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

Abstract

Anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS) are a challenge because of their various anatomic and clinical presentation. Although the prevalence is low, the absolute numbers of detected ACAOS are increasing because of the growing use of noninvasive anatomical imaging for ruling out coronary artery disease. As evidence-based guidelines are lacking, treating physicians are left in uncertainty for the optimal management of such patients. The sole presence of ACAOS does not justify surgical correction, and therefore a thorough anatomic and hemodynamic assessment is warranted. Invasive and noninvasive multimodality imaging provides information to the clinical question whether the presence of ACAOS is an innocent coincidental finding, is responsible for the patient's symptoms, or even might be a risk for sudden cardiac death. Based on recent clinical data, focusing on the pathophysiology of patients with ACAOS, myocardial ischemia is dependent on both the extent of fixed and dynamic components, represented by anatomic high-risk features. These varying combinations should be considered individually in the decision making for the different therapeutic options. This state-of-the-art review focuses on the advantages and limitations of the common contemporary surgical, interventional, and medical therapy with regard to the anatomy and pathophysiology of ACAOS. Further, we propose a therapeutic management algorithm based on current evidence on multimodality invasive and noninvasive imaging findings and highlight remaining gaps of knowledge.

Keywords: ACAOS; L‐ACAOS; PCI; R‐ACAOS; anomalous coronary artery originating from the opposite sinus of Valsalva; coronary unroofing.

Figures

Figure 1. Illustration with variants of proximal coronary artery anatomy.

A, The normal anatomic origin and intraparietal aortic wall course of a coronary artery, and the exit angle is equal to ≈90°. B, The abnormal angulated orifice: the coronary vessel passes obliquely through the aortic wall for a distance (a) that is equal or inferior to the coronary artery’s diameter (b), and the exit angle is <90°. C, The abnormal intramural course: the coronary vessel passes obliquely through the aortic wall for a distance (a), which is greater than the coronary artery’s diameter (b), and the exit angle is about >0° to 45°. Of note, proximal narrowing exceeds the intramural course in most cases. Hence, juxta‐aortic calibers are frequently smaller than the distal reference calibers.

Figure 2. Comparison of the diagnostic management in 2 similar cases of R‐ACAOS.

A, A 65‐year‐old woman with atypical chest pain and exertional dyspnea (New York Heart Association class II) since 4 months. Normal electrocardiographic and echocardiographic (LVEF 60%) findings, bicycle exercise testing with exercise‐induced dyspnea without ischemic ECG alterations at heart rate (HR) 154/min (99% of maximum HR). A1 and A2, Coronary computed tomography angiography showing absence of coronary artery diseases (CAD), a R‐ACAOS with acute take‐off angle (18.3°), an intramural course (7.8 mm) and an elliptic vessel shape (ratio 2.8). No slitlike ostium or proximal narrowing could be illustrated. A3, Single‐photon emission computed tomography with physical stress showing no exercise‐induced myocardial ischemia at a HR of 155/min. A4, Invasive coronary angiography illustrating both the anomalous and the left coronary artery with one injection (projection: right anterior‐oblique 2°, caudal 8°). A5, Invasive physiologic assessment with FFR during a dobutamine‐volume challenge demonstrating absence of hemodynamic relevance. B, A 48‐year‐old man with fatigue, atypical chest pain, and occasionally dyspnea/palpitations since 6 months. Normal electrocardiographic and echocardiographic (LVEF 60%) findings, bicycle exercise testing without symptoms or ischemic ECG alterations at max. HR of 167/min (97% of maximum HR), B1 through B3, CCTA showing absence of CAD, an R‐ACAOS with acute take‐off angle (3.9°), an intramural course (14.3 mm), an elliptic vessel shape (ratio 3.4) as well as a slitlike ostium. No relevant proximal narrowing could be illustrated. B4 and B5, Illustration of the oval vessel shape within the intramural course (B4) and normalization of the round vessel shape at the distal reference site (B5). B6, Invasive coronary angiography illustrating both the anomalous and the left coronary artery with 1 injection (projection: left anterior‐oblique 84°, caudal 7°) Invasive physiologic assessment with FFRDobutamine demonstrating hemodynamic relevance of the anomalous course. CAD indicates coronary disease; CCTA, coronary computed tomography angiography; FFR, fractional flow reserve; LVEF, left ventricular ejection fraction; and R‐ACAOS, right anomalous coronary artery originating from the opposite sinus of Valsalva.

Figure 3. Illustration of the different surgical procedures.

A, Coronary unroofing. Exploration of the intramural course using a coronary probe is followed by excision/incision of the common wall with the aorta over the probe along its entire intramural course proximal to the site where it emerges from the aortic wall. Then, a neo‐ostium is formed. B, Coronary translocation/reimplantation. After mobilization of the anomalous coronary artery, a transection with an aortic button and reimplantation in the “correct” sinus of Valsalva using an aortic punch is performed. C, Coronary ostioplasty. After transection of the aortic wall, an incision from the cut edge of the aorta is made into the ostium of the anomalous coronary artery and further extended into the artery itself. Then, a patch is sutured into this incision, resulting in the creation of a neo‐ostium as well as an enlarged proximal segment D, Pulmonary artery translocation. The main pulmonary artery is divided at its bifurcation and moved to the left, while a patch suture enlarges the pulmonary artery confluence (lateral approach). Alternatively, pulmonary artery translocation can be performed by moving the pulmonary arteries anterior to the aorta (anterior approach also called modified LeCompte maneuver). Panel (A) is adapted from Borns et al. Front Cardiovasc Med. 2021 Jan 21;7:591326. All images are drawn by Professor Alexander Kadner. LCA indicates left coronary artery; and LPA, left pulmonary artery.

Figure 4. Illustration of a percutaneous coronary intervention in a right anomalous coronary artery originating from the opposite sinus of Valsalva. (A and B).

Invasive coronary angiography illustrating the proximal narrowing within the intramural course from two projections (A, right anterior oblique 40°, caudal 8°; and B, right anterior oblique 46°, cranial 4°). C and D, Postinterventional CCTA illustrating the correct placement of the 2 stents within the intramural course. C, ostial cross‐section, D, distal cross‐section. E, 3‐dimensional reconstruction from the postinterventional CCTA‐images. CCTA indicates coronary computed tomography angiography; LAD, left anterior descending (coronary artery); LCX, left circumflex (coronary artery); PA, pulmonary artery; and RCA, right coronary artery.

Figure 5

Flow chart of the therapeutic management in patients with an anomalous coronary artery and confirmed myocardial ischemia related to ACAOS. ACAOS indicates anomalous coronary artery originating from the opposite sinus of Valsalva; CAD, coronary artery disease; CABG, coronary artery bypass grafting; CCTA, coronary computed tomography angiography; CMR, cardiovascular magnetic resonance imaging; FFR, fractional flow reserve; IVUS, intravascular ultrasound; OCT, optical coherence tomography; PCI, percutaneous coronary intervention; PET, positron emission tomography; and SPECT, single‐photon emission computed tomography.