|Year : 2014 | Volume
| Issue : 1 | Page : 43-45
"Four vessel disease": A case of diseased double split right coronary artery
Anshuman Darbari1, Devender Singh2
1 Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Cardiothoracic Surgery, Kovai Medical Centre and Hospital, Coimbatore, Tamil Nadu, India
|Date of Web Publication||7-Apr-2014|
Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, Rishikesh-249 201, Uttarakhand
Source of Support: None, Conflict of Interest: None
Coronary artery anomalies (CAAs) are assorted group of congenital disorder whose nomenclature, manifestations, and pathophysiological mechanisms are highly inconsistent. The prevalence of congenital anomalies of the coronary arteries is reported to be approximately 0.6%-1.3% of the general population. Of them, double right coronary artery (RCA) is one of the rarest coronary anomalies . In this case, double split right coronary arteries are diseased with these other two left-sided arteries are also having atherosclerosis disease with significant stenosis, labeling this patient as having "Four vessel disease." If this anomaly had gone undiagnosed or missed, the patient's symptoms would have been recurred despite revascularization.
Keywords: Congenital, Coronary artery, coronary bypass surgery, ischemic heart disease
|How to cite this article:|
Darbari A, Singh D. "Four vessel disease": A case of diseased double split right coronary artery. Nig J Cardiol 2014;11:43-5
| Introduction|| |
Variations in coronary arteries anatomy are very often recognized in association with structural forms of congenital heart disease and now as a separate entity. The prevalence of congenital anomalies of the coronary arteries are reported to be approximately 0.6-1.3% of the general population. Of them, the double right coronary artery is one of the rarest coronary anomalies.
| Case Report|| |
A 60-year-old male nonhypertensive, nondiabetic, previously diagnosed coronary artery disease (CAD) patient came to our hospital with complaints of severe chest pain suggestive of unstable angina. Physical examination was normal. The electrocardiogram at admission revealed T-wave inversion in the precordial and inferolateral leads. Troponin-T test was positive. Two-dimensional echocardiography showed akinesia of distal intraventricular septum, apex, and inferior wall with hypokonesia of other areas. Left ventricular ejection fraction was 30%. After stabilization, coronary angiogram (CAG) was performed that revealed multiple severe stenosis in both left coronary artery including the left anterior descending (LAD) and left circumflex artery (LCx) and in their branches (Proximal LAD discrete 60% stenosis, Obutse marginal branch - 99% and LCx (beyond OM1)-100%) [Figure 1]. The right ostium was selectively cannulated. The contrast injection during CAG revealed two equal caliber right coronary arteries with 70% stenosis in mid portion of one RCA [Figure 2] and 90% stenosis at origin of second RCA [Figure 3]. One RCA followed along the atrioventricular groove and terminated by giving off the posterior descending artery (PDA). Another RCA ran parallel and terminated by giving off a smaller PDA. We observed atherosclerotic narrowing in both these right coronary arteries. He was stabilized by standard medical management of nitrates, antiplateletes, diuretics, statins, and prepared for coronary artery bypass graft surgery. Under general anesthesia, off pump coronary artery bypasss grafting of left internal mammary artery to LAD and three reverse saphenous venous grafts to other diseased vessels was done with positive uneventful outcome.
|Figure 1: Selective left coronary angiography: Anteroposterior cranial view showing diseased left anterior descending and left circumflex arteries|
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|Figure 2: Selective right coronary angiography: Left anterior oblique view showing diseased double split right coronary artery (RCA). The proximal mid RCA having stenosis and another RCA having severe stenosis in its proximal portion|
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|Figure 3: Selective right coronary angiography: Right anterior oblique view showing diseased double split right coronary artery (RCA). The anterior RCA having severe stenosis in its proximal portion and the posterior RCA having stenosis in its mid and distal portions|
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| Discussion|| |
Coronary artery anomalies (CAAs) are the second most common cause of sudden death in older children and young adults in the absence of additional heart abnormalities after hypertrophic cardiomyopathy. No racial and sexual predisposition for CAAs is known. Because of considerable variation in coronary vasculature, terminology is confusing and what is considered atypical, abnormal, aberrant, anomalous, accessory, ectopic, incidental, variant, or significant is often remains unclear. In general, the terms anomalous or abnormal are used to define any variant form observed in less than 1% of the general population. ,
Double right coronary artery is a very rare coronary anomaly. The first report in the literature was by Barthe et al.,  As discussed by Chen et al.,  several concepts have been proposed in order to define what is and is not a double RCA. Various nomenclature or keywords "double right coronary artery," "duplicated right coronary artery," "dual right coronary artery," and "split right coronary artery" are used. However, some investigators contended that a split RCA is anatomically the same anomaly as the improperly named "double right coronary artery." Nonetheless, there is no scientific consensus over the definition and correct diagnosis of a double RCA.  So, there is a need for a consensus regarding the nomenclature as well diagnostic criteria of this coronary anomaly. Besides, it is also confusing to distinguish double RCA with single orifice or high take that off of a large right ventricular artery from RCA. The right anterior oblique view of CAG provides better demonstration of artery courses and may be helpful in differentiating double RCA from a large right ventricular branch.  However, proposed by Sato et al.,  and accepted view is that double RCAs are defined when they both supply the blood to the inferior left myocardium, thus both of the RCAs should course downwardly to reach the interventricular sulcus whether or not they cross the crux, as in this case.
In conclusion, there is still debate about correct definition and benign nature of double RCA, but it might be early atherosclerotic due to altered blood flow kinetics and can cause unusual picture of coronary ischemia. CAG is the most common and widely used diagnostic modality and multidetector computed tomography might also detect this. 
Every cardiologist and cardiothoracic surgeons should be aware with this rare anomaly to prevent subsequent error in management. In this type of case, one of the RCA can be missed due to total atherosclerotic occlusion at the proximal part of the RCA. Failure to recognize such an anomaly with atherosclerotic involvement could be disappointing and can lead to inadequate revascularization.
| References|| |
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|2.||Angelini P. Coronary artery anomalies: An entity in search of an identity. Circulation 2007;115:1296-305. |
|3.||Barthe JE, Benito M, Sala J, Houbani AJ, Quintana E, Esplugas E, et al. Double right coronary artery. Am J Cardiol 1994;73:622. |
|4.||Chen YF, Chien TM, Chen CW, Lin CC, Lee CS. Double right coronary artery or split right coronary artery? Int J Cardiol 2012;154:243-5. |
|5.||Sari I, Davutoglu V, Aksoy M. The correct number of double right coronary artery reported in the literature. Int J Cardiol 2008;130:283-4. |
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|7.||Sato Y, Kunimasa T, Matsumoto N, Saito S. "Detection of double right coronary artery by multi-detector row computed tomography: Is angiography still gold standard?" Int J Cardiol 2008;126:134-5. |
[Figure 1], [Figure 2], [Figure 3]