ABSTRACT
Introduction
There are many uncertainties surrounding anomalous aortic origin of a coronary artery (AAOCA) including the pathophysiology of sudden cardiac death, how to best risk stratify patients, how to best evaluate patients, who would benefit from exercise restriction, who should undergo surgical intervention, and which operation to perform.
Areas covered
The goal of this review is to provide a comprehensive but succinct overview of AAOCA to help clinicians with the difficult task of navigating optimal evaluation and treatment of an individual patient with AAOCA.
Expert opinion
Beginning in year 2012, some of our authors proposed an integrated, multi-disciplinary working group which has become the standard management strategy for patients diagnosed with AAOCA. A multi-disciplinary team with a focus on shared decision-making with the patients/families is likely necessary to optimize outcomes. Long-term follow-up and research are needed to improve our understanding of AAOCA.
Article highlights
AAOCA is the second leading cause of sudden cardiac death in young athletes in the US.
There continues to be heterogeneity in the management of patients with AAOCA across institutions.
A uniform approach can help to risk stratify and discern the best management strategy for optimal long-term outcome.
A multi-disciplinary collaborative team is needed to provide optimal services and support to patients and families with AAOCA.
Ongoing long-term data collection and research are important to answer the unknowns related to this disease.
Declaration of interest
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
Reviewer disclosures
Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.