Myocardial Ischemia in Children With Anomalous Aortic Origin of a Coronary Artery With Intraseptal Course

Abstract

Background: Intraseptal anomalous aortic origin of a coronary artery is considered a benign condition. However, there have been case reports of patients with myocardial ischemia, arrhythmia, and sudden cardiac death. The purpose of this study was to determine the clinical presentation, myocardial perfusion on provocative stress testing, and management of children with anomalous aortic origin of a coronary artery with an intraseptal course in a prospective cohort. Methods: Patients with anomalous aortic origin of a coronary artery and intraseptal course were prospectively enrolled from December 2012 to May 2019, evaluated, and managed following a standardized algorithm. Myocardial perfusion was assessed using stress imaging. Fractional flow reserve was performed in patients with myocardial hypoperfusion on noninvasive testing. Exercise restriction, β-blockers, and surgical intervention were discussed with the families. Results: Eighteen patients (female 6, 33.3%), who presented with no symptoms (10, 55.6%), nonexertional (4, 22.2%), and exertional symptoms (4, 22.2%), were enrolled at a median age of 12.4 years (0.3–15.9). Perfusion imaging was performed in 14/18 (77.8%) and was abnormal in 7/14 (50%); fractional flow reserve was positive in 5/8 (62.5%). All 4 patients with exertional symptoms and 3/10 (30%) with no or nonexertional symptoms had myocardial hypoperfusion. Coronary artery bypass grafting was performed in a 4-year-old patient; β-blocker and exercise restriction were recommended in 4 patients not suitable for surgery. One patient had nonexertional chest pain and 17 were symptom-free at median follow-up of 2.5 years (0.2–7.1). Conclusions: Up to 50% of patients with intraseptal anomalous aortic origin of a coronary artery had inducible myocardial hypoperfusion during noninvasive provocative testing. Long-term follow-up is necessary to understand the natural history of this rare anomaly.

Dobutamine stress cardiac MRI is safe and feasible in pediatric patients with anomalous aortic origin of a coronary artery (AAOCA)

“…To the best of our knowledge, this is the rst study to describe the use of DSCMR in the assessment of myocardial perfusion, wall motion, and myocardial viability in a large cohort of pediatric patients with AAOCA. We demonstrated feasibility and safety with DSCMR and this strategy has been helpful at our institution in risk strati cation and decision-making of these challenging patients at presentation, followup, and post-intervention assessment [16][17][18][19][20]. Inducible hypoperfusion resolved in the majority of patients after surgery.…”

“…Surgery or medical intervention would be considered based on individual patient discussion and recommended for patients with a positive DSCMR. If a patient is undergoing surgical repair of AAOCA, a similar approach would be used routinely at 3 months after surgery and help inform return to activities [15][16][17][18][19].…”

Beta-Blockade in Intraseptal Anomalous Coronary Artery With Reversible Myocardial Ischemia

“…2 This patient has a very long combined intraseptal and intramyocardial course of the LCA, LAD, and LCX totaling 58 mm, which was classified as type A 4 . 1 This type of anomaly is associated with myocardial ischemia with provocative testing in up to 50%, especially in those with longer intramyocardial course, 1 such as this young child. The patient’s symptoms improved after starting atenolol and resolved over a six-year follow-up.…”

“…Anomalous aortic origin of a left coronary artery (L-AAOCA) from the right sinus with an intraseptal course is a rare coronary anomaly and can be associated with myocardial ischemia and sudden cardiac death. 1 There is currently no medical or surgical intervention known to improve long-term patient outcomes. We herein report six-year follow-up data on a young child who was started on β-blockade after the initial assessment showed reversible myocardial ischemia.…”

Anatomical characteristics of anomalous left coronary artery from the opposite sinus (left-ACAOS) and its clinical relevance: A serial coronary CT angiography study

“…In addition to “interarterial courses”, some additional potential high-risk coronary artery abnormalities are typically demonstrated in patients with ischemia in ACAOS, including acute take-off angle [33] , intraseptal course [39] , intramural course and length, and orifice anomalies [40] etc., but no agreement has been reached concerning definite risk features. In our series, proximal narrowing exhibited a weak association with ischemic symptoms; this is in accordance with data in right-ACAOS patients from Opolski, et al’s [24] and Marano et al’s [41] .…”