was sprinting for the ball, there was no body contact, and he went down suddenly. The paramedics found him in ventricular fibrillation and achieved return of spontaneous circulation after two shocks and CPR. His twelve-lead ECG shows sinus rhythm with deep T-wave inversion in the lateral leads and voltage criteria for left ventricular hypertrophy. His father died suddenly at age thirty-eight. Talk me through your assessment and management. ^[1]

Exemplar opening (Candidate)

This child has been resuscitated from an out-of-hospital cardiac arrest due to ventricular fibrillation, and the combination of exertional collapse, lateral T-wave inversion, left ventricular hypertrophy on the ECG, and a family history of sudden death at thirty-eight makes hypertrophic cardiomyopathy the most likely diagnosis until proven otherwise. My immediate priorities are, first, to stabilise him in the post-arrest period — he needs a PICU or high-dependency bed, continuous cardiac monitoring, and haemodynamic support, because the risk of re-arrest is highest in the first twenty-four hours. Second, I need to confirm the diagnosis with echocardiography and cardiac MRI. Third, I need to involve the inherited cardiac conditions and electrophysiology service for the ICD decision and the family cascade screening. ^{[1] [3]}