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Image of the Month September 2025: Anomalous origin of the Right Coronary Artery from the Left Sinus of Valsalva: a rare case of coronary anomaly

October 2025

Author

Dr Phyo Khaing, British Heart Foundation Centre of Research Excellence, University of Edinburgh, United Kingdom

Case history:

A 61-years old lady underwent a research computed tomography coronary angiogram (CTCA) as per the allocated trial randomisation. She was generally fit and well, and has a history of mild hypercholesterolaemia. Her research CTCA revealed an incidental finding of a rare coronary anomaly with no evidence of coronary atheroma. She had an anomalous origin of the right coronary artery arising from the left sinus of Valsalva with an intra-arterial course. Given she was entirely asymptomatic at the age of 61, her risk of sudden cardiac death was deemed low and she was managed conservatively.

Discussion:

Coronary artery anomalies are rare congenital variations, often detected incidentally during CT coronary angiography and are present in less than 2% of the population¹. The incidence of anomalous origin of the right coronary artery is more common than anomalous origin of the left coronary artery². Although patients are usually asymptomatic, coronary anomalies can present with exertional syncope, angina, palpitations and sudden cardiac death depending on the level of patient’s activity and course of the vessel. Certain anatomical features are typically considered higher risk, and these include an acute angulation at the coronary origin, a slit-like ostium, an initial intramural course within the aortic wall, and an interarterial course between the aorta and pulmonary trunk³.

Previous research based on a registry of 6.3 million military recruits showed that among 126 non-traumatic deaths, 21 were attributed to coronary artery anomalies⁴. Amongst all these coronary artery anomaly deaths, the abnormality was the left coronary artery arising from the right coronary sinus with an interarterial course.

Several mechanisms have been proposed to explain the myocardial ischaemia and sudden cardiac death in anomalous interarterial courses. These include dynamic compression of the coronary artery between the great vessels during intense exertion, transient occlusion at the ostium due to an acute angulation, and hypoplasia of the anomalous coronary artery at its ostium⁵.

In contrast to left sided coronary anomalies, an anomalous right coronary artery arising from the left sinus of Valsalva is generally considered to have a more favourable prognosis. In this case, the risk of sudden cardiac death is lower when identified later in life without any troublesome symptoms.

Questions:

What does ALCAPA stand for?

1. Anomalous Left Coronary Artery from the Pulmonary Artery
2. Anomalous Left Circumflex Artery from the Pulmonary Artery
3. Anomalous Left Coronary Artery from the Posterior Aorta
4. Anomalous Left Circumflex Artery from the Posterior Aorta

Which surgical approach is commonly used to treat a high risk coronary anomaly if necessary?

1. Aortic root replacement
2. Septal myectomy
3. Coronary unroofing
4. Shunting

Which of the following courses is generally considered benign?

1. Interarterial course
2. Intramural course
3. Retroaortic course
4. Acute angle takeoff

Answers: a, c, c

References:

1. Cheezum MK, Ghoshhajra B, Bittencourt MS, Hulten EA, Bhatt A, Mousavi N, Shah NR, Valente AM, Rybicki FJ, Steigner M, Hainer J, MacGillivray T, Hoffmann U, Abbara S, Di Carli MF, DeFaria Yeh D, Landzberg M, Liberthson R, Blankstein R. Anomalous origin of the coronary artery arising from the opposite sinus: prevalence and outcomes in patients undergoing coronary CTA. Eur Heart J Cardiovasc Imaging. 2017 Feb;18(2):224-235. doi: 10.1093/ehjci/jev323. Epub 2016 Feb 3. PMID: 26848152; PMCID: PMC6279103.
2. Heo W, Min HK, Kang DK, Jun HJ, Hwang YH, Lee HC. Three different situations and approaches in the management for anomalous origin of the right coronary artery from the left coronary sinus: case report. J Cardiothorac Surg. 2014 Jan 23;9:21. doi: 10.1186/1749-8090-9-21. PMID: 24450442; PMCID: PMC3902410.
3. Bhatia, R.T., Forster, J., Ackrill, M.et al.Coronary artery anomalies and the role of echocardiography in pre-participation screening of athletes: a practical guide. Echo Res Pract 11, 5 (2024). https://doi.org/10.1186/s44156-024-00041-4
4. Eckart RE, Scoville SL, Campbell CL, Shry EA, Stajduhar KC, Potter RN, Pearse LA, Virmani R. Sudden death in young adults: a 25-year review of autopsies in military recruits. Ann Intern Med. 2004 Dec 7;141(11):829-34. doi: 10.7326/0003-4819-141-11-200412070-00005. PMID: 15583223.
5. Fuglsang S, Heiberg J, Byg J, Hjortdal VE. Anomalous origin of the right coronary artery with an interarterial course and intramural part. Int J Surg Case Rep. 2015;14:92-4. doi: 10.1016/j.ijscr.2015.07.018. Epub 2015 Jul 28. PMID: 26255002; PMCID: PMC5963140.