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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 10  |  Issue : 3  |  Page : 91-95

Anomalous origin of the right coronary artery from the left coronary sinus with medusa head left coronaries: Plethora of left coronary circulation with paucity of right one


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

Date of Submission23-Jul-2021
Date of Decision19-Aug-2021
Date of Acceptance22-Sep-2021
Date of Web Publication03-Nov-2021

Correspondence Address:
Dr. Debasish Das
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/rcm.rcm_42_21

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  Abstract 


We report a rare case of anomalous origin of right coronary artery (RCA) from the left coronary sinus with a peculiar medusa head pattern of left coronary system in an octogenarian presenting with inferior wall myocardial infarction. Although anomalous origin of RCA from the left coronary sinus is the most common anomaly to be reported, this rare association of extensive arborization of left coronary system appearing like a medusa head is not reported in literature so far. The anomalous RCA harbored critical mid-RCA lesion, but the crux of coronary intervention was that we engaged the anomalous RCA with extra back up guide catheter in place of conventionally used Judkins right guide catheter to engage the anomalous origin and accomplished the coronary intervention with buddy wire technique. Our case is unique and the first to describe the association of medusa head appearance of left coronary circulation in a case of anomalous origin of RCA from the left coronary sinus.

Keywords: Anomalous, right coronary artery, left coronary sinus, medusa head, coronaries


How to cite this article:
Das D, Acharya D, Mahanta DS, Singh S, Das T, Pramanik S. Anomalous origin of the right coronary artery from the left coronary sinus with medusa head left coronaries: Plethora of left coronary circulation with paucity of right one. Res Cardiovasc Med 2021;10:91-5

How to cite this URL:
Das D, Acharya D, Mahanta DS, Singh S, Das T, Pramanik S. Anomalous origin of the right coronary artery from the left coronary sinus with medusa head left coronaries: Plethora of left coronary circulation with paucity of right one. Res Cardiovasc Med [serial online] 2021 [cited 2021 Dec 3];10:91-5. Available from: https://www.rcvmonline.com/text.asp?2021/10/3/91/329847




  Introduction Top


Coronary anomalies per se are prevalent in more than 1% of the general population. The most common coronary anomaly reported is anomalous origin of right coronary artery (RCA) from the left coronary sinus followed by anomalous origin of left circumflex coronary artery from the right coronary sinus. Angiographic recognition of the coronary anomalies is of utmost importance before percutaneous coronary intervention, coronary artery bypass surgery, and valve replacement. Missing coronary anomalies may be catastrophic during cardiac surgery. For example, missing a large conus branch crossing the right ventricular outflow tract during intracardiac repair of Tetralogy of Fallot may lead to inadvertent transaction by the cardiac surgeon and subsequent left ventricular failure. As a dictum anomaly bears another anomaly. Therefore in syndromic patients, we do not get satisfied picking up a single anomaly, rather we exclude multiple associated anomalies. Our case is an interesting association of medusa head coronaries in a patient with anomalous origin of RCA from the left coronary sinus which has not been reported in literature so far. We also describe here an interesting anomalous heart with a plethora of left-sided circulation in the form of extreme arborization of left anterior descending and left circumflex coronary artery with typical medusa head appearance with a paucity of blood flow in the right-sided circulation in the form of obstructive atherosclerotic lesion in the mid-segment of RCA.


  Case Report Top


An octogenarian presented to the emergency department with a history of rest angina for the last 18 h with diaphoresis and shortness of breath. She was nondiabetic, nonhypertensive, normolipidemic without any family history of coronary artery disease. At presentation, her blood pressure was 134/80 mmHg in right arm supine position with a pulse rate of 68 beats/min. Cardiovascular system examination revealed the presence of left ventricular fourth heart sound (LVS4). Electrocardiogram revealed the presence of inferior wall ST-elevation myocardial infarction and echocardiography revealed hypokinesia in the inferior posterior wall with preserved left ventricular systolic function (EF – 55%) with mild mitral regurgitation. She was treated with a loading dose of antiplatelets, i.e., aspirin and ticagrelor with high-dose statin and was taken for elective coronary angiogram and intervention. Left coronary injection revealed an interesting highly arborized left coronary circulation with numerous branches of left anterior descending and left circumflex coronary artery looking like a typical medusa head appearance [Figure 1] which has not been described in the literature so far. We were not able to find the ostium of right coronary along the right edge of the right coronary sinus with tiger catheter. Even a bold injection of the right coronary sinus revealed that there was no origin of RCA from the right coronary sinus for which we thought we may be dealing with an anomalous RCA. As a rule of thumb, we scanned for the anomalous origin of RCA from the left sinus toward the right sinus with clockwise rotation of the tiger catheter which revealed anomalous origin of RCA from the left coronary sinus anterior and high to the origin of the left main coronary artery (LMCA) [Figure 2] and [Figure 3]. Right coronary injection also revealed a long segment critical lesion in mid-RCA. We did a computed tomography (CT) coronary angiogram to delineate the three-dimensional route of the anomalous coronary which revealed the same [Figure 4], [Figure 5], [Figure 6] without any interarterial or retroaortic or interstitial course. In view of ongoing angina with presence of ischemic mitral regurgitation, we decided for coronary intervention. RCA could not be engaged with conventional Judkins right (JR) catheter for which we engaged the anomalous RCA with extra back up (EBU) guide catheter with a nonusual loop [Figure 7]. Crux of the intervention was that the guide catheter was not stable due to this anomalous origin. We stabilized the guide catheter with buddy wire technique [Figure 7], predilated the lesion with a 2 mm × 10 mm semicompliant balloon, and revascularized the segment by deploying a drug-eluting stent of 2.75 mm × 30 mm size at 14 atm pressure. Post revascularization, RCA revealed that TIMI III flow [Figure 8] and vitals were stable. Anomalous coronaries sometimes have a peculiar anatomy or association, as illustrated in our case, and coronary intervention in those cases requires a slightly different technique or trick to achieve successful revascularization.
Figure 1: Medusa head appearance of the left coronary circulation

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Figure 2: Anomalous origin of right coronary artery from left coronary sinus anterior and high to the origin of left main coronary artery in AP view

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Figure 3: Anomalous origin of right coronary artery from the left coronary sinus anterior and high to the origin of left main coronary artery in right anterior oblique caudal view

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Figure 4: Anomalous origin of right coronary artery

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Figure 5: Right coronary artery having mid-segment critical lesion with origin of right coronary artery high and anterior to the origin of left main coronary artery

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Figure 6: Highly arborized left ventricle

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Figure 7: Engaging the anomalous right coronary artery with extra back up guide and stabilizing with buddy wire technique

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Figure 8: Post-Percutaneous transluminal coronary angioplasty Thrombolysis in Myocardial Infarction (PTCA TIMI) III flow in right coronary artery

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  Discussion Top


Anomalous origin of the RCA from the left sinus of Valsalva is a rare congenital anomaly representing <3% of the congenital coronary anomalies. Patients with this coronary anomaly can present with myocardial ischemia, arrhythmia, presyncope, syncope, or sudden cardiac death. Incidence of sudden cardiac death is rare after the age of 35 years in cases with anomalous origin of RCA from the left coronary sinus. Pathophysiology of the symptoms is due to systolic compression of the proximal RCA between aorta and pulmonary artery, slit-like orifice of the anomalous coronary, acute angle takeoff, and spasm of the proximal segment.[1] Although, in our case, the anomalous coronary artery had an acute angle takeoff, it was not the culprit behind the coronary ischemia. Interestingly, in our case, the anomalous coronary artery presented in the eighth decade of life and had obstructive coronary artery disease. Mid-RCA was harboring long segment critical lesion for which the patient presented with inferior wall myocardial infarction with the need of revascularization. The variety of anomalous RCA that courses between the aorta and the pulmonary artery carries a high risk of sudden cardiac death due to systolic compression of the artery between the aorta and the pulmonary artery.[2] Beta-blockers reduce the rate of sudden cardiac death in those varieties of anomalous origin of RCA from left coronary sinus as they reduce the systolic compression of the RCA between the aorta and pulmonary artery.[3] This anomalous RCA can present with arrhythmia also, Narayanan et al. reported a case of paroxysmal supraventricular tachycardia in a case of anomalous origin of RCA from the left coronary sinus presenting with recurrent acute coronary syndrome.[4] In our case, there was no interarterial course of the anomalous coronary artery between the aorta and the pulmonary artery which serves as a risk factor for sudden cardiac death in young age. Anomalous coronary artery does not increase the incidence of coronary artery disease per se,[5],[6] but in our case, it was associated with obstructive coronary artery disease; age may the contributing factor behind the development of coronary artery disease. Interestingly, this most common coronary anomaly is associated with congenital anomalies in 4.2%–24% of cases in the form of bicuspid aortic valve and mitral valve prolapse, as reported in literature.[7] However, extensive arborization of the left coronary circulation resembling a typical medusa head appearance has not been described in the literature so far as illustrated in our case. One-sided coronary anomaly associated with anomaly of other side is extremely rare to be reported in literature. Our case did not have associated structural anomaly in the form of mitral valve prolapse or bicuspid aortic valve and had ischemic mitral regurgitation with normal mitral valve apparatus and regional wall motion abnormality in inferior posterior wall with preserved left ventricular systolic dysfunction. Young patients <35 years with coronary ischemia which occurs in one in 24 patients with this anomaly are treated with coronary reimplantation.[8] Elderly patients are treated symptomatically with beta-blockers or conservatively. One explanation behind extensive arborization of the left coronary circulation may be when the inferior wall becomes hypokinetic to compensate the hypokinesia rest of the myocardium becomes hypercontractile. To meet the demand of the hypercontractile myocardium, the left circulation may have been extensively arborized in a peculiar medusa head appearance, but this is not commonly seen in routine practice. We hypothesize that this octogenarian may be having obstructive coronary artery lesion for long which may have led to extensive arborization of the left-sided coronary circulation but contrary to our finding was that he did not have no such robust ischemia driven collaterals from the left anterior descending coronary artery to the RCA. There is a high likelihood it may be a congenital extensive arborization of the left coronary circulation. In anomalous takeoff the RCA, multidetector CT (MDCT) delineates the slit-like orifice, abnormal takeoff angle, and course of the anomalous RCA.[9],[10] We did an MDCT in the aforesaid patient which clearly delineated the high and anterior origin of anomalous RCA as compared to the origin of LMCA with no interarterial course, i.e., between aorta and pulmonary artery. MDCT revealed left ventricle was extensively arborized left ventricle especially the lateral wall, as illustrated in [Figure 5]. Selective cannulation and stent insertion in this anomalous RCA often become difficult due to a small ectopic orifice.[11],[12] We tried to engage with the conventional JR catheter with which we were not able to engage the anomalous RCA. We could engage the anomalous RCA with an EBU guide catheter but with an abnormal angle [Figure 7]. We stabilized the guide and also negotiated the stent by putting two balanced middle weight wires in the coronary (buddy wire technique) [Figure 7] and successfully accomplished the percutaneous intervention. Anomalous origin of RCA from the left coronary sinus has not been reported in an octogenarian so far in the literature. Anomalous coronaries although are often detected incidentally during invasive coronary angiogram, many times they become evident in the young and middle age with exertional angina, shortness of breath, presyncope, syncope, or with an history of aborted sudden cardiac death. It may be due to extensive arborization with very good perfusion of the left ventricle, the octogenarian was not hypotensive in spite of having an inferior wall myocardial infarction with proximal RCA occlusion. We ruled out the “malignant” interatrial course or the course between the aorta and the pulmonary artery by doing a CT coronary angiogram. We managed the patient with dual antiplatelet (aspirin, ticagrelor), high dose statin, and beta-blocker and she was discharged in a stable condition the next day. Our case is unique and the first to illustrate the association of extensive arborization of the left coronary circulation assuming a medusa head appearance with anomalous RCA arising from the left coronary sinus in an octogenarian.


  Conclusion Top


Our case is the first and unique illustration of association of extensive arborization of the left coronary circulation in the form of a “Medusa” head appearance in a patient with anomalous origin of RCA from the left coronary sinus responsible for the preservation of left ventricular systolic function in spite of inferior wall myocardial infarction with proximal RCA occlusion in an octogenarian. Interestingly left-sided coronary circulation was plethoric, while the right side was suffering with paucity with proximal occlusion and anomalous course. Nature anomaly with high arborization of left ventricle has preserved the left ventricular systolic function in this octogenarian with anomalous RCA from the left coronary sinus.

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.

Ethical clearance

Due Ethical Clearance has been obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Virmani R, Chun PK, Goldstein RE, Rabinowitz M, McAllister HA. Acute take offs of the coronary arteries along the aortic wall and congenital coronary ostial valve-like ridges: Association with sudden death. J Am Coll Cardiol 1984;3:766-71.  Back to cited text no. 1
    
2.
Taylor AJ, Byers JP, Cheitlin MD, Virmani R. Anomalous right or left coronary artery from the contralateral coronary sinus: “High-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. Am Heart J 1997;133:428-35.  Back to cited text no. 2
    
3.
Kaku B, Shimizu M, Yoshio H, Ino H, Mizuno S, Kanaya H, et al. Clinical features of prognosis of Japanese patients with anomalous origin of the coronary artery. Jpn Circ J 1996;60:731-41.  Back to cited text no. 3
    
4.
Narayanan SR, Al Shamkhani W, Rajappan AK. Anomalous origin of RCA from left coronary sinus presenting as PSVT and recurrent acute coronary syndromes. Indian Heart J 2016;68:208-10.  Back to cited text no. 4
    
5.
Angelini P, Villason S, Chan AV Jr., Diez JG. Normal and anomalous coronary arteries in humans. In: Angelini P, editor. Coronary Artery Anomalies: A Comprehensive Approach. Philadelphia: Lippincott Williams and Wilkins; 1999. p. 27-150.  Back to cited text no. 5
    
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Zhang F, Ge JB, Qian JY, Fan B, Wang QB, Chen HZ. Frequency of the anomalous coronary origin in the Chinese population with coronary artery stenosis. Zhonghua Nei Ke Za Zhi 2005;44:347-9.  Back to cited text no. 6
    
7.
Topaz O, DeMarchena EJ, Perin E, Sommer LS, Mallon SM, Chahine RA. Anomalous coronary arteries: Angiographic findings in 80 patients. Int J Cardiol 1992;34:129-38.  Back to cited text no. 7
    
8.
Lee BY. Anomalous right coronary artery from the left coronary sinus with an interatrial course: Is it really dangerous. Korean Circ J 2009;39:175-9.  Back to cited text no. 8
    
9.
Kim JY, Yoon SG, Doh JH, Choe HM, Kwon SU, Namgung J, et al. Two cases of successful primary percutaneous coronary intervention in patients with an anomalous right coronary artery arising from the left coronary cusp.Korean Circulation Jouurnal 2008;38:179-83.  Back to cited text no. 9
    
10.
Moon JY, Jeong HC, Cho JY, Sim DS, Park HW, Hong JY, et al. Anomalous origin of a right coronary artery with extrinsic compression between the great vessels: the intravascular ultrasound images. Korean Circulation Journal 2008;38:390-92.  Back to cited text no. 10
    
11.
Hariharan R, Kacere RD, Angelini P. Can stent-angioplasty be a valid alternative to surgery when revascularization is indicated for anomalous origination of a coronary artery from the opposite sinus? Tex Heart Inst J 2002;29:308-13.  Back to cited text no. 11
    
12.
Ceyhan C, Tekten T, Onbasili AO. Primary percutaneous coronary intervention of anomalous origin of right coronary artery above the left sinus of Valsalva in a case with acute myocardial infarction. Coronary anomalies and myocardial infarction. Int J Cardiovasc Imaging 2004;20:293-7.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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