The Present and Future
State-of-the-Art Review
Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva

https://doi.org/10.1016/j.jacc.2017.01.031Get rights and content
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Abstract

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva is increasingly recognized by cardiac imaging. Although most AAOCA subtypes are benign, autopsy studies report an associated risk of sudden death with interarterial anomalous left coronary artery (ALCA) and anomalous right coronary artery (ARCA). Despite efforts to identify high-risk ALCA and ARCA patients who may benefit from surgical repair, debate remains regarding their classification, prevalence, risk stratification, and management. We comprehensively reviewed 77 studies reporting the prevalence of AAOCA among >1 million patients, and 20 studies examining outcomes of interarterial ALCA/ARCA patients. Observational data suggests that interarterial ALCA is rare (weighted prevalence = 0.03%; 95% confidence interval [CI]: 0.01% to 0.04%) compared with interarterial ARCA (weighted prevalence = 0.23%; 95% CI: 0.17% to 0.31%). Recognizing the challenges in managing these patients, we review cardiac tests used to examine AAOCA and knowledge gaps in management.

Key Words

anomalous coronary artery
interarterial
management
outcomes
prevalence
sudden cardiac death

Abbreviations and Acronyms

AAOCA
anomalous aortic origin of a coronary artery
ALCA
anomalous left coronary artery
ANOCOR
anomalous connections of coronary arteries
ARCA
anomalous right coronary artery
CAA
coronary artery anomaly
CI
confidence interval
CTA
computed tomography angiography
FFR
fractional flow reserve
ICA
invasive coronary angiography
IVUS
intravascular ultrasound
MRA
magnetic resonance angiography
PCI
percutaneous coronary intervention
SCD
sudden cardiac death
TTE
transthoracic echocardiography

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The opinions and assertions herein are those of the authors alone, and do not represent the views of the U.S. Army, Office of the Surgeon General, Department of Defense, or the U.S. Government. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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