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Year : 2023  |  Volume : 24  |  Issue : 2  |  Page : 114-118  

Anomalous double right coronary artery presenting as inferior wall ST-elevation myocardial infarction: A rare coronary anomaly

Department of Cardiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Date of Submission14-Oct-2022
Date of Acceptance16-Feb-2023
Date of Web Publication24-Mar-2023

Correspondence Address:
Dr. Vivek Mohanty
CCU-6th Floor, IPD Building Cardiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/heartviews.heartviews_94_22

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Congenital anomalous origin of coronary arteries is rare and occurs in 0.2%–2% of patients undergoing coronary angiography (CAG). Most of the cases are benign but may present with life-threatening symptoms such as myocardial ischemia or sudden cardiac death. The prognosis depends on the site of origin of the anomalous artery, intramyocardial course, and relation to other great vessel and cardiac structures. Increased awareness and easy availability of noninvasive methods like computed tomography CAG have led to more reporting of such cases. Here, we report the case a 52-year-old male with a double right coronary artery having anomalous origin from a noncoronary aortic cusp detected during CAG which has not been reported in the literature before.

Keywords: Anomalous right coronary artery, double right coronary artery, noncoronary cusp

How to cite this article:
Mohanty V, Sharma S, Deora S. Anomalous double right coronary artery presenting as inferior wall ST-elevation myocardial infarction: A rare coronary anomaly. Heart Views 2023;24:114-8

How to cite this URL:
Mohanty V, Sharma S, Deora S. Anomalous double right coronary artery presenting as inferior wall ST-elevation myocardial infarction: A rare coronary anomaly. Heart Views [serial online] 2023 [cited 2023 Jun 9];24:114-8. Available from: https://www.heartviews.org/text.asp?2023/24/2/114/372463

   Introduction Top

Congenital anomalies of the coronary artery are rare, and their prevalence varies from 0.2% to 2% in the general population.[1] Most of the cases of anomalous origin of coronary vessels (AOCVss) are benign but may present with chest pain, dyspnea, acute coronary syndrome (ACS), syncope, or even sudden cardiac death.[2],[3]

The most common coronary anomaly is the separate origin of the left anterior descending artery and left circumflex artery (LCX) which has an incidence of about 0.41% followed by the LCX arising from the right coronary cusp with an incidence of 0.37%.[4] Anomalous origin of the right coronary artery (RCA) from noncoronary cusp is an extremely rare anomaly with a reported prevalence of 0.003% based on catheter-based angiography series.[5]

Split RCA arising from noncoronary cusp has not been reported in the literature before.

Here, we highlight the case of a 52-year-old patient who presented with inferior wall ST-elevated myocardial infarction and underwent thrombolysis with reteplase. His angiography was suggestive of split RCA arising from a noncoronary cusp without any significant obstruction making it an extremely rare presentation.

   Case Presentation Top

A 52-year-old male presented to emergency with chief complaints of chest pain for 4 h. Twelve-lead electrocardiogram showed ST-elevation in lead II, III, and aVF [Figure 1] suggestive of inferior wall ST-elevation myocardial infarction (STEMI). He was thrombolysed with reteplase. Serum biochemistry was normal except for significantly raised cardiac enzymes and troponin.
Figure 1: Twelve-lead ECG showing ST elevation in lead II, III, and aVF suggestive of inferior wall STEMI. ECG: Electrocardiogram, STEMI: ST-elevation myocardial infarction

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The patient subsequently underwent two-dimensional (2D) transthoracic echocardiography which showed normal left ventricular dimensions and ejection fraction with RCA arising from a no aortic cusp [Figure 2].
Figure 2: 2D-transthoracic echocardiography showing the origin of the right coronary artery from the noncoronary cusp. 2D: Two-dimensional, white arrow shows Right Coronary Artery originating from Non-Coronary cusp in 2D-Transthoracic Echocardiography short axis view

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He was taken for catheter angiography through the right radial route which showed the main RCA originating from noncoronary cusp bifurcating into anterior and posterior RCA [Figure 3]a and [Supplementary Video 1]. Anterior RCA supplied the free wall of the right ventricle and distal half of the interventricular septum, whereas posterior RCA supplied the right atrioventricular groove and basal interventricular septum after reaching the crux [Figure 3]b and [Figure 3]c and [Supplementary Video 2]. The left angiogram showed nonobstructive left anterior descending and LCX present in the AV groove without any significant obstruction [Figure 3]d. The diagnosis was confirmed on CT coronary angiography (CAG) which showed RCA originating from non coronary cusp (NCC) with a normal proximal cross-sectional lumen and no acute take-off angle or intramural course [Figure 4].
Figure 3: (a) Catheter right coronary angiogram in left anterior oblique 30° view showing RCA originating from noncoronary cusp and bifurcating into anterior and posterior branches without significant obstruction. (b) Anterior-posterior cranial view shows both branches supply the inferior part of left ventricle after reaching the interventricular sulcus. (c) RAO view showing double RCA from noncoronary cusp. (d) RAO caudal view showing nonobstructive left anterior descending and LCX arteries. RCA: Right coronary artery, LCX: Left circumflex artery

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Figure 4: Reconstructed three-dimensional volume rendering technique image of coronary computed tomography angiography demonstrating double RCA arising from the noncoronary aortic cusp. A. RCA: Anterior right coronary artery, AMB: Acute marginal branch, CA: Conus artery, P. RCA: Posterior right coronary artery, RAO: Right anterior oblique

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[Additional file 1]

[Additional file 2]

The patient was managed medically with antiplatelets, statins, and angiotensin-converting enzyme inhibitors and discharged in a hemodynamically stable condition.

   Discussion Top

AOCVs are not routinely seen in clinical practices. Most of them have a benign course and are incidental findings during routine angiography. However, it is important to recognize them as they may present with angina, ACS, or even sudden cardiac death (SCD). Catheter angiography is an effective tool for the diagnosis of AOCV but it is invasive and has procedural morbidity (1.5%) and mortality (0.15%).[6] However, it has a disadvantage that it cannot show the relationship of aberrant vessels with underlying structures due to its 2D nature.[7] Noninvasive methods like electrocardiographic-gated multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) help in three dimensional of these anomalous and shows detailed anatomical relationship with underlying cardiac structures and are now becoming the investigation of choice for the same.[8]

It is important to recognize these anomalies as cannulation may prove difficult for these patients and may require prompt adjustment of the access vector during CAG.[9]

Only a few cases of RCA originating from the noncoronary cusp have been reported in the literature before.[10] The double RCA itself is an extremely rare anomaly.[11],[12] Cases have been reported in the last few years where double RCA complicated by atherosclerosis presented as ACS.[13],[14],[15]

An attempt to classify double RCA [Table 1] has been made by researchers from Turkey.[16] To the best of our knowledge, this is the first case reported in literature where the double RCA has anomalous origin from noncoronary cusp presented with inferior wall STEMI having undergone successful thrombolysis making it an extremely rare presentation. There are still no official guidelines published on the management of these complex cases and hence treatment needs to be individualized on a case-to-case basis.
Table 1: Classification of double right coronary artery

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   Conclusion Top

Anomalous RCA from noncoronary cusps and duplicate RCA are rare coronaries anomalies. Presence of both these anomalies in the same patient has not been reported in the literature before. Although most of the cases are benign and the anatomical course of the vessel is typically normal still recognition of these anomalies is important as a prompt intervention may be required in some cases to prevent myocardial ischemia, ventricular arrhythmia, or even sudden cardiac death.

Due to limitations of catheter angiography, noninvasive methods are MDCT and MRI are now becoming the investigation of choice in these patients with advances in these noninvasive methods sensitivity of diagnosing these lesions has increased considerably and hence may pave the way for future guidelines for the management of these cases.

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

Fujimoto S, Kondo T, Orihara T, Sugiyama J, Kondo M, Kodama T, et al. Prevalence of anomalous origin of coronary artery detected by multi-detector computed tomography at one center. J Cardiol 2011;57:69-76.  Back to cited text no. 1
Taylor AJ, Rogan KM, Virmani R. Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 1992;20:640-7.  Back to cited text no. 2
Angelini P. Novel imaging of coronary artery anomalies to assess their prevalence, the causes of clinical symptoms, and the risk of sudden cardiac death. Circ Cardiovasc Imaging 2014;7:747-54.  Back to cited text no. 3
Young PM, Gerber TC, Williamson EE, Julsrud PR, Herfkens RJ. Cardiac imaging: Part 2, normal, variant, and anomalous configurations of the coronary vasculature. AJR Am J Roentgenol 2011;197:816-26.  Back to cited text no. 4
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. 5
Pasaoglu L, Toprak U, Nalbant E, Yagiz G. A rare coronary artery anomaly: Origin of all three coronary arteries from the right sinus of valsalva. J Clin Imaging Sci 2015;5:25.  Back to cited text no. 6
[PUBMED]  [Full text]  
Ben-Dor I, Weissman G, Rogers T, Slack M, Pichard A, Ben-Dor N, et al. Catheter selection and angiographic views for anomalous coronary arteries: A practical guide. JACC Cardiovasc Interv 2021;14:995-1008.  Back to cited text no. 7
Montaudon M, Latrabe V, Iriart X, Caix P, Laurent F. Congenital coronary arteries anomalies: Review of the literature and multidetector computed tomography (MDCT)-appearance. Surg Radiol Anat 2007;29:343-55.  Back to cited text no. 8
Greenberg MA, Fish BG, Spindola-Franco H. Congenital anomalies of the coronary arteries. Classification and significance. Radiol Clin North Am 1989;27:1127-46.  Back to cited text no. 9
Jhaveri S, Tower-Rader A, Zahka K. Anomalous origin of right coronary artery from the non-coronary cusp demonstrated by echocardiography and CT angiography. Pediatr Cardiol 2019;40:454-5.  Back to cited text no. 10
Altun A, Akdemir O, Erdogan O, Ozbay G. An interesting diagnostic dilemma: Double right coronary artery or high take off of a large right ventricular branch. Int J Cardiol 2002;82:99-102.  Back to cited text no. 11
Sari I, Kizilkan N, Sucu M, Davutoglu V, Ozer O, Soydinc S, et al. Double right coronary artery: Report of two cases and review of the literature. Int J Cardiol 2008;130:e74-7.  Back to cited text no. 12
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. 13
Sari I, Uslu N, Gorgulu S, Nurkalem Z, Eren M. Inferior myocardial infarction and extensive atherosclerosis in a patient with double right coronary artery. Int J Cardiol 2006;111:321-3.  Back to cited text no. 14
Tatli E, Buyuklu M, Altun A, Yilmaztepe M. A patient with double right coronary artery and acute inferior myocardial infarction due to the critical lesion in one of them. Int J Cardiol 2007;119:e30-1.  Back to cited text no. 15
Vural M. Suggestions for definition and classification of double right coronary artery, which is frequently seen in Turkey despite its rarity in the literature. Anadolu Kardiyol Derg 2008;8:303-4.  Back to cited text no. 16


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

  [Table 1]


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