Congenital coronary anomalies and anomalous left coronary artery

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Target exams

Red flags

• A two- to three-month-old who is pale, sweaty and irritable with feeds, or who breathes fast and fails to thrive, has anomalous left coronary artery from the pulmonary artery (ALCAPA) until proven otherwise — the anterolateral Q waves on ECG and a dilated poorly contracting left ventricle on echo are the clues, and missing it as colic, bronchiolitis or dilated cardiomyopathy costs the infant a preventable infarct
• Exertional chest pain, palpitation or syncope in a child or adolescent is ischaemia or a malignant arrhythmia until proven otherwise — an anomalous aortic origin of a coronary artery with an interarterial course can present with sudden cardiac death as its first symptom, so these symptoms must never be reassured away
• Anomalous origin of the left coronary artery from the right sinus with an interarterial course carries the highest sudden-death risk of any coronary anomaly — restrict exercise, image the course urgently, and refer to congenital cardiology before discharge
• A continuous murmur in an infant or child can be a coronary artery fistula — a large fistula causes volume load, steal and heart failure, and needs closure rather than reassurance that it is an innocent venous hum
• A dilated cardiomyopathy phenotype in infancy is not idiopathic until the left coronary artery origin has been shown to come from the aorta on echocardiography — treating ALCAPA as medical cardiomyopathy rather than a surgical emergency is a classic and avoidable error

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognise ALCAPA in the irritable two- to three-month-old with feeding-related pallor and a dilated left ventricle
• Identify exertional chest pain or syncope in a young athlete as a coronary anomaly until proven otherwise
• State that echocardiography must define the origin and course of both coronary arteries in any infant with heart failure
• Explain the ALCAPA steal and why ischaemia emerges as pulmonary vascular resistance falls at two to three months
• Contrast surgical reimplantation with the Takeuchi intrapulmonary tunnel and when each is used
• Risk-stratify anomalous aortic origin by anatomy (left versus right sinus) and course (interarterial, intramural)
• Stabilise the infant with ALCAPA and cardiogenic shock with anti-failure therapy and mechanical support as a bridge to surgery
• Restrict exercise and refer urgently for the adolescent with interarterial AAOCA and exertional symptoms
• Present the differential of infantile dilated cardiomyopathy and the echocardiographic step that excludes ALCAPA
• Counsel a young athlete and family on exercise restriction and the decision to operate in AAOCA
• Cardiology: recognises congenital coronary anomalies as a cause of infant heart failure and exertional sudden death
• Neonates and infants: differentiates ALCAPA from bronchiolitis, sepsis and idiopathic cardiomyopathy
• Describe the embryology and classification of coronary anomalies and their haemodynamic consequences
• Interpret the anterolateral Q-wave ECG pattern and echocardiographic findings of ALCAPA
• Cardiology short case: examine and discuss a child with a continuous murmur or dilated cardiomyopathy phenotype
• Communication: explain AAOCA and exercise restriction to a young athlete and family
• Differentiate ALCAPA from dilated cardiomyopathy, myocarditis and sepsis in the infant
• Identify the high-risk coronary anomaly in a young athlete and initiate restriction and referral
• Formulate the acute and definitive management of ALCAPA and interarterial AAOCA
• Apply the AATS expert consensus and ACC/AHA adult congenital guidance to clinical decisions
• Recognise and manage congenital coronary anomalies across infancy, childhood and adolescence
• Apply pre-participation screening principles for coronary anomalies in young athletes