Ischemia in Anomalous Aortic Origin of a Right Coronary Artery: Large Pediatric Cohort Medium-Term Outcomes

Abstract

Background: Anomalous aortic origin of a right coronary artery may cause myocardial ischemia and sudden death in the young. Data on myocardial ischemia or longitudinal outcomes are sparse in pediatric anomalous aortic origin of a right coronary artery population. Methods: Patients <21 years with anomalous aortic origin of a right coronary artery were prospectively enrolled. Computerized tomography angiography defined morphology. Exercise stress test and stress perfusion imaging (sPI) were performed if >7 years or younger with concern for ischemia. High-risk features included intramural length, slit-like/hypoplastic ostium, exertional symptoms, or evidence of ischemia. Results: A total of 220 patients (60% males) were enrolled December 2012 to April 2020 at a median age 11.4 years (interquartile range, 6.1–14.5), including 168 (76%) with no/nonexertional symptoms (group 1) and 52 (24%) with exertional chest pain/syncope (group 2). Computerized tomography angiography was available in 189/220 (86%), exercise stress test in 164/220 (75%), and sPI in 169/220 (77%). Exercise stress test was positive in 2/164 (1.2%) patients in group 1, both had positive sPI. Inducible ischemia (sPI) was detected in 11/120 (9%) in group 1 and 9/49 (18%) in group 2 ( P =0.09). Intramural length was similar in patients with/without ischemia (5 [interquartile range, 4–7] versus 5 [interquartile range, 4–7] mm; P =0.65). Surgery was recommended in 56/220 (26%) patients with high-risk features. In 52 surgical patients (38 unroofing, 14 reimplantation), all subjects were alive and have returned to exercise at last median follow-up of 4.6 (interquartile range, 2.3–6.5) years. Conclusions: Anomalous aortic origin of a right coronary artery patients can present with inducible ischemia on sPI despite symptoms or intramural length. Exercise stress test is a poor predictor of ischemia and caution should be given to determine low-risk based solely on this assessment. All patients are alive at medium-term follow-up.

Congenital heart disease: types, pathophysiology, diagnosis, and treatment options

“…Stress perfusion imaging can be used to determine the rate of myocardial ischemia in these patients. 209 However, this was also a single-center study with a limited patient population, which may result a selection bias. Therefore, multicenter, large-scale prospective studies with long follow-up period should be conducted in the future to determine the features of patients with AAOCA who are at an increased risk of adverse events.…”

“…210 In another study of 220 patients, 168 (76%) did not experience exertional symptoms and only 52 (24%) had exertional chest pain or syncope. 209 Notably, AAOCA is associated with myocardial ischemia and SCD. SCD may occur in asymptomatic patients during or shortly after exertion.…”

“…SCD may occur in asymptomatic patients during or shortly after exertion. 209 However, the identification of patients who may be at a higher risk of ischemia and sudden adverse cardiac events remains unclear. Molossi et al 210 recently reported that the African American race, an older age at diagnosis, an intramural course, and syncope on exertion were predictors of high-risk lesions, suggesting that these may be associated with SCD.…”

“…210 Previous guidelines reported that asymptomatic individuals with AAOCA have a low risk of SCD and require no additional testing upon a normal exercise stress test. 211 In a 2023 study of 220 patients <21 years with AAOCA, Doan et al 209 proposed that exercise stress was a poor predictor of ischemia. Therefore, the determination of patient risk based solely on this test should be conducted with caution.…”

Assessment of Intramural Segment Compression in Anomalous Coronary Arteries through Patient-Specific Finite Element Modeling

“…Diagnosis and morphological evaluation are routinely performed with computed tomography angiography (CTA), which allows the definition of anomalous coronary anatomical features. Functional myocardial evaluation is performed with an exercise stress test, cardiac MRI, or myocardial scintigraphy, which showed poor ability to detect inducible myocardial ischemia in AAOCA [12,13]. Cardiac catheterization with fractional flow reserve (FFR) and intravascular ultrasound (IVUS) may help with risk stratification, but they are invasive procedures and not always conclusive.…”