Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate?

Abstract

OBJECTIVES Anomalous aortic origin of the coronary artery (AAOCA) with an interarterial ± an intramural course is a rare anomaly that carries a high risk of ischaemic events and even sudden death. The unroofing of the intramural course has been adopted as the gold standard surgical treatment. However, some anatomical forms need alternative techniques. METHODS We reviewed the surgical cohort with AAOCA managed at our institution between 2005 and 2019 and analysed the anatomical and clinical outcomes. RESULTS Thirty-nine patients underwent surgical interventions. The median age was 14 years (10–26 years). Twenty-eight patients (72%) had right AAOCA, and 11 (28%) had left AAOCA. Thirty-one (80%) patients presented with symptoms. The symptoms were chest pain in 22 patients (56%), syncope in 5 patients (13%), cardiac arrest during exercise in 2 patients (5%), dyspnoea in 6 patients (15%) and dizziness in 13 patients (33%). An ischaemic test was performed in 32 patients: Only 4 patients (10%) had positive results from the ischaemic test. All patients had computed tomography angiography scans to confirm the precise anatomical features of the anomaly. Repair techniques included 30 unroofing procedures (77%) with an associated translocation of the pulmonary artery for 11 patients in our early experience. In 6 patients the unroofing procedure was not feasible because of the absence of an intramural distinct segment or was judged intraoperatively not appropriate. A reimplantation of the anomalous coronary artery was performed in 2 patients (5%); 3 patients had coronary artery bypass grafting procedures (7%); and 3 (8%) had an isolated translocation of the pulmonary artery. There were no early or late deaths. All patients were free of symptoms. Computed tomography angiography scans performed in 31 cases showed a patent, non-restrictive coronary artery ostium. Seventeen patients underwent postoperative ischaemia testing and showed no evidence of ischaemia. CONCLUSIONS Surgical correction in AAOCA is mandatory both for symptomatic and for asymptomatic patients with evidence of myocardial ischaemia under stress or with a restricted coronary artery segment. Surgical unroofing remains the gold standard but is not appropriate for all forms: alternative techniques should be considered. Surgical results are promising.

Therapeutic Management of Anomalous Coronary Arteries Originating From the Opposite Sinus of Valsalva: Current Evidence, Proposed Approach, and the Unknowing

“… 14 In fact, most patients are asymptomatic; however, encountered symptoms may include angina pectoris, exercise‐ related or ‐unrelated chest pain, palpitations, dyspnea, dizziness, syncope, and myocardial infarction as well as SCD. 6 , 11 , 28 , 37 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 Besides acute ischemia–induced arrhythmias, repetitive minor ischemic events with consecutive myocardial fibrosis may serve as a substrate for ventricular tachyarrhythmias and SCD. 6 , 65 Consequently, multimodality diagnostic management of patients with ACAOS should not only imply the detection of anatomic high‐risk features and myocardial ischemia of ACAOS but also provide information for possible myocardial fibrosis/scar in suspected cases (Figure 2 ).…”

“… 52 , 75 , 76 An overview of the most relevant clinical trials with regard to treatment of patients with ACAOS is depicted in Table 2 . 28 , 37 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 Although coronary “unroofing” is the most common surgical procedure reported in the literature, 76 currently there are no recognized guideline recommendations on surgical techniques (neither in the American Heart Association/American College of Cardiology 2018 guidelines 19 nor in the European Society of Cardiology 2020 guidelines 20 ). Various surgical techniques have been reported, and the most common procedures with their advantages and limitations are outlined below.…”

“… † References 11 , 28 , 45 , 51 , 53 , 54 , 58 , 59 , 60 , 62 , 63 , 65 , 67 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 , 85 , 86 , 87 , 88 , 89 . …”

Coronary unroofing does not fits all anomalous aortic origin of coronary arteries

“…Several surgical techniques have been developed in the last years with excellent results in terms of mortality and freedom from re‐operations. Although the unroofing is the most used technique, neo‐ostioplasty and coronary reimplantation are effective and have showed comparable outcomes 11–13 . Regardless of the technique used, is it the overall shape and the normalization of the take‐off angle the mainstay of surgical management 13 .…”

“…Although the unroofing is the most used technique, neo-ostioplasty and coronary reimplantation are effective and have showed comparable outcomes. [11][12][13] Regardless of the technique used, is it the overall shape and the normalization of the take-off angle the mainstay of surgical management. 13 Also, unfortunately, there were some reports that have showed symptoms recurrence after surgery.…”