Anomalous left main coronary artery arising from the right sinus of valsalva in a young male: A rare anomaly of the origin of the coronary arteries

Pankaj Jariwala

doi:10.4103/JICC.JICC_17_20

CASE REPORT

Year : 2020 | Volume : 10 | Issue : 3 | Page : 139-141

Anomalous left main coronary artery arising from the right sinus of valsalva in a young male: A rare anomaly of the origin of the coronary arteries

Pankaj Jariwala
Department of Cardiologist, Yashoda Hospitals, Hyderabad, Telangana, India

Date of Submission	01-Apr-2020
Date of Decision	08-May-2020
Date of Acceptance	10-Jun-2020
Date of Web Publication	23-Dec-2020

Correspondence Address:
Dr. Pankaj Jariwala
Department of Cardiologist, Yashoda Hospitals, Somajiguda, Raj Bhavan Road, Hyderabad - 500 082, Telangana
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JICC.JICC_17_20

Abstract

The left coronary artery culminating from the right sinus of Valsalva is a rare congenital anomaly of the coronary artery. A high level of clinical suspicion is required in younger patients presenting with a chest pain and positive stress test. A 34-year-old male with a previous family history of premature coronary artery disease, a diagnosis of the anomalous aortic origin of the coronary arteries, needs thorough evaluation. A future fatal outcome can be prevented if diagnosed with the aid of coronary angiography and should be treated promptly. Noninvasive tests, such as echocardiography and computed tomography, can help in the diagnosis and is becoming incredibly popular.

Keywords: Computed tomography, coronary angiography, coronary anomalies, sudden cardiac death

How to cite this article:
Jariwala P. Anomalous left main coronary artery arising from the right sinus of valsalva in a young male: A rare anomaly of the origin of the coronary arteries. J Indian coll cardiol 2020;10:139-41

How to cite this URL:
Jariwala P. Anomalous left main coronary artery arising from the right sinus of valsalva in a young male: A rare anomaly of the origin of the coronary arteries. J Indian coll cardiol [serial online] 2020 [cited 2022 Dec 7];10:139-41. Available from: https://www.joicc.org/text.asp?2020/10/3/139/304373

Introduction

The left main coronary artery (LMCA) or left anterior descending (LAD) coronary artery stemming from the right sinus of Valsalva or the right coronary artery (RCA) is defined as the “anomalous aortic origin of the coronary artery” (AAOCA). The malignant course is mostly between the aorta and the pulmonary artery on its way to the left ventricle, which might compress the vessel and lead to fatal outcomes such as myocardial ischemia, ventricular arrhythmias, and sudden cardiac death.^[1] This is a unusual case of Left coronary artery that emerges from the right sinus of Valsalva without substantial stenosis in a 34-year-old man who had chest pain and myocardial ischemia provocation during stress testing.

Case Report

A 34-year-old male was admitted with complains of rest chest pain for 2 days. He had a family history of premature coronary artery disease. His electrocardiography and echocardiography were unremarkable. Serial troponin levels were normal. The exercise stress test was positive at stage 2 with significant ST-segment depressions. During coronary angiography, the left coronary artery could not be located in the left coronary sinus. Selective angiography of the RCA delineated the origin of the left coronary artery from the right coronary sinus as a common trunk. RCA had a normal course, while LMCA traversed upward and anteriorly to resume its normal course. It further trifurcated into three epicardial branches such as LAD, ramus intermidius, and left circumflex arteries [Figure 1]a, [Figure 1]b, [Figure 1]c.

Figure 1: (a-c) Conventional coronary angiography in the left anterior oblique cranial (Panel A), right anterior oblique caudal (Panel B), and right anterior oblique cranial (Panel C) views demonstrating the normal origin and course of the right coronary artery (solid black arrows) and anomalous origin of the left main coronary artery from the right coronary sinus. Left main coronary artery trifurcated into the left anterior descending artery (white dashed arrows), ramus intermidius (white arrow heads), and left circumflex artery (white solid arrows)

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There were no significant stenoses of the coronaries. The patient was discharged with the advice of computed tomography angiography. Thereafter, the patient lost to the follow-up.

Discussion

The incidence of AAOCAs is 0.2%–0.64%, which is associated with the risk of sudden cardiac death.^[2] AAOCA of the left coronary artery is rare than that of RCA and its prevalence is around 0.047%–0.15%,^[3],[4] with a ratio of 6:1 in the general population. The LMCA may have a septal course starting from the right ventricular infundibulum; it may have an anterior course, a retro-aortic course, or an intra-arterial course.^[5],[6] Some of the inherent anatomical features that may increase the risk of ischemia-related events include ostial stenosis of coronary arteries, intramural and interarterial course of the proximal segment of the coronary artery inside the aortic wall, and possible compression between the major arteries.^[7]

The pathophysiology behind the occurrence of the chest pain is myocardial ischemia secondary to the compression of the anomalous coronary artery between the aorta and the pulmonary artery on its way to the left ventricle. Severe ischemia can lead to syncope or sudden cardiac death in young age those who participate in athletic events.^[8]

There is a lack of guidelines for diagnosing and assessing these kinds of features to risk stratify patients on the basis of evidence available and to plan a definitive surgical strategy. Regrettably, 50%–90% of patients who succumb unexpectedly secondary to malignant types of coronary anomaly have little advance warning symptoms and <10% are considered to still have a cardiological assessment of symptoms relevant to anomaly. The symptoms such as breathlessness, recurrent chest pains, syncope, and aborted sudden death definitely need a thorough evaluation. The electrocardiogram is often normal as in our case, and the utility of regular submaximal stress test in the assessment of coronary function is largely invalidated by the relatively high occurrence of false-positive and false-negative tests.^[4] However, transthoracic echocardiography (TTE) is the most effective initial screening method for the diagnosis of AAOCA in younger patients and cost-effective in young athletes. Lorber et al. developed echocardiography protocol for the diagnosis of AAOCA and to delineate the intramural, interarterial course, intra-conal and high ostial take off of the anomalous coronary artery. Trans-esophageal echocardiography (TEE) should be used in adults where TTE does not clearly delineate the coronary arteries.^[7] The presence of AAOCA, number of ostia in aortic sinuses, length of intramural segments, retro-aortic or prepulmonic or interarterial or intraconal course, acute angle of coronary takeoff, and color Doppler evaluation of intramural segment in diastole are features that need consideration to detect unfavorable features of AAOCA using TTE and TEE. TTE has been shown to be diagnostic for the intramural coronary course in 92.5% of all patients in one of the surgical series.^[9]

The definitive diagnosis is only possible with the help of angiography. Multidetector computed tomography is preferred over conventional angiography as a modality of investigation, particularly in patients with a high index of suspicion but equivocal results with noninvasive evaluation.^[10],[11] In our case, we performed conventional angiography as the patient had a family history of CAD and a diagnosis of AAOCA was not suspected initially.

Conclusion

AAOCA should be suspected in young age if there are no ECG changes and positive stress test. If the anomaly is not associated with obstructive coronary lesions, the ischemic symptoms are generally nonetheless attributed to the anomaly. It may also be the cause of the symptoms, and treatment planning in these cases is complex. In such symptomatic patients, timely surgical intervention is imperative.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Peñalver JM, Mosca RS, Weitz D, Phoon CK. Anomalous aortic origin of coronary arteries from the opposite sinus: A critical appraisal of risk. BMC Cardiovasc Disord 2012;12:83.
2.	Kimbiris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic origin of coronary arteries. Circulation 1978;58:606-15.
3.	Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21:28-40.
4.	Angelini P. Coronary artery anomalies – Current clinical issues: Definitions, classification, incidence, clinical relevance, and treatment guidelines. Tex Heart Inst J 2002;29:271-8.
5.	Verma PK, Bisht DS. Anomalous Origin of Left Main Coronary Artery from Right Sinus of Valsalva: A Case Report. Angiol 2015; 3: 160. doi:10.4172/2329- 9495.1000160.
6.	Ishikawa T, Brandt PW. Anomalous origin of the left main coronary artery from the right anterior aortic sinus: Angiographic definition of anomalous course. Am J Cardiol 1985;55:770-6.
7.	Lorber R, Srivastava S, Wilder TJ, McIntyre S, DeCampli WM, Williams WG, et al. Anomalous aortic origin of coronary arteries in the young: Echocardiographic evaluation with surgical correlation. JACC Cardiovasc Imaging 2015;8:1239-49.
8.	Khalighi K, Sharma M, Toor A, Toor RS, Costacurta G. Anomalous left main coronary artery arising from the right sinus of Valsalva in a young man presenting with recurrent syncope and myocardial infarction. Case Rep Cardiol 2018;2018:9805061.
9.	Turner II, Turek JW, Jaggers J, Herlong JR, Lawson DS, Lodge AJ. Anomalous aortic origin of a coronary artery: Preoperative diagnosis and surgical planning. World J Pediatr Congenit Heart Surg 2011;2:340-5.
10.	Ozeren A, Aydin M, Bilge M, Cam F. Anomalous left coronary artery arising from the right sinus of Valsalva: A case report. Int J Cardiol 2005;101:491-3.
11.	Jadhav M, Pflaumer A, Naimo PS, Konstantinov IE. Anomalous aortic origin of the left coronary artery from the right coronary sinus: Diagnosis and surgical repair of intramural retrovalvular coronary artery. Ann Thorac Surg 2015;100:2357-9.