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 Table of Contents  
Year : 2019  |  Volume : 3  |  Issue : 1  |  Page : 33-35

Rare coronary circulation anomaly causing chest pain: superdominant right coronary artery with absent left circumflex artery

1 Department of Radiodiagnosis, Medipulse Hospital, Jodhpur, Rajasthan, India
2 Department of Radiodiagnosis, SRL Diagnostics, Jodhpur, Rajasthan, India
3 Department of Cardiology, Medipulse Hospital, Jodhpur, Rajasthan, India

Date of Web Publication15-Mar-2019

Correspondence Address:
Sanjay Nathani
Medipulse Hospital, Basni, Jodhpur - 342 001, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_32_18

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Coronary circulation anomalies are the uncommon cause of chest pain when hemodynamically significant. Multidetector computed tomography (CT) of coronary arteries is rapidly replacing conventional angiography, nowadays, as the first line of investigation for the imaging of coronary artery disease due to its noninvasive nature. CT coronary angiography in addition to diagnosing anomalies of coronary arteries is especially good in the delineation of ostial origin and proximal course. Superdominant right coronary artery (RCA) with absent left circumflex artery (LCx) is such a rare congenital coronary artery anomaly which can mimic atherosclerotic disease clinically with very few case reports in the literature which can be diagnosed and evaluated with accuracy by CT angiography.

Keywords: Absent left circumflex coronary artery, coronary circulation anomaly, superdominant right coronary artery

How to cite this article:
Nathani S, Mehta P, Singh K, Golcha S. Rare coronary circulation anomaly causing chest pain: superdominant right coronary artery with absent left circumflex artery. J Indian Acad Echocardiogr Cardiovasc Imaging 2019;3:33-5

How to cite this URL:
Nathani S, Mehta P, Singh K, Golcha S. Rare coronary circulation anomaly causing chest pain: superdominant right coronary artery with absent left circumflex artery. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2019 [cited 2020 Nov 26];3:33-5. Available from: https://www.jiaecho.org/text.asp?2019/3/1/33/254253

  Introduction Top

Coronary artery anomalies are congenital variations in their origin, course, or structure and usually discovered as incidental findings on coronary angiographies. Multidetector computed tomography (CT) of coronary arteries is rapidly replacing catheter angiography as the first line of investigation for the imaging of coronary artery disease. CT coronary angiography in addition to detecting and characterizing atherosclerotic coronary artery disease is also a good imaging modality for the evaluation anomalies of coronary arteries. Superdominant right coronary artery (RCA) with absent left circumflex artery (LCx) is a very rare congenital coronary artery anomaly which can be diagnosed and evaluated with CT angiography with incidence of 0.003% in patients undergoing coronary angiography. Very few case reports are available in the literature.

  Clinical Presentation Top

An elderly nondiabetic hypertensive male of age 60 years presented to our hospital with intermittent chest pain related to exertion. The patient did not have any associated significant history or comorbidities. Routine blood investigations, echocardiogram (ECG), and treadmill test (TMT) at the time of hospital visit were within normal limits. A CT coronary angiogram was requested and performed using 64-slice Siemens Multislice CT scanner which revealed nonvisualization of the left circumflex coronary artery [Figure 1]. The RCA was of good caliber with a long tortuous course crossing the crux of the heart and then entering into the left atrioventricular groove. The posterolateral ventricular branches were arising from the RCA seen perfusing posterior and lateral walls of the left ventricle (in the usual vascular territory of the LCx). The patient was subsequently managed with conservative medical management and observed on regular follow-up without evidence of myocardial infarction on ECG.
Figure 1: Volume-rendered (a and b) and maximum intensity projection (c) images of heart demonstrate absent left circumflex artery (arrow) and superdominant right coronary artery gives off branches to the posterolateral wall of the left ventricle. RPLV: Right postero-lateral ventricular branch, RCA: Right coronary artery

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  Discussion Top

Coronary artery anomalies are uncommon and have been reported in 0.3%–5.6% of cases.[1],[2] They encompass a wide spectrum: some are hemodynamically insignificant and are often asymptomatic, whereas some are hemodynamically significant and may present with atypical chest pain, dyspnea, exertion-related syncope, or sometimes sudden cardiac death. In the normal coronary artery anatomy, the RCA originates from the right coronary sinus and supplies the free wall of the right ventricle. The left coronary artery trunk originates from the left coronary sinus and bifurcates into the anterior descending and LCxs. The anterior descending artery supplies the anterior wall and the interventricular septum and the circumflex artery supplies the free wall of the left ventricle.

Various classifications have been used in literature to classify coronary artery anomalies. Angelini et al.[3] proposed a classification of coronary anomalies as: (a) anomalies of origination and course, (b) anomalies of intrinsic coronary arterial anatomy, and (c) anomalies of coronary termination. Hemodynamically significant anomalies include atresia, origin from the pulmonary artery, interarterial course, and congenital fistula. Congenital coronary artery anomalies have been found to increase the risk of sudden death in young athletes.[4],[5],[6] Anomalies of coronary artery origination increase the risk for sudden death among young athletes 79 folds than in nonathlete individuals.[7]

Conventionally, conventional angiography has been considered as a gold standard for the evaluation of coronary artery anomalies, but with the emergence of multidetector scanners, CT of the coronary arteries has moved into the diagnostic realm due to its good spatial resolution and rapid noninvasive acquisition. Furthermore, with the development of ECG-synchronized scanning and new reconstruction techniques, the movement and cardiac pulsation artifacts can be minimized. Multislice CT not only helps in the diagnosis of these coronary artery anomalies but also provides detailed anatomy of adjacent structures with limitations related radiation exposure and potentially nephrotoxic or allergenic contrast agents.[8],[9] CT has been reported better for defining the ostial and proximal path of anomalous coronary arteries and their branches.

Atresia or absent coronary artery is a rare condition, most commonly described in the literature being congenital atresia of the left main coronary artery,[10] although atresia of RCA[11] and atresia of LCx coronary in association with a superdominant RCA[12] have also been reported. Absent LCx with superdominant RCA is a very rare coronary artery anomaly in which case the left main coronary artery continues as the left anterior descending artery (LAD) with a complete absence of the LCx and obtuse marginal branches. The superdominant RCA is large, crosses the crux of the heart coursing in the left atrioventricular groove, and gives off the branches to the posterior and lateral walls of the left ventricle (the territory that is normally supplied by the LCx).[9],[13] Not more than 25 cases of this anomaly have been reported according to a recent article providing a review of the literature.[14] In few of them, coronary CT angiography was performed for diagnosis of absent LCx. Our patient did not have any significant history or associated comorbidities; ECG and TMT were within normal limits at the time of hospital visit.

Most common differential diagnosis of congenitally absent LCx is complete occlusion of the LCx from its ostium in which case usually there would be a small contrast opacified “stump”-like appearance at its takeoff.[15] Superdominant RCA or prominent diagonal branches from the LAD supplying the posterior and lateral walls of the left ventricle are also helpful in differentiating these two conditions. In our case, no such “stump” was seen and the LCx was completely absent with prominent right posterior descending artery continuing in the left atrioventricular groove.

In our case study, the patient presented with the symptom of exertional chest pain which was thought to be due to transient ischemia of the left ventricular inferior and septal walls in conditions of increased oxygen demand.[8] Normally, these areas are supplied by the LCx; however, in the absence of the LCx, the oxygen demand of these areas is supplied by the RCA which may not be sufficient during physical exertion. Hence, the identification of this anomaly becomes important because the symptoms may mimic atherosclerotic coronary artery disease.

  Conclusion Top

All interventional cardiologists and radiologists performing angiographies should be familiar with these anatomic variants since accurate recognition of the course, and distribution of the coronary vessels is crucial for proper revascularization strategies in the presence of coronary artery disease.[16] Treatment of coronary artery anomalies is dependent on the type of anomaly, age, and presentation of the patient.[17] In young adults, the aim of treatment is to prevent sudden death. In symptomatic patients, most authors would advise revascularization, whereas in asymptomatic patients, though the literature is less clear, conservative management may be opted with option of revascularization. In older patients who are not athletically active, the risk of sudden death is probably not clinically significant, so our aim in these patients is to treat symptoms; however, each patient should be considered individually.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Angelini P. Coronary artery anomalies: An entity in search of an identity. Circulation 2007;115:1296-305.  Back to cited text no. 1
Kim SY, Seo JB, Do KH, Heo JN, Lee JS, Song JW, et al. Coronary artery anomalies: Classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics 2006;26:317-33.  Back to cited text no. 2
Angelini P, Velasco JA, Flamm S. Coronary anomalies: Incidence, pathophysiology, and clinical relevance. Circulation 2002;105:2449-54.  Back to cited text no. 3
Shriki JE, Shinbane JS, Rashid MA, Hindoyan A, Withey JG, DeFrance A, et al. Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics 2012;32:453-68.  Back to cited text no. 4
Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A, et al. Coronary artery anomalies overview: The normal and the abnormal. World J Radiol 2016;8:537-55.  Back to cited text no. 5
Kastellanos S, Aznaouridis K, Vlachopoulos C, Tsiamis E, Oikonomou E, Tousoulis D, et al. Overview of coronary artery variants, aberrations and anomalies. World J Cardiol 2018;10:127-40.  Back to cited text no. 6
Krasuski RA, Magyar D, Hart S, Kalahasti V, Lorber R, Hobbs R, et al. Long-term outcome and impact of surgery on adults with coronary arteries originating from the opposite coronary cusp. Circulation 2011;123:154-62.  Back to cited text no. 7
Majid Y, Warade M, Sinha J, Kalyanpur A, Gupta T. Superdominant right coronary artery with absent left circumflex artery. Biomed Imaging Interv J 2011;7:e2.  Back to cited text no. 8
Alexander RW, Griffith GC. Anomalies of the coronary arteries and their clinical significance. Circulation 1956;14:800-5.  Back to cited text no. 9
Fortuin NJ, Roberts WC. Congenital atresia of the left main coronary artery. Am J Med 1971;50:385-9.  Back to cited text no. 10
Karadag B, Ayan F, Ismailoglu Z, Goksedef D, Ataev Y, Vural VA, et al. Extraordinary cause of ischemic chest pain in a young man: Congenital ostial atresia of the right coronary artery. J Cardiol 2009;54:335-8.  Back to cited text no. 11
Ilia R, Jafari J, Weinstein JM, Battler A. Absent left circumflex coronary artery. Cathet Cardiovasc Diagn 1994;32:349-50.  Back to cited text no. 12
Ali FS, Khan SA, Tai JM, Fatimi SH, Dhakam SH. Congenital absence of left circumflex artery with a dominant right coronary artery. BMJ Case Rep 2009;2009. pii: bcr08.2008.0772.  Back to cited text no. 13
Fugar S, Issac L, Okoh AK, Chedrawy C, Hangouche NE, Yadav N, et al. Congenital absence of left circumflex artery: A case report and review of the literature. Case Rep Cardiol 2017;2017:6579847.  Back to cited text no. 14
Varela D, Teleb M, Said S, Fan J, Mukherjee D, Abbas A, et al. Congenital absence of left circumflex presenting after an emotional stressor. Pol J Radiol 2015;80:529-31.  Back to cited text no. 15
Maheshwari M, Mittal SR. Superdominant right coronary artery with double posterior descending artery. Heart Views 2015;16:19-20.  Back to cited text no. 16
[PUBMED]  [Full text]  
Ali M, Hanley A, McFadden EP, Vaughan CJ. Coronary artery anomalies: A practical approach to diagnosis and management. Heart Asia 2011;3:8-12.  Back to cited text no. 17


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