Clinical Presentation and Medium-Term Outcomes of Children With Anomalous Aortic Origin of the Left Coronary Artery: High-Risk Features Beyond Interarterial Course

Abstract

Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes. Methods: All patients with AAOLCA <21 years old were prospectively enrolled (December 2012–November 2020), including group 1: AAOLCA from the right aortic sinus with interarterial course, group 2: AAOLCA from the right aortic sinus with intraseptal course, and group 3: AAOLCA with a juxtacommissural origin between the left and noncoronary aortic sinus. Anatomic details were assessed using computed tomography angiography. Provocative stress testing (exercise stress testing and stress perfusion imaging) was performed in patients >8 years old or younger if concerning symptoms. Surgery was recommended for group 1 and in select cases in group 2 and group 3. Results: We enrolled 56 patients (64% males) with AAOLCA (group 1, 27; group 2, 20; group 3, 9) at median age of 12 years (interquartile range, 6–15). Intramural course was common in group 1 (93%) compared with group 3 (56%) and group 2 (10%). Seven (13%) presented with aborted sudden cardiac death (group 1, 6/27; group 3, 1/9); 1 (group 3) with cardiogenic shock. Fourteen/42 (33%) had inducible ischemia on provocative testing (group 1, 32%; group 2, 38%; group 3, 29%). Surgery was recommended in 31/56 (56%) patients (group 1, 93%; group 2, 10%; and group 3, 44%). Surgery was performed in 25 patients at a median age 12 (interquartile range, 7–15) years; all have been asymptomatic and free from exercise restrictions at median follow-up of 4 (interquartile range, 1.4–6.3) years. Conclusions: Inducible ischemia was noted in all 3 AAOLCA subtypes while most aborted sudden cardiac deaths occurred in interarterial AAOLCA (group 1). Aborted sudden cardiac death and cardiogenic shock may occur in AAOLCA with left/nonjuxtacommissural origin and intramural course, thus also deemed high-risk. A systematic approach is essential to adequately risk stratify this population.

Invasive Coronary Assessment in a 9-Year-Old With Anomalous Aortic Origin of the Left Coronary Artery

“…Our current algorithm followed in our program has since been modified to specify age at which we believe surgery should be recommended in AAOLCA, and additional studies to be pursued in younger patients if deemed appropriate, including cardiac catheterization with measurement of intracoronary flow. 7 Of note, surgical unroofing successfully placed the LCA ostium into the appropriate sinus with uniform caliber throughout despite residual interarterial course. Our program 8 and others 9 have described transection and reimplantation as another suitable surgical technique, although it is typically performed when there is no or a very short intramural course and unroofing would be unsuccessful in placing the anomalous coronary ostium in the correct sinus.…”

High-Risk Anomalous Aortic Origin of the Left Coronary Artery: Consecutive Admissions Presenting With Sudden Cardiac Arrest

“…Furthermore, exertional syncope or chest pain indicate potential severity, as demonstrated in the association with inducible hypoperfusion. 4 Congenital coronary anomalies are commonly diagnosed in asymptomatic young people. 3,5 Further, evaluation with a resting ECG is not helpful in the diagnosis, as it is normal, and exercise stress test has exceedingly low sensitivity.…”

“…2,3 In one-third of cases, the first presentation of AAOLCA is aborted sudden cardiac arrest (SCA) and represents the second leading overall cause of SCA in the young. 1,4 This series aims to describe two consecutive patients with AAOLCA from the right aortic sinus presenting with aborted SCA on the same day with diverse outcomes and management strategies upon arrival at Texas Children's Hospital.…”

Anomalous Right Coronary Artery Clinical Presentations and Considerations

“…These developmental rarities occur in less than 1% of the population and are characterized by numerous variations in their shape, origins, and location of ostia [2]. Despite its variable nature, an anomalous coronary artery is usually asymptomatic, although it can also present with clinically significant features such as acute decompensated heart failure, cardiogenic shock, syncope, and sudden cardiac death [3][4][5][6][7][8][9]. Clinical heterogeneity is a hallmark, and ominous presentations are often associated with clinically significant lesions such as the right coronary artery (RCA) originating from the left sinus of Valsalva or the left anterior descending artery (LAD) [10].…”