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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 138-141

Short dual right coronary artery without obstructive coronary artery disease causing obligatory coronary ischemia


Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission26-Apr-2021
Date of Decision15-Jun-2021
Date of Acceptance09-Jul-2021
Date of Web Publication25-Aug-2021

Correspondence Address:
Dr. Debasish Das
Associate Professor and HOD, Department of Cardiology, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha, Pin: 751019
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/heartindia.heartindia_76_21

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  Abstract 


We present an extremely rare case series of two short dual right coronary arteries (RCAs), causing coronary ischemia without the presence of obstructive coronary artery disease. We describe here the presence of short dual RCA in a 55-year-old nondiabetic, nonhypertensive, and nondyslipidemic male presenting with effort angina New York Heart Association Class II for the last 6 months with strongly positive stress test and another 40-year-old athlete (Central Reserve Police Force Army) without conventional risk factors with effort angina Class II for the last 3 months also with strongly positive stress test. Dual RCA, otherwise known as double RCA, duplicate RCA, or split RCA is extremely rare to encounter in routine clinical practice. The patient had a significant effort angina; in spite of nonatherosclerotic coronaries, small dual RCA terminating much earlier than the intended area of supply was the reason behind coronary ischemia with strongly positive provocative stress test. Short normal dual RCA can be a cause of coronary ischemia although mentioned in the literature; there has been no case report in the world literature till now about short dual nonatherosclerotic RCA causing coronary ischemia. Our case is unique and first to describe short dual RCA terminating much earlier than the intended area of supply can attribute to demand ischemia in the intended area of supply without the presence of obstructive coronary artery disease. We treated the patient with nicorandil to improve myocardial microvascular flow in ischemic region with symptomatic improvement of the patient.

Keywords: Artery, coronary, dual, right


How to cite this article:
Das D, Acharya D, Das T, Gupta JD, Pramanik S. Short dual right coronary artery without obstructive coronary artery disease causing obligatory coronary ischemia. Heart India 2021;9:138-41

How to cite this URL:
Das D, Acharya D, Das T, Gupta JD, Pramanik S. Short dual right coronary artery without obstructive coronary artery disease causing obligatory coronary ischemia. Heart India [serial online] 2021 [cited 2021 Dec 4];9:138-41. Available from: https://www.heartindia.net/text.asp?2021/9/2/138/324613




  Introduction Top


Coronary artery anomalies are usually incidentally diagnosed at the time of coronary angiography or autopsy with an incidence of 0.24%–1.3%. The majority of these are anomalies of origin or distribution, with the most common being separate ostia of left anterior descending artery and left circumflex (LCx) artery.[1] Although most of the primary congenital coronary anomalies are hemodynamically insignificant, it is important to know the anatomic variants in patients with coronary artery disease undergoing coronary angioplasty or surgical intervention.[2] Dual right coronary artery (RCA) is an extremely rare benign coronary anomaly, but an interesting phenomenon observed in our case was that small normal dual right coronary was the cause behind provocative coronary ischemia in both patients as it was terminating much earlier than the intended area of supply causing relative ischemia of the inferior myocardium. Our case is unique and first to describe the fact that short length of the dual coronary artery terminating much earlier than the intended area of supply even without the presence of obstructive CAD can attribute indirectly to the development of coronary ischemia.


  Case Reports Top


Case 1

Fifty-five-year-old male devoid of conventional risk factors presented with effort angina and shortness of breath New York Heart Association (NYHA) Class II for the past 6 months. Electrocardiogram (ECG) was within normal limit without ischemic ST-T changes and echocardiography revealed no regional wall motion abnormality with normal left ventricular systolic function. Treadmill test (TMT) positive with the presence of ST-T changes in recovery phase suggestive of coronary ischemia. In view of Canadian Cardiovascular Society (CCS) Class II angina with positive stress test, he was subjected to coronary angiogram which revealed normal left side coronaries with the presence of dual RCA. The patient had left dominant coronary circulation with normal left anterior descending artery and LCx artery [Figure 1]. Right coronary injection revealed a common origin of right coronary trunk and splitting into two separate right coronary arteries after a short distance which were running parallelly in the right atrioventricular groove [Figure 2]. Right posterior descending coronary artery (PDA) and posterior left ventricular (PLV) branch were originating from the LCx artery. In view of significant symptoms with positive stress test without any evidence of atherosclerosis, we noted that this dual coronary artery was very short in length and was terminating much earlier than the intended area of supply causing the inferior myocardium relatively ischemic. Although LCx was dominant, it was not able to supply the large area of myocardium made relatively ischemic by short length of dual RCA. Nicorandil improved the microvascular flow in the watershed ischemic region and improved the patient's symptoms, and he was found to be happy and doing well in the follow-up visit. Dual RCA is usually considered a benign entity, those dual right coronary arteries may present with coronary atherosclerosis, myocardial infarction, arrhythmia, and even sudden cardiac death. Although the presence of coronary anomaly is usually thought of in young persons presenting with an acute coronary syndrome without the presence of conventional risk factors, we encountered this rarest form of coronary anomaly in an elderly person without conventional risk factors without the evidence of coronary atherosclerosis where the shortness was the culprit behind relative myocardial inferior wall ischemia with positive stress test.
Figure 1: Left anterior oblique cranial view showing normal left coronary system

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Figure 2: Left anterior oblique cranial view showing small dual right coronary artery with marked rounded area of relatively ischemic inferior myocardium

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Case 2

A forty-year-old athlete, working as an army officer in Central Reserve Police Force presented with effort angina CCS Class II with shortness of breath NYHA Class II for the past 3 months, ECG and echocardiography were within normal limits without any regional wall motion abnormality and was subjected to TMT which was strongly positive for provocative ischemia with more than 2 mm ST depression in more than 5 leads [Figure 3] and was subjected to invasive coronary angiogram which revealed no evidence of atherosclerosis in the coronaries [Figure 4] with the presence of dual RCA which was relatively short in length, was terminating much earlier than the intended area of supply causing the inferior wall relatively ischemic [Figure 5], and was responsible for provocative stress test in the patient with significant symptoms. Although LCx was dominant in this case, it was not covering the large area of flow deficit inferior myocardium due to the presence of short length dual RCA. Although shortness of dual RCA is a cause of coronary ischemia mentioned in the literature, our case is unique and first to describe the short length of dual RCA to be the reason behind coronary ischemia in the presence of nonatherosclerotic coronaries, shortness also contributes to myocardial ischemia in the subset when nature had provided two arteries to supply that region; a form of demand ischemia of the inferior myocardium caused by short dual RCA.
Figure 3: Strongly positive treadmill test in the Athlete with short dual right coronary artery

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Figure 4: Left anterior oblique cranial view showing normal left coronary system

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Figure 5: Left anterior oblique cranial view showing small dual right coronary artery with marked rounded area of relatively ischemic inferior myocardium

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  Discussion Top


Coronary anomalies are incidentally detected during routine coronary angiography. Congenital coronary anomalies are seen in approximately 1% of adult patients.[3] Double RCA is a very rare type of coronary anomaly. There was no description of this anomaly in a series of 126,595 patients who underwent coronary angiography.[3] The first report about dual RCA anomaly was described by Barthe et al.[4] They described dual RCA originating from a single ostium and running parallelly in right atrioventricular groove as described in our case. They described after the origin of conus artery and a ventricular branch, the most anterior RCA descended toward the acute margin of the heart and terminated in a small posterior descending artery. The second RCA also terminated in a small posterior descending and posterolateral branches. In our cases, both right coronary arteries were almost identical in size and both were not giving rise to PDA as the patient had left dominant circulation with LCx artery crossing the crux of the heart and was giving rise to both left PDA and PLV branch. Interestingly, both cases of dual RCA were very small and were terminating much earlier than the intended area of supply causing the myocardium relatively ischemic and were the reason for provocative ischemia without the evidence of coronary atherosclerosis. Although double RCA is an extremely rare anomaly, it is not necessarily benign, as it has been associated with atherosclerosis, life-threatening arrhythmia, and myocardial infarction.[5],[6],[7] However, our patients' coronaries were free of atherosclerosis. In the absence of atherosclerotic stenosis, ischemia in dual RCA can be a result of anatomical malformations including an acute takeoff angle of the anomalous vessel, myocardial squeezing, vasospasm, and a small artery.[1] Our case is the unique and first demonstration of the landmark sentence in Tuncers[1] article that small dual RCA can be a cause of obligatory ischemia of the myocardium. In our case, myocardial ischemia was due to the presence of two small right coronary arteries which were terminating much earlier before their destination making the inferior territory relatively ischemic with strongly positive provocative stress test in both cases. Dual RCAs most commonly present as isolated coronary anomalies. Two previous studies have reported double RCA in combination with other anomalies.[8],[9] Interestingly, most of the cases of dual RCA have been described in Turkish population, suggesting an association with a particular genetic background. The double RCA anomaly is seen mostly in males, as other congenital coronary anomalies. In conclusion, although double RCA is a rare anomaly and eventually benign, small double RCA can result in relative myocardial ischemia without the presence of flow-limiting lesion in coronaries. The majority of the cases of double RCA originates from single ostia. The course of one RCA corresponds to anterior RCA, whereas the course of the other RCA corresponds to posterior RCA. Capuñay et al.[10] observed a similar course of double RCA. In contrast, Karabay et al.[11] and Harikrishnan et al.[12] documented superior and inferior RCAs. Split RCA is the same anomaly, improperly named as double RCA. Where split portions of the RCA branches with two separate courses. There is no distinct definition between double RCA and the separate conus branch or the right ventricular branch from the right sinus of valsalva. Young-hyman et al.[8] reported a new dual RCA anomaly, in which the RCA originated above the coronary sinus, giving off circumflex artery. Akçakoyun et al.[13] described double RCA coexisting with separately originating left anterior descending and circumflex arteries. Rohit et al.[7] described a case of double RCA with acute inferior wall infarction. We report an extreme rare case series of small dual RCA where shortness was the culprit behind coronary ischemia. Meticulous grossing and careful observation could unearth hidden importance of coronary anomalies behind coronary ischemia.


  Conclusion Top


Our case series is unique and first to describe small dual RCA where shortness in length was the culprit behind coronary ischemia without the presence of flow-limiting lesion in coronaries as both small dual RCA were terminating much earlier than the intended area of supply causing inferior myocardium relatively ischemic which was the principal reason behind strongly positive stress test in both cases. Meticulous observation unmasks the etiology of coronary ischemia in the setting of nonflow limiting lesions in coronaries. Obstructive coronary artery disease is not the only cause behind the coronary ischemia in the presence of coronary anomalies; length also matters for myocardium.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Authors' contributions

Debasish Das: Final Manuscript Editing Debasis Acharya: Manuscript Preparation Tutan Das, Jaideep Das Gupta: Literature Search Subhas Pramanik: Image Editing.

Ethical approval

Due ethical approval has been obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.







 
  References Top

1.
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;68:574-85.  Back to cited text no. 1
    
2.
Erbagcı H, Davutoglu V, Turkmen S, Kizilkan N, Gumusburun E. Double right coronary artery: Review of literature. Int J Cardiovasc Imaging 2006;22:9-11.  Back to cited text no. 2
    
3.
Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21:28-40.  Back to cited text no. 3
    
4.
Barthe JE, Benito M, Sala J, Houbani AJ, Quintana E, Esplugas E, et al. Double right coronary artery. Am J Cardiol 1994;73:622.  Back to cited text no. 4
    
5.
Timurkaynak T, Ciftci H, Cengel A. Double right coronary artery with atherosclerosis: A rare coronary artery anomaly. J Invasive Cardiol 2002;14:337-9.  Back to cited text no. 5
    
6.
Ozeren A, Aydin M, Bilge M, Dursun A, Onuk T. Atherosclerotic double right coronary artery and ectasia of left coronary arteries in a patient with presented acute coronary syndrome and ventricular tachycardia. Int J Cardiol 2005;102:341-3.  Back to cited text no. 6
    
7.
Rohit M, Bagga S, Talwar KK. Double right coronary artery with acute inferior wall myocardial infarction. J Invasive Cardiol 2008;20:E37-40.  Back to cited text no. 7
    
8.
Young-Hyman PJ, Tommaso CL, Singleton RT. A new double coronary artery anomaly: The right coronary artery originating above the coronary sinus giving off the circumflex artery. J Am Coll Cardiol 1984;4:1329-31.  Back to cited text no. 8
    
9.
Garg N, Goel PK, Sinha N. Double right coronary artery with anomalous left main and septal arteries originating from the right coronary sinus. Indian Heart J 2002;54:428-31.  Back to cited text no. 9
    
10.
Capuñay C, Carrascosa P, Deviggiano A. MDCT detection of double right coronary artery arising from two separated ostia in the right sinus of Valsalva. Int J Cardiol 2010;139:e33-5.  Back to cited text no. 10
    
11.
Karabay KO, Catakoglu AB, Demiroglu IC, Aytekin V. Double right coronary artery originating from separate orifices – A case report and review of the literature. Int J Cardiol 2007;118:e6-7.  Back to cited text no. 11
    
12.
Harikrishnan S, Bhat A, Tharakan JM. Double right coronary artery. Int J Cardiol 2001;77:315-6.  Back to cited text no. 12
    
13.
Akçakoyun M, Acar G, Avcy A, Kargyn R, Esen AM. Double right coronary artery co-existing with separately originating left anterior descending and circumflex arteries. Eur J Gen Med 2010;7:345-7.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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