UMEM Educational Pearls

Title: What Sound Does an ALCAPA Make?

Keywords: pediatric cardiology, ALCAPA (anomalous left coronary artery from the pulmonary artery) (PubMed Search)

Posted: 10/7/2022 by Rachel Wiltjer, DO
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Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect in which there is an altered origin of the left coronary artery (also known as Bland-White-Garland syndrome)
Generally asymptomatic at birth, but can present in late infancy, toddlerhood, or later with signs of congestive heart failure, a myocarditis picture, or sudden cardiac death
Flow through the left coronary artery is normal at birth due to high pulmonary pressures, but as those pressures drop the blood flow drops as well and may become reversed due to the pressure gradient
This can cause chronic myocardial ischemia, the severity of which, is dependent on collateral flow
Most patients will also develop mitral regurgitation
Cardiomegaly may be seen on CXR (and some patients will present with respiratory symptoms/wheezing)
EKG findings include: findings consistent with ischemia (ST changes, q waves – specifically in the anterolateral leads), leftward axis (for age), abnormal R wave progression (loss of R wave amplitude in affected leads)
Diagnosis can generally be made with echocardiogram (although not 100% sensitive) and the disease is generally treated with surgical repair

References

Cashen K, Kwiatkowski DM, Riley CM, Buckley J, Sassalos P, Gowda KN, Iliopoulos I, Bakar A, Chiwane S, Badheka A, Moser EAS, Mastropietro CW; Collaborative Research from the Pediatric Cardiac Intensive Care Society (CoRe-PCICS) Investigators. Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery: A Retrospective Multicenter Study. Pediatr Crit Care Med. 2021 Dec 1;22(12):e626-e635.

Hoffman JI. Electrocardiogram of anomalous left coronary artery from the pulmonary artery in infants. Pediatr Cardiol. 2013 Mar;34(3):489-91.

Levitas A1, Krymko H, Ioffe V, Zalzstein E, Broides A. Anomalous Left Coronary Artery From the Pulmonary Artery in Infants and Toddlers Misdiagnosed as Myocarditis. Pediatr Emerg Care. 2016 Apr;32(4):232-4