Cardiol Cardiovasc Med 2021; 5 (2): 272-276 DOI: 10.26502/fccm.

92920200

Case Report

Single Coronary Artery Anomaly: Case Report

Firas Ajam1*, Ndausung Udongwo2, Vandan Updahyaya2, Bharath Sathya1, Daniel Kiss1

1
Department of Cardiology, Jersey Shore University Medical Center, New Jersey, United States
2
Department of Internal Medicine, Jersey Shore University Medical Center, New Jersey, United States

*Corresponding Author: Firas Ajam, Department of Cardiology, Jersey Shore University Medical Center, 1945
Route 33, Neptune, NJ 07712, USA

Received: 23 March 2021; Accepted: 31 March 2021; Published: 30 April 2021

Citation: Firas Ajam, Ndausung Udongwo, Vandan Updahyaya, Bharath Sathya, Daniel Kiss. Single Coronary
Artery Anomaly: Case Report. Cardiology and Cardiovascular Medicine 5 (2021): 272-276.

Abstract angiographic classification was proposed by Lipton

Single coronary anomaly is a rare congenital et al in 1979, and modified in 1990 [1, 3].
coronary artery disease where only one coronary
artery arises from the aortic trunk, it could be an 2. Case Presentation
isolated finding or as part of other congenital cardiac A 55-year-old male with a history of hypertension,
diseases. We sought to present a patient with anginal hyperlipidemia, pulmonary embolism, former smoker
symptoms was found to have single coronary artery. for 12 years, and history of supraventricular
tachycardia, who was sent for an elective cardiac
Keywords: Absent right coronary artery; Ischemic catheterization. He’s been having recurrent episodes
heart disease; Congenital heart disease of retrosternal chest pain, lasting 10-15 minutes,
exertional, relieved with rest, associated with

1. Introduction shortness of breath, and no palpitations. Family

Single coronary artery anomaly is a rare congenital history was remarkable for coronary artery disease.

coronary artery disease, found as an isolated anomaly Patient denied any cough, fever or recent trauma.

in approximately (0.024%-0.044%) [1]. The first case Home medications: metoprolol succinate (50mg

was reported by Thebesius in 1761 [2]. The first daily) and aspirin (81mg daily). Vital signs: BP

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Cardiol Cardiovasc Med 2021; 5 (2): 272-276 DOI: 10.26502/fccm.92920200

168/96 mmHg, HR 70 bpm, temp 97 F. Physical artery (LCx) extending into the right coronary artery
examination was within normal limits. (RCA) territory and terminating near the right sinus
Electrocardiogram (EKG): normal sinus rhythm with of Valsalva, the RCA origin was unclear, with an
a rate of 60 bpm, no ischmic changnes. Computed absent ostium and proximal segment (Figure 1).
Tomography Cornoary Angiography showed non- Coronary angiography was recommended to evaluate
obstructive calcified plaque in the mid left anterior if the patient had a dominant left circumflex artery
descending artery (LAD) (coronary artery calcium (LCx), and an obstructed proximal RCA or an
score of 25.6 agatston units), the left circumflex anomaly.

Figure 1: (A) CT Coronary Angiography showing the course of Left Circumflex (LCX) Artery; (B) CTA coronary
reconstruction showing the course of Left Circumflex Artery; (C) CTA coronary reconstruction showing the course
of the Left Anterior Descending (LAD) Artery.

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Cardiol Cardiovasc Med 2021; 5 (2): 272-276 DOI: 10.26502/fccm.92920200

Figure 2: Left Anterior Oblique (LAO) coronary angiography showing the course of Left Circumflex artery (LCX).

Figure 3: Right Anterior Oblique (RAO) coronary angiography of the LAD and LCX arteries.

Cardiac catheterization revealed the left coronary atrioventricular (AV) groove and supplies the right
artery originating from the left ostium giving rise to coronary territory, giving off the posterior descending
the left anterior descending (LAD) and Left artery (PDA) and several acute marginals, several
Circumflex (LCx) arteries. LCx gives rise to four attempts were made to visualize the RCA and was
obtuse mariginals (OMs) and continues in the deemed to be absent (Figures 2, 3). Overall, there were

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Cardiol Cardiovasc Med 2021; 5 (2): 272-276 DOI: 10.26502/fccm.92920200

no hemodynamically significant coronary artery Impaired myocardial perfusion can be related to

obstructions, only very mild coronary disease. The anatomical malformations, including the acute angle
patient was managed medically and discharged in take-off of the anomalous vessel, with a tapered slit-
stable condition, and was scheduled for outpatient like orifice that breakdowns in a valve-like manner,
follow up. thereby limiting the blood flow. Other anatomical
structures responsible for ischemia are the proximal
3. Discussion intramural course of the anomalous vessel, which is
The incidence of SCA according to Lipton et al was squeezed within the aortic wall, and the compression
0.024% and according to Yamanaka and Hobbs was of the anomalous vessel along its path between the
0.044%. A retrospective analysis by Desmet et al. aorta and the pulmonary artery during exercise as both
showed low incidence of associated congenital heart vessels get larger [5, 6]. Taylor et al observed the
disease and suggested no association brtween SCA and records of 242 deceased patients with remote
certain other diseases [4]. In a study of 142 patients congenital coronary anomalies and found that one-
with an SCA and associated congenital heart disease, third of the patients had sudden cardiac death, and half
in 49% SCA arose from the right sinus of Valsalva of these were exercise-related deaths, with younger
(SoV), and 45% arose from the left SoV. Major patients were obsereved to die suddenly during
congenital cardiac anomalies were reported in 41%, physical exertion (Taylor AJ, Rogan KM). Moreover,
the most common anomalies were transposition of the Basso et al. claim that (59%) of patients with SCA,
great vessels, coronary artery fistula, improper division died under the age of 30 years of age, mainly during
of the truncus arteriosus, tetralogy of Fallot and exercise [6].
bicuspid aortic valve.
Coronary angiography is the gold standard to
Lipton’s classification considers the beginning of the diagnose, assess and classify coronary anomalies,
ostium from the sinus of Valsalva, anatomical path of echocardiography can be used to detect any other
the vessel, and the sequence of the transverse trunk. associated structural heart diseases. Cardiovascular
Alphabets R or L are used to identify the ostial origin magnetic resonance (CMR) is an alternative, non-
of the vessel, roman numerals I, II, or III are used to invasive tool, with the advantages of no radiation
represent the anatomical distribution of the vessel, and exposure, no body habitus limitations, and
letters A, B, P, S, and C are used to outline the instantaneous assessment of cardiovascular structure
progression of the vessel with respect to the pulmonary and perfusion, it aslo provides high spatial and
artery and the aorta. The majority of patients are temporal resolutions [7]. Percutaneous Coronary
asymptomatic. However, around 15% might have Intervention (PCI) considered the gold starndartd for
ischemic heart disease directly triggered by the treatement, the abnormal source and path of coronary
atypical anatomy of the arteries and not by coronary arteries might be challenging and could lead to
artery disease. Ischemic symptoms might range from complications in cannulation of the coronary ostium as
exertional angina to sudden death in rare types. well as technical difficulties in catheter support during

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Cardiol Cardiovasc Med 2021; 5 (2): 272-276 DOI: 10.26502/fccm.92920200

PCI [8, 9]. Cardiol 19 (1967): 424-427.

4. Desmet W, Vanhaecke J, Vrolix M, Van de
4. Conclusion Werf F, Piessens J, et al. Isolated single
Single coronary anomaly is a rare congenital disease coronary artery: a review of 50,000 consecutive
with wide variety of symptoms, ranging from anginal coronary angiographies. Eur Heart J 13 (1992):
symptoms to sudden cardiac death. Multi-disciplinary 1637-1640.
approach should be used in evaluating those patients. 5. Angelini P. Coronary artery anomalies-current
Further studies are recommended to help in earlier clinical issues: Definitions, classification,
diagnosis and management. incidence, clinical relevance, and treatment
guidelines. Tex Heart Inst J 29 (2002): 271-278.

Funding 6. Basso C, Maron BJ, Corrado D, Thiene G.

No funding or sponsorship was received for Clinical profile of congenital coronary artery

publication of this article. anomalies with origin from the wrong aortic
sinus leading to sudden death in young

Authorship competitive athletes. J A Coll Cardiol 35

(2000): 1493-1501.
All named authors take responsibility for the integrity
7. Rudan D, Todorovic N, Starcevic B, Raguz M,
of the work as a whole, and have given their approval
Bergovec M. Percutaneous coronary
for this version to be published.
intervention of an anomalous right coronary
artery originating from the left coronary artery.
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