Department of Pediatric and Adult Congenital Heart Diseases, Marie Lannelongue Hospital, Groupe Hospitalier Saint Joseph Reference Center of Complex Congenital Heart Diseases M3C, Le Plessis Robinson, France.
Eur J Cardiothorac Surg. 2021 Apr 13;59(3):705-710. doi: 10.1093/ejcts/ezaa379.
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.
We reviewed the surgical cohort with AAOCA managed at our institution between 2005 and 2019 and analysed the anatomical and clinical outcomes.
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.
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.
冠状动脉异常起源（AAOCA）伴动脉间和/或动脉内-壁内走行是一种罕见的异常，可导致缺血事件甚至猝死的高风险。作为金标准手术治疗的方法是对壁内段进行“开窗”。然而，一些解剖形态需要采用替代技术。
我们回顾了 2005 年至 2019 年在我院接受治疗的 AAOCA 患者的手术队列，并分析了其解剖和临床结果。
39 例患者接受了手术干预。中位年龄为 14 岁（10-26 岁）。28 例（72%）患者为右冠状动脉异常起源，11 例（28%）为左冠状动脉异常起源。31 例（80%）患者有症状。症状为胸痛 22 例（56%）、晕厥 5 例（13%）、运动时心脏骤停 2 例（5%）、呼吸困难 6 例（15%）和头晕 13 例（33%）。32 例患者进行了缺血试验：仅 4 例（10%）患者的缺血试验结果阳性。所有患者均行计算机断层扫描血管造影以明确异常的精确解剖特征。修复技术包括 30 例“开窗”术（77%），其中 11 例患者早期经历了肺动脉移位术。6 例患者因不存在明确的壁内段或术中认为不合适而无法进行“开窗”术。2 例（5%）患者进行了异常冠状动脉再植入术，3 例（7%）患者进行了冠状动脉旁路移植术，3 例（8%）患者仅进行了肺动脉移位术。无早期或晚期死亡。所有患者均无症状。31 例患者行计算机断层扫描血管造影检查显示冠状动脉开口通畅，无狭窄。17 例患者行术后缺血试验，均无缺血证据。
对于有症状和无症状但有心肌缺血证据（应激时或冠状动脉节段受限）的患者，AAOCA 必须进行手术矫正。手术“开窗”仍然是金标准，但并非所有形式都适用：应考虑替代技术。手术结果有前景。
