Abstract
This case report presents a rare scenario involving a congenital anomaly of the right coronary artery’s (RCA) origin in association with an ascending aortic aneurysm. While both anomalies are individually recognized in the literature, their coexistence and potential interplay remain understudied. The aim of this report is to emphasize the challenges and implications associated with such a combination. A 78-year-old male patient with an enlarged ascending aortic aneurysm necessitating surgical intervention was found to have an anomalous origin of the RCA during preoperative coronary angiography, confirmed by computed tomography scan. Transesophageal echocardiography further elucidated the coronary abnormality. Intraoperatively, successful aortic replacement was performed, and careful repositioning of the anomalous right coronary ostium was achieved. This case raises important considerations regarding the potential complications arising from coronary anomalies and their impact on the surgical management of ascending aortic aneurysms. The rarity of this combination limits our understanding of their association, making a multidisciplinary approach crucial for optimal patient care. Further research and comprehensive evaluation of similar cases are necessary to better understand the relationship between coronary anomalies and ascending aortic aneurysms. Such investigations will help in improving treatment strategies and outcomes for patients with these complex conditions.
Keywords: Anomalous right coronary artery, ascending aortic aneurysm, congenital coronary anomaly
INTRODUCTION
Congenital coronary anomalies are rare cardiac conditions in which the coronary artery may be malpositioned, have an abnormal course, or exhibit other structural variations.
While the overall incidence of right coronary artery (RCA) anomalies ranges from 0.15% to 1.2% in the general population, the specific case we present, where the RCA originates from the proximal portion of the ascending aorta, has an incidence in the general population of 0.03%–0.50%, making it an extremely rare occurrence.[1,2]
Congenital coronary anomalies can be classified into different categories based on their clinical presentation and impact on blood flow. Some anomalies may be asymptomatic and require no treatment, while others can cause serious cardiac problems, including angina, arrhythmias, ischemia, or sudden cardiac arrest death.
Accurate diagnosis of congenital coronary anomalies requires a thorough evaluation of cardiac anatomy using advanced imaging techniques such as coronary angiography, computed tomography (CT), or magnetic resonance imaging.
The treatment of coronary anomalies depends on the severity of the condition and the clinical manifestations. In some cases, surgical intervention may be necessary to restore normal blood flow to the heart, while in others, pharmacological treatment may be sufficient to manage symptoms.
Despite their rarity, congenital coronary anomalies present significant clinical challenges, and understanding the implications of these anomalies is crucial for proper management.
CASE REPORT
A 78-year-old male patient was admitted for evaluation of a 55-mm ascending aortic aneurysm, which had significantly increased in size over the past year. The diagnosis was made with a CT angiography, performed in another center. Due to the marked enlargement, surgical treatment was indicated according to the current ESC/EACTS guidelines.[3]
Before the scheduled surgery, a coronary angiography was performed. The anatomy of the left coronary artery was found to be normal, whereas there was difficulty in cannulating the ostium of the RCA, suggesting an underlying anomaly.
The subsequent aortography confirmed an anomalous origin of the RCA [Figure 1a and b]. The anomalous RCA arose anteriorly from the tubular segment of the ascending aorta, above the left sinus. This finding explained the challenges encountered during the cannulation of the RCA during coronary angiography.
Figure 1.

The coronary angiography showed a normal left coronary artery (a), whereas the right coronary artery was not in the usual position. (b) The aortography showed the anomalous origin of the right coronary artery arising from the ascending aorta and descending vertically to then take its normal course (white arrows)
After assessing again the images of CT scan, we found the presence of anomalous origin of the RCA [Figure 2a and b].
Figure 2.

The computed tomography scan coronal image of ascending aorta (a) revealed the origin of the right coronary artery from the tubular tract on the left side (black arrow) passing anteriorly of the aortic wall (b), from which is clearly separated (white arrow)
This particular anomaly, where the RCA arises from the anterior aspect of the tubular segment of the ascending aorta, is a really rare finding representing a deviation from the normal anatomical arrangement of the coronary arteries.
The presence of this coronary anomaly raises additional considerations for the surgical procedure planned to address the ascending aortic aneurysm. The surgical team needed to carefully evaluate the course and relationship of the anomalous RCA to ensure the preservation of adequate blood supply to the heart during the intervention.
Given the patient’s age and the concurrent presence of the ascending aortic aneurysm and coronary anomaly, a multidisciplinary approach involving cardiovascular surgeons, interventional cardiologists, and echocardiographers was crucial to optimize the surgical strategy and minimize the risk of complications.
The preoperative transesophageal echocardiogram revealed normal structure and morphology of the semilunar valves and no dilation of the aortic annulus. In addition, the anomalous ostium of the RCA was clearly visualized over the sinotubular junction [Figure 3a and b].
Figure 3.
The three-dimensional transesophageal echocardiography image of the aortic valve and root as seen from the ascending aorta revealed the normal origin of the left coronary artery (white arrow) and the absence of the right coronary artery (a), which was found in the long-axis view 120 (b) arising from the tubular segment of the ascending aorta (white arrow)
Based on these findings, it was decided to spare the valve and replace the dilated segment of the ascending aorta with repositioning of the right coronary ostium [Figure 4a and b].
Figure 4.

The right coronary artery arises from the anterior wall of the ascending aorta and it is encircled with a blue vessel loop (b). After opening the aorta, the ostium was clearly seen (black arrow) and isolated (a)
After replacement of the ascending aorta and reimplantation of the right coronary ostium on the tubular graft, the extracorporeal circulation was discontinued. At that moment, there was evidence of ST-segment elevation in the inferior leads, indicating a potential myocardial ischemia. To address this, it became necessary to perform a beating heart aortocoronary bypass grafting with a saphenous vein graft to restore blood flow to the RCA. Afterward, a normalization of the electrocardiogram was seen at the end of the procedure suggesting a successful reperfusion of the myocardium and resolution of the ischemic event.
DISCUSSION
The presented case highlights an almost unique instance of a congenital anomaly of the RCA origin associated with a lesser-studied condition, ascending aortic aneurysm. Congenital coronary anomalies are rare, and they can pose significant diagnostic and management challenges.[4,5,6,7] This specific case raises several considerations regarding the potential complications related to coronary anomalies and ascending aortic aneurysms.
The combination of these two pathological conditions, the anomalous origin of the RCA and the ascending aortic aneurysm, represents a rare occurrence that warrants further investigation to better understand their relationship and potential association. Due to the limited number of reported cases in the literature, it is currently not possible to ascertain with certainty whether these two conditions are linked or independent of each other.[8,9,10,11]
Nevertheless, it is important to emphasize that the presence of an anomalous origin of the RCA can complicate the surgical management of ascending aortic aneurysms. These aneurysms may be burdened with postsurgical ischemic complications due to kinking or extrinsic compression of the repositioned coronary artery. This underscores the significance of a multidisciplinary evaluation in such cases.
CONCLUSIONS
This case highlights the rare finding of an anomalous origin of the RCA from the anterior wall of the tubular segment of the ascending aorta, above the left sinus. The patient’s clinical management will require careful consideration of both the ascending aortic aneurysm and the associated coronary anomaly to ensure an optimal surgical outcome.
Further research and case studies are needed to explore the relationship between coronary anomalies and ascending aortic aneurysms, their potential impact on surgical outcomes, and the optimal management strategies. A multidisciplinary approach involving cardiovascular surgeons, interventional cardiologists, and imaging specialists is crucial to ensure comprehensive evaluation and individualized treatment plans for patients with these complex and rare conditions. Although this multidisciplinary approach, the surgeons have to be always ready to deal with unforeseen complications as ischemia in the territory of the implanted RCA, which are impossible to predict during the preoperative planning.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
REFERENCES
- 1.Angelini P, Velasco JA, Flamm S. Coronary anomalies:Incidence, pathophysiology, and clinical relevance. Circulation. 2002;105:2449–54. doi: 10.1161/01.cir.0000016175.49835.57. [DOI] [PubMed] [Google Scholar]
- 2.Angelini P, Villason S, Chan AV, Jr, Diez JG. Coronary Artery Anomalies: A Comprehensive Approach. 1st ed. Philadelphia: Lippincott Williams and Wilkins; 1999. Normal and anomalous coronary arteries in humans; pp. 27–79. Chapter 4. [Google Scholar]
- 3.Vahanian A, Beyersdorf F, Praz F, Milojevic M, Baldus S, Bauersachs J, et al. 2021 ESC/EACTS guidelines for the management of valvular heart disease. Eur Heart J. 2022;43:561–632. doi: 10.1093/eurheartj/ehab395. [DOI] [PubMed] [Google Scholar]
- 4.Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493–501. doi: 10.1016/s0735-1097(00)00566-0. [DOI] [PubMed] [Google Scholar]
- 5.Brothers JA, McBride MG, Seliem MA, Marino BS, Tomlicon RS, Pampaloni MH, et al. Evaluation of Myocardial Ischemia After Surgical Repair of Anoumalous Aortic Origina of a Coronary Artery in a Series of Pediatric Patients. J. Am Coll. Cardiol. 2007;50:2078–82. doi: 10.1016/j.jacc.2007.06.055. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long-term management of Kawasaki disease:A scientific statement for health professionals from the American heart association. Circulation. 2017;135:e927–99. doi: 10.1161/CIR.0000000000000484. [DOI] [PubMed] [Google Scholar]
- 7.Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease:A report of the American College of Cardiology/American Heart Association task force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease) Circulation. 2008;118:e714–833. doi: 10.1161/CIRCULATIONAHA.108.190690. [DOI] [PubMed] [Google Scholar]
- 8.Kimbiris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic origin of coronary arteries. Circulation. 1978;58:606–15. doi: 10.1161/01.cir.58.4.606. [DOI] [PubMed] [Google Scholar]
- 9.de Gregorio C, Ceresa F, Ferrazzo G, Patanè F. Malignant right coronary artery origin from the left sinus of Valsalva:Complementary role for transesophageal echocardiography upon the cath-lab diagnosis. J Clin Ultrasound. 2021;49:167–9. doi: 10.1002/jcu.22845. [DOI] [PubMed] [Google Scholar]
- 10.Angelini P. Coronary artery anomalies:An entity in search of an identity. Circulation. 2007;115:1296–305. doi: 10.1161/CIRCULATIONAHA.106.618082. [DOI] [PubMed] [Google Scholar]
- 11.Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies overview:The normal and abnormal. World J. Radiol. 2016;8:537–55. doi: 10.4329/wjr.v8.i6.537. [DOI] [PMC free article] [PubMed] [Google Scholar]

