Abstract Background Anomalous aortic origin of a right coronary artery (R-AAOCA) may cause myocardial ischemia and sudden death in the young. Methods Cohort study of patients presenting to our Coronary Artery Anomalies Program at our hospital with R-AAOCA. CTA defined R-AAOCA morphology. EST and stress perfusion imaging (sPI) with nuclear (sNPI) or dobutamine stress cardiac MRI (DSCMR) were performed if ≥11 yrs or 11 years with concern for ischemia. High-risk features (HRF) included intramural length, slit-like/hypoplastic ostium, exertional symptoms or evidence of ischemia. Results Patients presenting with R-AAOCA between 12/2012-12/2024 were included. Of 522 patients (male 319, 61%), median age at presentation was 12.2 IQR 7.5-15.1 years. All patients had R-AAOCA from the left sinus but 3 with origin from the non-coronary sinus. CTA was available in 437/522 (84%) with median intramural length of 5 IQR 4-7 mm and similar in both non-surgical and surgical patients. EST performed in 379/522 (73%) was positive in 6/379 (1.6%) patients. sPI was performed in 408/522 (78%) (319 (78%) DSCMR and 89 (22%) SNPI). In the total cohort, inducible ischemia on sPI was seen in 32/408 (8%) (26 DSCMR and 6 SNPI). LGE was present in 3 but all at inferior hingepoint. Surgery was recommended in 86/522 (16%) patients with HRF, (62 unroofing; 18 reimplantation; 1 CABG; 2 ostioplasty, 3 declined), median age at surgery 15 years IQR 11.5-15.5. In the surgical cohort, CTA was available in 85/86 (99%) and slit-like ostium found in 70/86 (81%). Abnormal EST with significant ST depression with exercise was seen in 4/75 (5%) patients. sPI was available in 81/86 (94%) (50 DS-CMRs and 31 sNPI). Inducible perfusion defect was present in 26/81 (32%) patients. Preoperative cardiac cath was performed in 13 of which 4 (31%) had abnormal values for FFR/iFR measurements, and 4 (31%) could not have the ostium engaged and found hypoplastic (1-1.5 mm) at surgery. Postoperative CTA was available in 44/86 (51%) with no residual stenosis. Postoperative sPI at about 3 months was available in 41 patients (33 DSCMR, 8 sNPI). In those with abnormal testing for ischemia, there was resolution of ST segment changes on EST, inducible perfusion defect on sPI and abnormal FFR/iFR values on cardiac cath. All (but 1 – death secondary to oncological causes) patients were alive and have returned to exercise at last median follow-up of 3.6 IQR 1.3-6 years in non-surgical and surgical patients. Follow up DS-CMR (34 postoperatively) completed in 67 at 4.5 IQR 3-5.5 years from initial evaluation were negative for evidence of inducible ischemia Conclusions R-AAOCA can present with inducible ischemia on sPI and have similar intramural length in unrepaired and repaired patients. All undergoing surgery for HRF had resolution of ischemia on provocative testing. All patients were alive at medium to long-term follow-up with no evidence of myocardial ischemia.
Sachdeva et al. (Sat,) studied this question.