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 Table of Contents  
Year : 2017  |  Volume : 1  |  Issue : 4  |  Page : 149-152

Symptomatic high-risk congenital coronary anomaly

1 Department of Internal Medicine, St. Louis University School of Medicine, St. Louis, Missouri, USA
2 Department of Cardiovascular Medicine, The Center for Comprehensive Cardiovascular Care, St. Louis University School of Medicine, St. Louis, Missouri, USA
3 MedStar Washington Hospital Center, Washington, DC, USA
4 Department of Cardiovascular Medicine, The Center for Comprehensive Cardiovascular Care, St. Louis University School of Medicine; Department of Cardiology, John Cochran Veterans Affairs Medical Center, St. Louis, Missouri, USA

Date of Web Publication29-Oct-2018

Correspondence Address:
Dr. Nauman Khalid
MedStar Washington Hospital Center, 110 Irving Street, NW, Suite 4B-1, Washington, DC 20010
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/hm.hm_6_18

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Anomalous coronary arteries are rare entities with certain categories being potentially malignant with the risk of sudden cardiac death. We present two cases of the highest risk variant consisting of an anomalous left coronary artery arising from the right sinus of Valsalva traversing between the aorta and pulmonary artery leading to recurrent syncope and chest pain in middle-aged patients.

Keywords: Anomalous, coronary artery, sinus of Valsalva

How to cite this article:
Oman Z, Ampadu J, Khalid N, Abraham L, Helmy T, Nasir A. Symptomatic high-risk congenital coronary anomaly. Heart Mind 2017;1:149-52

How to cite this URL:
Oman Z, Ampadu J, Khalid N, Abraham L, Helmy T, Nasir A. Symptomatic high-risk congenital coronary anomaly. Heart Mind [serial online] 2017 [cited 2023 Mar 25];1:149-52. Available from: http://www.heartmindjournal.org/text.asp?2017/1/4/149/244374

  Introduction Top

Anomalous coronary arteries arising from the opposite sinus (ACAOS) is an uncommon event occurring in 301 cases out of 210,700 (0.14%) cardiac catheterizations in a prior study.[1] The incidence of the right coronary artery (RCA) originating from the left sinus of Valsalva (R-ACAOS) has been found to occur in 79%–92% of ACAOS cases, whereas left coronary artery (LCA) originating from the right sinus of Valsalva (L-ACAOS) occurs in 15%–21% of ACAOS cases.[2],[3] The interarterial course (IAC) of the L-ACAOS has been found in 0.03%[4] of patients undergoing coronary angiography and is associated with up to 57% of the sudden cardiac death cases of younger adults found at autopsy.[1],[3] We present two patient cases in their fifth and sixth decades of life presenting with syncope and atypical chest pain found to have L-ACAOS with IAC.

  Case Reports Top

Case 1

A 58-year-old male with medical history of hyperlipidemia, anemia, and tobacco use presented with recurrent syncope and atypical chest pain. A 12-lead electrocardiogram (ECG) showed normal sinus rhythm at 60 bpm. Patient underwent treadmill stress test during which he developed 2.9-mm ST-segment depression in lead V5 at 8 minutes which resolved after 2 minutes into the recovery period; however the patient denied any associated symptoms. Resting echocardiogram showed a left ventricular ejection fraction (LVEF) of 65% without regional wall motion abnormalities. The patient underwent coronary angiography for ischemic evaluation which revealed no obstructive epicardial coronary disease. However, it revealed a single coronary ostium arising from the right sinus of Valsalva, immediately bifurcating into the left main coronary artery and RCA. Computerized tomography (CT) coronary angiography was performed for further anatomical characterization which confirmed an IAC course of the LCA between the aorta and pulmonary artery (PA) [Figure 1].
Figure 1: (a-c) Coronary angiograms showing common ostium of the right coronary artery and left coronary artery from the right sinus of Valsalva. Left main coronary artery transverses between the aorta and pulmonary artery. (d-f) Computed tomographic angiography displaying common ostium of coronary arteries from the right sinus of Valsalva with left main coronary artery interarterial course between the aorta and pulmonary artery. (g-i) Cardiac computerized tomography with three-dimensional replication of cardiac anatomy showing common ostium from the right sinus of Valsalva (green asterisk) and left main coronary artery coursing posterior to the aorta. RCA=Right coronary artery, LCA=Left coronary artery, RSOV=Right sinus of Valsalva, LM=Left main coronary artery, AO=Aorta, PA=Pulmonary artery

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Case 2

A 62-year-old male with medical history of paroxysmal atrial fibrillation, hypertension, and chronic alcohol use presented with recurrent syncope and atypical chest pain. A 12-lead ECG demonstrated normal sinus rhythm with left atrial enlargement and prior inferior infarct. A Lexiscan Cardiolite nuclear stress test revealed LVEF of 50% without evidence of stress-induced ischemia or prior infarction. A chest CT angiogram, performed for concerns of aortic dissection secondary to an echolucency noted on a transthoracic echocardiogram, was negative for aortic dissection. However, this did reveal an anomalous origin of the LCA from the right sinus of Valsalva, with an IAC between the aorta and PA. Cardiac catheterization confirmed these findings, adding that the LCA ostium was anterior to and separate from the RCA ostium [Figure 2]. A treadmill stress test was performed with no inducible chest pain or syncope.
Figure 2: (a) Coronary angiogram showing both the right coronary artery and left coronary artery coronary ostium originating from the right sinus of Valsalva; left coronary artery ostium slightly anterior and separate from right coronary artery ostium. (b) Computerized tomography angiogram showing interarterial course of left main coronary artery between the aorta and pulmonary artery. RCA=Right coronary artery, LCA=Left coronary artery, LM=Left main coronary artery, LCX=Left circumflex coronary artery, AO=Aorta, PA=Pulmonary artery

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Due to the significance of these symptomatic incidental findings and its associated risk of sudden cardiac death, both these patients were referred to cardiac surgery for definitive therapy.

  Discussion Top

ACAOS are rare, affecting 0.3%–5.6% of patients who undergo coronary angiography. Anomalous coronary arteries are the second-most common cause of death among young athletes with 33%–57% of cases secondary to L-ACAOS with an IAC.[2],[3] In L-ACAOS, the anomalous proximal LCA can take one of five pathways: prepulmonic, petro-aortic, IAC, transseptal, and retrocardiac [Figure 3].[2] Depending on the route taken by the anomalous coronary artery and degree of impaired coronary blood flow, patients may experience palpitations, dyspnea, angina, syncope, or sudden cardiac death.
Figure 3: Conceptual diagrams for the visualization of the five different pathways in which the right coronary artery, left anterior descending artery, and left circumflex artery can potentially connect to the opposite coronary cusp. AL=Antero-left; AR=Antero-right; P=Posterior; M=Mitral valve; T=Tricuspid valve. Reproduced from Angelini et al. with permission from Lippincott Williams and Wilkins. Copyright 1999 (2)

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These symptoms are often secondary to coronary hypoplasia, acute angle takeoff with a slit-like opening of the coronary ostium, intracoronary atherosclerosis with stenotic lesions, and lateral compression limiting coronary perfusion.[3] These symptoms are exacerbated with physical exertion as the heart rate increases creating less diastolic coronary perfusion time, increased myocardial oxygen demand, and engorgement of aorta and PA compressing the anomalous portion of the LCA.[4]

Typically, this coronary anomaly is found in younger individuals, but it can also be diagnosed at a later age as noted in both of our patients. ACAOS patients, who have an IAC tend to be younger, have a higher association of ischemia presenting with chest pain and are commonly found to have L-ACAOS.[3] We believe that our patients presented with symptoms later in life most likely due to lack of heavy physical exertion at younger ages, absence of uncontrolled hypertension, and that neither of them had significant ventricular hypertrophy necessitating increased oxygen requirements.

While there are no specific guidelines for the management of patients with ACOS, treatment options include observation, medical therapy, percutaneous coronary intervention, and surgical repair depending on the specific coronary anomaly and patient symptoms. The ACC/AHA Adult Congenital Heart Disease management guidelines give a Class IB recommendation for surgical correction of anomalous coronary arteries in the following scenarios: (1) an anomalous left main from the right sinus of Valsalva coursing between the aorta and PA, (2) evidence of coronary ischemia from an intramural/between the great vessels course of anomalous coronary artery, or (3) RCA from the left sinus of Valsalva passing between the aorta and PA with documented ischemia.[5] Several surgical approaches have been utilized for correction of anomalous coronary arteries, direct reimplantation of the anomalous coronary artery into the aorta, coronary artery bypass grafting, ligation of the anomalous coronary, and coronary artery baffling procedures. Each approach is guided by the specific anatomy of the anomalous coronary artery and carries its own set of risk and complications, as well as benefit. With bypass grafting, complications of graft patency, regression in collaterals, and reduction in coronary artery size have been described due to competitive flow between the graft and the native coronary artery. Simple ligation of the anomalous coronary artery avoids this complication, but dependent on the coronary anatomy may have continued risk of subendocardial ischemia, angina, and sudden cardiac death.[6] Specifically, for an anomalous coronary artery of the LCA arising from the PA, the Takeuchi technique has been employed. This technique involves establishing a pulmonary arteriotomy, transverse flap of PA tissue, and aortopulmonary window. The transverse flap is this used as a baffle for the LCA into the aorta. This technique carries a unique set of complications, supravalvular pulmonary stenosis, and baffle leak.[7]

  Conclusion Top

There are many variations of anomalous coronary arteries, as well as varying clinical manifestations and ramifications. Most of these are incidentally found, adding to the difficulty of predicting which are benign or potentially fatal. Comprehensive imaging and testing are required, along with clinical features, to best individualize the appropriate treatment option for these patients.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Krasuski RA, Magyar D, Hart S, Kalahasti V, Lorber R, Hobbs R, et al. Long-term outcome and impact of surgery on adults with coronary arteries originating from the opposite coronary cusp. Circulation 2011;123:154-62.  Back to cited text no. 1
Angelini P. Coronary artery anomalies: An entity in search of an identity. Circulation 2007;115:1296-305.  Back to cited text no. 2
Kochar A, Kiefer T. Coronary artery anomalies: When you need to worry. Curr Cardiol Rep 2017;19:39.  Back to cited text no. 3
Anantha Narayanan M, DeZorzi C, Akinapelli A, Mahfood Haddad T, Smer A, Baskaran J, et al. Malignant course of anomalous left coronary artery causing sudden cardiac arrest: A Case report and review of the literature. Case Rep Cardiol 2015;2015:806291.  Back to cited text no. 4
Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults with Congenital Heart Disease). Developed in Collaboration with the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008;52:e143-263.  Back to cited text no. 5
Rajbanshi BG, Burkhart HM, Schaff HV, Daly RC, Phillips SD, Dearani JA, et al. Surgical strategies for anomalous origin of coronary artery from pulmonary artery in adults. J Thorac Cardiovasc Surg 2014;148:220-4.  Back to cited text no. 6
Zhang HL, Li SJ, Wang X, Yan J, Hua ZD. Preoperative evaluation and midterm outcomes after the surgical correction of anomalous origin of the left coronary artery from the pulmonary artery in 50 infants and children. Chin Med J (Engl) 2017;130:2816-22.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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