Echocardiography of Anomalous Coronary Arteries

Study Questions:

What is the agreement between institutional echocardiographic reports and imaging core laboratory (ICL) review of patients in a multicenter registry with anomalous aortic origin of a coronary artery (AAOCA)?


The Congenital Heart Surgeons’ Society (CHSS) registry of 24 participating institutions was used as the data set for this study. Enrollment in the registry began in 2009 and consisted of patients diagnosed with AAOCA at age 30 years or younger. Transthoracic echocardiography images were available for 159 of the first 243 patients enrolled. For a prescribed set of anatomic AAOCA features, percentages of missing data in institutional echocardiogram reports were calculated. For each feature, agreement between institutional echocardiogram reports, ICL review of images, and surgical reports were evaluated using the weighted kappa statistic. An echocardiographic imaging protocol was developed to reduce differences between institutional echocardiogram reports and ICL review.


Of the 159 echocardiograms studied, 13%, 33%, and 62% were missing images necessary for diagnosis of intra-arterial course, proximal intramural course, and high ostial takeoff, respectively. Agreement between institutional reports and ICL review was poor, with kappa of 0.74 for diagnosis of origin of coronary artery, 0.11 for interarterial course, -0.03 for intramural course, and 0.13 for acute angle takeoff. Surgical findings were significantly different from both institutional and ICL reports, with ICL reports differing to a lesser extent.


Poor agreement between institutional reports and ICL review for AAOCA suggests need for an imaging protocol to permit evidence-based stratification and surgical planning.


Given the rarity of congenital coronary anomalies, the CHSS registry will likely prove important in better understanding their diagnosis and optimal management strategies. This study demonstrates the challenges of noninvasive imaging of coronary arteries. The echocardiograms in this study were plagued by incomplete data as well as poor agreement between institutional reports and the ICL. The authors propose an imaging protocol that will hopefully improve quality of imaging in this challenging group. An additional concern was that this was a highly selected group of patients, many of whom underwent surgical intervention. It is likely that in the general population undergoing pediatric echocardiograms, many of these coronary anomalies remain undiagnosed or otherwise not optimally imaged.

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