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DOI: 10.1055/s-0041-1739381
Superdominant Right Coronary Artery with Absent Left Coronary Artery and Left Circumflex Artery with Anomalous Left Anterior Descending Artery
Authors
Abstract
The occurrence of super-dominant “single coronary artery” is an extremely rare and seldom reported phenomenon. The heart is dependent on a single vessel which makes its occlusion, if present, catastrophic. Here, the authors present an extremely rare combination of superdominant right coronary artery coexisting with absent left coronary artery and left circumflex artery with abnormal origin of left anterior descending artery from right coronary sinus. Precise morphological and physiological knowledge and evaluation of these anomalies is a must for opting the best available therapeutic modality and better prognosis.
Keywords
left circumflex artery - coronary angiography - coronary artery - superdominant right coronary arteryIntroduction
The occurrence of super-dominant “single coronary artery” is an extremely rare and seldom reported phenomenon. These are detected incidentally while evaluating the patient for atherosclerotic coronary artery disease (CAD) with an incidence of 0.05%. The clinical importance of having a superdominant vessel is increased dependence of heart on one vessel, which makes its occlusion, if present, catastrophic.
Case History
We report a 50-year-old nondiabetic non-hypertensive female who presented with complaints of palpitations and episodic dyspnea for 1 year. Her vitals were stable. ECG showed Qs waves in lead II, III, and aVF and ST elevation in lead III and aVF (inferior wall ischemia). Cardiac biomarkers were normal. 2D-echocardiography showed regional wall motion abnormality and ejection fraction: 57%. Routine blood tests were normal. Computed tomography (CT) and invasive coronary angiography were done subsequently with suspicion of CAD. CT coronary angiography showed the prominent and dilated right coronary artery (RCA) originating from the right coronary sinus and giving off a prominent marginal artery ([Fig. 1]). After coursing through posterior right atrioventricular (AV) groove and posterior cardiac crux, it was entering posterior left AV groove, running parallel to coronary sinus (the usual course of LCX) ([Fig. 2]), then continuing in anterior left AV groove ending at anterior cardiac crux. LCA and LCX were absent ([Fig. 3]). LAD originated from the right coronary sinus with separate ostium. It appeared attenuated and revealed focal kink followed by a tiny saccular aneurysm in front of the right ventricular outflow tract (RVOT) and thereafter coursing along the anterior surface of the right ventricle (demonstrating the anterior free wall course) ([Figs. 1] and [4]). Invasive coronary angiogram showed similar findings ([Fig. 5]).










Discussion
Coronary artery anomaly (CAA) is defined as any morphological feature of coronary arterial system with prevalence of <1%. The incidence of CAAs varies from 0.3 to 5.6%.[1] [2] [3]
Single coronary artery (SCA) is one such rare anomaly with an incidence of 0.05%.[4] [5] In SCA, only one coronary artery arises from aorta by a single ostium, supplying the whole heart. A detailed classification was proposed by Lipton et al, later modified by Yamanaka and Hobbs. According to this, Group I coronary arteries are super-dominant which follows the course of either RCA or LCA.[6] [7]
Superdominant RCA (R-I variant of Lipton classification) is the seldom reported anomaly. It is almost always associated with the congenital absence of LCX as a part of anatomical compensation to meet the demands of usual territory of LCX, such that approximately 90.4% of patients with congenitally absent LCX have super-dominant RCA.[8] The congenitally absent LCX itself has reported incidence of 0.067%.[9]
The origin of LAD from the right coronary sinus is another rare anomaly. It is usually seen associated with various congenital heart diseases like tetralogy of Fallot or double outlet left ventricle as LAD is influenced by pulmonary conus development.[10] The presence of this anomaly in normal heart is rarely reported with incidence of 0.03%.[11] The LAD after its origin may follow any of following pathways: prepulmonic (anterior to RVOT)—seen in our case, retroaortic, interarterial, transeptal/subpulmonic, and retrocardiac.[3] [12]
The explanation of clinical symptoms in these anomalies is largely unknown. Various causes are attributed—spasm of coronaries due to endothelial injury, myocardial squeezing, acute angles of take-off, slit-like orifices, and vessel hypoplasia. Other explanation is “steal phenomenon,” where an increased blood supply to a territory causes transient ischemia in other territories leading to symptoms. In our case, ECG suggested inferior wall ischemia, which may be caused by insufficiency of super-dominant RCA to adequately supply the LCX territory.[3] [10] [13] Literature suggests no definite correlation between CAA and atherosclerosis.[5] In our case, no atherosclerotic change was noted.
According to a literature review by Fugar et al, super dominant RCA with absent LCA and LCX is reported with other concomitant anomalies like atretic mid LAD originating from the sinus of Valsalva, dual LAD, and non-existing left subclavian artery.[8] However, there are very few reported cases of superdominant RCA with absent LCA and LCX along with LAD arising from the right coronary sinus. In one case, LAD was arising from the same ostium as superdominant RCA; while in other cases, LAD originated from different ostia (here, LAD had septal course rather than anterior free wall course).[14]
To our knowledge and best of literature search, our “combination of superdominant RCA with absent LCA and LCX with LAD originating from right coronary sinus with different ostia and having anterior free wall course” has been reported only once before by Enezate et al in 2017.[15]
Radiologists and Cardiologists should be acquainted with these anomalies as occlusion of a single coronary vessel may have deadly consequences. Knowledge of these anomalies would help in accurate revascularization in the presence of coronary artery disease. Precise anatomical and physiological evaluation of these anomalies is a must for opting for the best available therapeutic modality and better prognosis.
Conflict of Interest
None declared.
Financial Support and Sponsorship
None.
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References
- 1 Angelini P, Velasco JA, Flamm S. Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation 2002; 105 (20) 2449-2454
- 2 Kim SY, Seo JB, Do KH. et al. Coronary artery anomalies: classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics 2006; 26 (02) 317-333 , discussion 333–334
- 3 Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies overview: the normal and the abnormal. World J Radiol 2016; 8 (06) 537-555
- 4 Shriki JE, Shinbane JS, Rashid MA. et al. Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics 2012; 32 (02) 453-468
- 5 Erol C, Seker M. Coronary artery anomalies: the prevalence of origination, course, and termination anomalies of coronary arteries detected by 64-detector computed tomography coronary angiography. J Comput Assist Tomogr 2011; 35 (05) 618-624
- 6 Lipton MJ, Barry WH, Obrez I, Silverman JF, Wexler L. Isolated single coronary artery: diagnosis, angiographic classification, and clinical significance. Radiology 1979; 130 (01) 39-47
- 7 Desmet W, Vanhaecke J, Vrolix M. et al. Isolated single coronary artery: a review of 50,000 consecutive coronary angiographies. Eur Heart J 1992; 13 (12) 1637-1640
- 8 Fugar S, Issac L, Okoh AK, Chedrawy C, Hangouche NE, Yadav N. Congenital absence of left circumflex artery: a case report and review of the literature. Case Rep Cardiol 2017; 2017: 6579847
- 9 Hongsakul K, Suwannanon R. Congenital absence of left circumflex artery detected by computed tomography coronary angiography: a case report. Case Rep Vasc Med 2012; 2012: 204657
- 10 Tuncer C, Batyraliev T, Yilmaz R, Gokce M, Eryonucu B, Koroglu S. Origin and distribution anomalies of the left anterior descending artery in 70,850 adult patients: multicenter data collection. Catheter Cardiovasc Interv 2006; 68 (04) 574-585
- 11 Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990; 21 (01) 28-40
- 12 Angelini P. Coronary artery anomalies: an entity in search of an identity. Circulation 2007; 115 (10) 1296-1305
- 13 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 (01) e2
- 14 Vijayvergiya R, Kumar Jaswal R. Anomalous left anterior descending, absent circumflex and unusual dominant course of right coronary artery: a case report–R1. Int J Cardiol 2005; 102 (01) 147-148
- 15 Enezate T, Omran J, Aggarwal K. Super-dominant right coronary artery, absent left circumflex artery and left anterior descending artery arising from right coronary sinus. Postepy Kardiol Interwencyjnej 2017; 13 (01) 86-87
Address for correspondence
Publikationsverlauf
Artikel online veröffentlicht:
10. Januar 2022
© 2022. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 Angelini P, Velasco JA, Flamm S. Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation 2002; 105 (20) 2449-2454
- 2 Kim SY, Seo JB, Do KH. et al. Coronary artery anomalies: classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics 2006; 26 (02) 317-333 , discussion 333–334
- 3 Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies overview: the normal and the abnormal. World J Radiol 2016; 8 (06) 537-555
- 4 Shriki JE, Shinbane JS, Rashid MA. et al. Identifying, characterizing, and classifying congenital anomalies of the coronary arteries. Radiographics 2012; 32 (02) 453-468
- 5 Erol C, Seker M. Coronary artery anomalies: the prevalence of origination, course, and termination anomalies of coronary arteries detected by 64-detector computed tomography coronary angiography. J Comput Assist Tomogr 2011; 35 (05) 618-624
- 6 Lipton MJ, Barry WH, Obrez I, Silverman JF, Wexler L. Isolated single coronary artery: diagnosis, angiographic classification, and clinical significance. Radiology 1979; 130 (01) 39-47
- 7 Desmet W, Vanhaecke J, Vrolix M. et al. Isolated single coronary artery: a review of 50,000 consecutive coronary angiographies. Eur Heart J 1992; 13 (12) 1637-1640
- 8 Fugar S, Issac L, Okoh AK, Chedrawy C, Hangouche NE, Yadav N. Congenital absence of left circumflex artery: a case report and review of the literature. Case Rep Cardiol 2017; 2017: 6579847
- 9 Hongsakul K, Suwannanon R. Congenital absence of left circumflex artery detected by computed tomography coronary angiography: a case report. Case Rep Vasc Med 2012; 2012: 204657
- 10 Tuncer C, Batyraliev T, Yilmaz R, Gokce M, Eryonucu B, Koroglu S. Origin and distribution anomalies of the left anterior descending artery in 70,850 adult patients: multicenter data collection. Catheter Cardiovasc Interv 2006; 68 (04) 574-585
- 11 Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990; 21 (01) 28-40
- 12 Angelini P. Coronary artery anomalies: an entity in search of an identity. Circulation 2007; 115 (10) 1296-1305
- 13 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 (01) e2
- 14 Vijayvergiya R, Kumar Jaswal R. Anomalous left anterior descending, absent circumflex and unusual dominant course of right coronary artery: a case report–R1. Int J Cardiol 2005; 102 (01) 147-148
- 15 Enezate T, Omran J, Aggarwal K. Super-dominant right coronary artery, absent left circumflex artery and left anterior descending artery arising from right coronary sinus. Postepy Kardiol Interwencyjnej 2017; 13 (01) 86-87









