Description
A 53-year-old man with a history of tobacco and alcohol abuse, presented to the emergency department with intermittent atypical chest pain and exertional dyspnoea for several days. His physical examination, electrocardiogram (ECG) and cardiac biomarkers were normal. He underwent exercise stress echo with an average exercise capacity and without inducible angina or ECG changes. The stress images were suboptimal but suggested ischaemia in the inferior wall; therefore, he was referred for selective coronary angiogram. Interestingly, this revealed anomalous coronary arteries (ACAs) with the right coronary artery (RCA), left anterior descending (LAD) and left circumflex (LCX) arteries all arising from the right sinus of Valsalva (videos 1–7). The ejection fraction was normal and there was no obstructive coronary disease.
Video 1.
Selective coronary angiogram using 5F FL4 Catheter (Boston Scientific) in left anterior oblique projection showing no coronary artery originating from the left coronary cusp.
Video 2.
Selective coronary angiogram using 5F FR4 Catheter (Boston Scientific) in left anterior oblique projection showing normal right coronary artery originating from the right coronary cusp.
Video 3.
Selective coronary angiogram using 5F FR4 Catheter (Boston Scientific) in left anterior oblique and cranial projection showing normal right coronary artery originating from the right coronary cusp.
Video 4.
Selective coronary angiogram using 5F FR4 Catheter (Boston Scientific) in the left anterior oblique projection showing the left anterior descending artery originating from the right coronary cusp.
Video 5.
Selective coronary angiogram using 5F FR4 Catheter (Boston Scientific) in right anterior oblique projection showing the anterior course of the left anterior descending artery.
Video 6.
Selective coronary angiogram using 5F MPA2 Catheter (Boston Scientific) in left anterior oblique projection showing the left circumflex artery originating from the right coronary cusp with posterior course.
Video 7.
Selective coronary angiogram using 5F MPA2 Catheter (Boston Scientific) in right anterior oblique projection showing the left circumflex artery originating from the right coronary cusp with posterior course.
To further identify the proximal course of these anomalous arteries, CT angiography was performed. This confirmed that the RCA, LAD and LCX all arose independently from separate ostia in the right coronary cusp. None of the ACAs demonstrated an inter-arterial course between the aorta and the pulmonary trunk. In fact, the LAD was found to take a course anterior to the pulmonary artery and the LCX was coursing posterior to the aortic root (figure 1A, B). The RCA was a dominant vessel. The patient was discharged home with extensive counselling about lifestyle modifications, and alcohol and smoking cessation.
Learning points.
Anomalous coronary arteries (ACAs) without any other congenital heart defects is a rare condition with a reported incidence varying between 0.3% and 1% in the general population.1 Anomalous origin of all three major coronary arteries from the right sinus of Valsalva has been described as ‘exceedingly rare’ with an incidence of 3.1% of those patients with known ACAs.2
An ACA that originates from the opposite sinus and courses between the aorta and the pulmonary trunk (inter-arterial course) has been associated with sudden cardiac death during exercise. This could be attributed to several issues compromising the flow in the ACA, such as multiple curves or angulations, abnormal ostium or compression between the pulmonary artery and aorta as a result of increased flow in these major arteries with exertion.
Anomalous origin of the coronary artery should be suspected during an angiogram in the absence of any coronary artery originating from the right or left sinuses of Valsalva. Cardiologists and general practitioners need to be well aware of the various anomalies of the coronary arteries and able to recognise the type associated with sudden cardiac death.
Defining the proximal origin and the course of the ACA is an essential part of the evaluation to estimate the clinical impact and plan subsequent management. Cardiac MRI or CT angiography are extremely helpful tools that could be utilised for this purpose.
Figure 1.
(A and B) Multi-planar reformatted (A), and volume rendered (B) CT images showing separate origins of all three coronaries from the right coronary sinus. AO, aorta; PA, pulmonary artery; LA, left atrium; LAD, left anterior descending artery; RCA, right coronary artery; LCX, left circumflex artery.
Footnotes
Contributors: MI wrote the manuscript and collected all images and clips. RR performed the coronary angiogram. SCK provided care for the patient during his hospitalisation. MFM read the coronary CT and edited the manuscript. All the coauthors have reviewed the manuscript and agreed with its contents.
Competing interests: None declared.
Patient consent: Obtained.
Provenance and peer review: Not commissioned; externally peer reviewed.
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.Patel KB, Gupta H, Nath H et al. Origin of all three major coronary arteries from the right sinus of Valsalva: clinical, angiographic, and magnetic resonance imaging findings and incidence in a select referral population. Catheter Cardiovasc Interv 2007;69:711–18. doi:10.1002/ccd.21078 [DOI] [PubMed] [Google Scholar]