Congenital coronary anomalies and ALCAPA — branching viva

Examiner opening (Examiner)

You are the general paediatric registrar in the emergency department. A ten-week-old infant is brought in with pallor, sweating and irritability during feeds, poor weight gain, and increasing breathlessness. He has a gallop rhythm, a pansystolic murmur at the apex, and hepatomegaly. The electrocardiogram shows deep Q waves in the anterolateral leads. Talk me through your assessment and immediate management. ^{[1] [2]}

Exemplar opening (Candidate)

This infant has heart failure with a presentation that is highly suggestive of anomalous left coronary artery from the pulmonary artery, and I will move quickly because this is a surgical emergency. The feeding-related pallor, diaphoresis and irritability are the anginal equivalents of infancy, and the anterolateral Q waves on the electrocardiogram reflect the anterolateral infarction that characterises ALCAPA. I will examine for the degree of heart failure and perfusion, take blood for a troponin, full blood count and gases, and arrange an urgent echocardiogram with the explicit request to demonstrate the origin and proximal course of both coronary arteries. While I wait for imaging I will start oxygen, give intravenous furosemide, begin gentle afterload reduction, and involve the paediatric intensive care and cardiology teams, because the definitive treatment is surgery to re-establish a dual coronary system. ^{[1] [2]}