Cardiomyopathies in children — structured clinical encounter

Station brief (candidate)

You are the paediatric registrar in the emergency department. A 14-year-old competitive runner collapsed briefly during a 1500-metre race and recovered fully within seconds. His father died suddenly at 38 of an unspecified "heart attack". On examination he is well, with a soft ejection systolic murmur at the left sternal border that grows louder on standing. The team asks you to establish the diagnosis, order and interpret the investigations, perform the sudden-death risk stratification, and proceed to the management and family-counselling plan. You have 12 minutes with the team and 5 minutes for examiner discussion. ^{[4] [6]}