Slide 1: Which patient could be at risk for sudden cardiac death? Two patients present to the ED with chest pain. ACS workup is negative. Coronary CTA is preformed for further risk assessment.

Slide 2: Which patient could be at risk for sudden cardiac death? Normal coronary anatomy. RCA. Aorta. LCA. Anomalous right coronary artery. RCA. LCA. Aorta. This patient has an anomalous origin of the right coronary artery (RCA) off the left sinus of valsalva. Malignant courses of the coronary arteries have been reported as the second most common cause of sudden cardiac death among young athletes.

Slide 3: First let us examine normal coronary anatomy. The region of the aorta just above the aortic valve is called the Sinus of Valsalva. Diagram showing aorta, heart, RCA, LCA, sinus of valsalva, main pulmonary artery, right coronary cusp, non-coronary cusp, left coronary cusp.

This level is divided into three valve leaflets, containing origins of the left and right main coronary arteries. Anomalies in this anatomy have been reported in 1-2% of the population.

Slide 4: Most variants of anomalous coronary anatomy are benign. What are features of malignant variants? Legend. ALCA = anomalous left coronary artery. ARCA – anomalous right coronary artery. Malignant variants that have been associated with sudden cardiac death arise when a coronary artery courses between the aorta and the pulmonary artery (especially the inter-arterial ALCA). Graphic showing some benign variants. Retro-aortic ARCA and Pre-pulmonic ARCA. or Pre-pulmonic ALCA and retro-aortic ALCA. Graphic showing malignant variants. Inter-arterial ARCA. Inter-arterial ALCA.

Slide 5: The exact reason for increased SCD risk is not exactly understood. These are common mechanistic theories: Here, we are looking at the openings of the coronaries from inside the aorta, notice how the abnormal angle origin of the vessel creates a slit-like opening, which can limit blood flow!

Looking at the vessel from above, notice how, due to the anomalous insertion of the coronary, the proximal aspect of the artery is squeezed by the walls of the aorta.

Slide 6: Some patients may benefit from surgical management of anomalous coronary arteries. Debate exist as to the correct management of anomalous coronary arteries. Factors favoring surgical management. Age. Decreased. Symptoms. Chest pain. EKG. Ischemic features. Features of CTA. Size of opening. Decreased. Intramural length. Increased. Angle of takeoff. Decreased. Type of course. Inter-arterial.

Typically, older patients with an incidentally discovered anomaly can be managed conservatively, while younger patients at higher risk of SCP and may benefit from de-roofing and repositioning.


  • Gräni C, Kaufmann PA, Windecker S, Buechel RR. Diagnosis and Management of Anomalous Coronary Arteries with a Malignant Course. Interv Cardiol. 2019 May 21;14(2):83-88. PMID 31178934.
  • Shriki JE, Shinbane JS, Rashid MA, Hindoyan A, Withey JG, DeFrance A, Cunningham M, Oliveira GR, Warren BH, Wilcox A. Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics. 2012 Mar-Apr;32(2):453-68. PMID 22411942.

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