Open Access

ARTICLE

Introduction to anomalous aortic origin of a coronary artery

by

1 Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
2 Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA

* Corresponding Author: Julie Brothers, MD, Department of Pediatrics, The Children’s Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104. Email:

Congenital Heart Disease 2017, 12(5), 600-602. https://doi.org/10.1111/chd.12497

Abstract

Anomalous aortic origin of a coronary artery (AAOCA) occurs when both coronary arteries arise from the same aortic sinus from a single ostium or two separate ostia. While most coronary anomalies are benign, the two most common subtypes that predispose to sudden cardiac death in the young are interarterial anomalous right coronary artery and interarterial anomalous left coronary artery. Practitioners face many challenges with AAOCA. Diagnosing patients may be difficult because children and adolescents are often asymptomatic and first presentation may be sudden death or sudden cardiac arrest. Risk stratification is also challenging as determining which unique characteristics place the child at highest risk of sudden death has not been adequately delineated and ischemic testing may give false negative results. Last, there is significant variability in decision making regarding management of youth with AAOCA. Future research is needed to help determine the best way to identify at-risk children and which treatment is the safest and most efficacious.

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