Hypoplastic left heart syndrome — branching viva

Branch one — the neonatal collapse

The examiner opens with a three-day-old term infant in shock. Take the candidate through the immediate assessment, the prostaglandin-E1-first rule, the bedside distinction from coarctation, and the echocardiographic anatomy of atretic mitral and aortic valves with a hypoplastic ascending aorta. Probe what happens if the atrial septum is restrictive. [2]

Branch two — the interstage infant

The examiner moves to a four-month-old between the Norwood and the Glenn with a falling home saturation. Explore why this window carries the highest mortality, what an interstage home-monitoring programme contains, and the immediate management of any deterioration. [4]

Branch three — the Fontan survivor

The examiner closes with a sixteen-year-old Fontan patient with palpitations and leg swelling. Ask about the late complications of the Fontan circulation and the role of the general paediatrician in sustaining lifelong follow-up and neurodevelopmental surveillance into the transition to adult congenital heart disease services. [9]