Anomalous aortic origin of a coronary artery (AAOCA) is observed in approximately 0.14% of the population (1–4), with the most common variations being an anomalous right coronary artery originating from the left sinus (ARCA-L) and an anomalous left main coronary artery originating from the right sinus (ALCA-R), especially when an interarterial course (IAC) exists between the aorta and pulmonary artery. AAOCA is linked to a heightened risk of myocardial ischemia, infarction, and infrequent cause of sudden cardiac death (SCD). Surgical intervention is typically pursued to mitigate ischemic events and includes procedures such as coronary artery bypass grafting (CABG), patch enlargement of the proximal coronary artery, reimplantation into the correct sinus, and unroofing, particularly in cases involving an intramural coronary course (1).

Despite the known benefits and limitations of each surgical technique, there is ongoing debate regarding the optimal choice of procedure, which is highly individualized. This study seeks to review our institutional experience with AAOCA and evaluate the medium-term outcomes following surgical repair. We conducted a retrospective observational analysis of 12 AAOCA patients treated at our institution, including the first documented case of off-pump reimplantation for an ARCA-L.

The preoperative characteristics of the patients and the surgical procedures performed are summarized in Table 1. The median age at the time of surgery was 26 years (range, 13–57 years). Five patients (41.7%) with ARCA-L were older than 32 years, while seven patients (58.3%) with ALCA-R were younger than 32 years. All 12 patients presented with symptoms of angina, dyspnea, or syncope prior to surgery, and three patients with ALCA-R had experienced preoperative myocardial infarctions. All patients had high-risk anatomical features associated with AAOCA, including a slit-like ostium, acute angle of takeoff, and interarterial course. A comparison of the clinical characteristics between ALCA-R and ARCA-L patients is shown in Table 2. There were no significant differences between ALCA-R and ARCA-L in terms of sex or preoperative left ventricular ejection fraction (EF). However, patients with ALCA-R were significantly younger (P < 0.001) and appeared to have a longer duration of symptoms and larger preoperative left ventricular end-diastolic dimension (LVEDD), although these differences were not statistically significant. No significant differences were observed between preoperative and postoperative EF or LVEDD in either group (Figure 1).

Of the 12 patients, three underwent unroofing, three underwent CABG, three underwent patch angioplasty of the proximal coronary artery, and three underwent reimplantation. There were no operative mortalities or early morbidities (death in 30 days after surgery). The median hospital stay was 9 days (range, 7–12 days).

The median follow-up period was 13 years (range, 8–15years), with a 100% follow-up rate. All patients were alive at the most recent evaluation; however, four patients (33.3%) with ALCA-R reported experiencing cardiac-type symptoms (angina, syncope,dyspnea) postoperatively, most commonly angina and dyspnea. For this study, cardiac-type symptoms were defined as those occurring 3 months or longer after surgical repair. Of these four patients, two were restricted from physical activity. Compared with ARCA-L, the incidence of postoperative cardiac-type symptoms was higher in ALCA-R patients (P = 0.049) (Figure 2). All patients underwent follow-up coronary angiography or computed tomography angiography (CCTA) (Figures 3–6). All repaired coronary arteries were widely patent, except for case 5, in which the left internal mammary artery graft was found to be occluded 14 months postoperatively.

Case 10, a 47-year-old woman, presented with dyspnea for 2 months before surgery. Coronary angiography and CCTA revealed that the RCA originated from the left coronary sinus with an IAC between the aorta and pulmonary artery. The procedure was performed through a median sternotomy without cardiopulmonary bypass (Supplementary Video S1). After determining that the origin segment of the RCA was of sufficient length, and following adequate mobilization of the RCA and 1 min of ischemic preconditioning, the aorta was partially cross-clamped, and the RCA was clamped with vascular clips at both the emergence and a distal point. The RCA was then transected at a normal point proximal to the stenosis. A 5 mm hole was punched into the right coronary sinus, and the RCA was reimplanted in an end-to-side fashion using continuous 5-0 polypropylene sutures. The stub of the RCA was closed by oversewing it to the aortic wall using 7-0 polypropylene sutures. The total cross-clamping time for the RCA was 5 min. Hemodynamics were stable throughout the procedure, and the patient reported no postoperative complaints. Follow-up CT at 6 months confirmed that the repaired coronary artery remained patent. To the best of our knowledge, this is the first reported case of RCA reimplantation without cardiopulmonary bypass.

Studies of young athletes have identified anomalous coronary arteries (ACAs) as the second leading cause of sudden cardiac death, with anomalous left coronary artery from the right sinus (ALCA-R) and associated interarterial course (IAC) being the most common contributors (5, 6). Diagnosing these anomalies poses a significant challenge, as survivors of aborted cardiac death are frequently misdiagnosed with primary ventricular arrhythmia instead of an anomalous coronary origin (7). Although echocardiography can reveal an anomalous origin, coronary anatomy is rarely the primary focus in examinations of young individuals (8). In our study, patients with ALCA-R were notably younger and experienced a longer duration of symptoms before seeking medical care, despite severe cardiac symptoms. Reports also suggest that patients with anomalous right coronary artery from the left sinus (ARCA-L) are more likely to survive undiagnosed into adulthood compared to those with ALCA-R (9), aligning with our findings. Additionally, postoperative cardiac symptoms were more prevalent in ALCA-R patients, underscoring the need for heightened vigilance when assessing young patients with ischemic symptoms. The higher incidence of postoperative cardiac-type symptoms in ALCA-R patients may relate to incomplete revascularization, patient anatomy, and specific surgical challenges. While most postoperative symptoms were manageable with medication, some patients reported limited physical activity.

The 2018 American College of Cardiology (ACC) and American Heart Association (AHA) guidelines recommend revascularization for anomalous aortic origin of a coronary artery (AAOCA) patients with documented ischemia as a Class I indication (10). Various surgical approaches have been documented, with the unroofing technique being the most frequently performed.

This study presents retrospective data from a single center, subject to all inherent limitations of this design. The small sample size may have introduced type II statistical errors. A well-powered trial to evaluate optimal AAOCA management would be valuable in confirming these findings. The retrospective nature of the study and the absence of a control group (e.g., patients managed conservatively or non-surgically) make it difficult to assess whether surgical intervention, particularly the novel off-pump technique, offers superior outcomes compared to non-surgical approaches or traditional surgery. Due to the shorter follow-up duration, this study has limitations in assessing the long-term durability of AAOCA repairs. We plan to continue following our patient cohort to gain additional insight into long-term patency and survival, especially for younger patients. Future studies should consider extended follow-up to better evaluate surgical durability and outcomes. Given the low incidence of AAOCA, single-center data limit generalizability. We propose future studies should aim to collaborate across multiple centers to increase sample size and enhance statistical power, thus providing more widely applicable insights.

Patients with ALCA-R were significantly younger and had a longer duration of symptoms before seeking medical attention, despite experiencing severe cardiac symptoms. Additionally, the incidence of postoperative cardiac-type symptoms was higher in ALCA-R patients. Given the association of AAOCA with an increased risk of myocardial ischemia and infarction, as well as the high incidence of postoperative cardiac-type symptoms, greater attention should be paid to young patients presenting with ischemic symptoms. The selection of the surgical procedure should be based on specific anatomical details and the surgeon's experience, as this can lead to successful outcomes in AAOCA repair. In our opinion, CABG may not be the optimal choice for AAOCA, but more evidence is needed. When anatomically feasible, unroofing, reimplantation, and patch angioplasty are viable alternatives for this congenital anomaly.

To the best of our knowledge, this study reports the first case of ALCA-R treated with pulmonary artery patch angioplasty and the first case of RCA reimplantation without cardiopulmonary bypass. Both techniques present promising alternative surgical options with satisfactory outcomes.