Download A Rare Case of a Congenital Single Coronary Artery: Right

CASE REPORT
A Rare Case of a Congenital Single
Coronary Artery: Right Coronary Artery
Originating From the Distal Left
Circumflex Artery
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Florian Blaschke, MD1, Elke Zimmermann, MD2, Johannes Greupner, MD2, Dietlind Zohlnhoefer, MD1,
Florian Krackhardt, MD1, Wilhelm Haverkamp, MD1, Mattias Roser, MD1
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From 1Charite, Universitaetsmedizin Berlin, Campus Virchow-Klinikum, Department of Cardiology and 2Charite, Universitaetsmedizin Berlin, Campus Virchow-Klinikum, Department of Radiology.
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ABSTRACT: A 59-year-old female was admitted to the emergency department with retrosternal pain that began
that morning. A resting electrocardiogram showed sinus rhythm without ST-segment abnormalities. In addition,
cardiac enzymes (troponin T, CK) were within the normal range. Coronary angiography and contrast-enhanced
320-slice multidetector computed tomography with subsequent 3-dimensional reconstruction revealed the absence of the right coronary artery (RCA) and a left circumflex artery (LCX) supplying the right coronary artery territory. This case describes the extremely rare anomaly of a congenital single coronary artery, with the RCA arising
as a terminal extension of the LCX.
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VASCULAR DISEASE MANAGEMENT 2013;10(11):E244-E247
Key words: anomalies, interventional cardiology, coronary disease, computed tomography,
stress echocardiography
n October 2012, a 59-yearold female presented to the
emergency department with
retrosternal pain with radiation
to the left arm since the morning.
She reported a history of exerciseinduced dyspnea and stable angina
pectoris for several months. On
physical examination, her blood
pressure was 126/66 mm Hg and her
heart rate was 80 beats per minute.
A resting electrocardiogram displayed sinus rhythm without any
ST-segment abnormalities (Figure
1A). Transthoracic echocardiography
demonstrated normal biventricu-
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lar function (left ventricular ejection fraction by biplane Simpson’s
method was 65%) without regional
wall motion abnormalities. Mild mitral and tricuspid regurgitation was
noted without sign of increased pulmonary artery pressure. Laboratory
testing revealed slightly increased Creactive protein levels (12.8 mg/L)
and suppressed TSH (0.06/L) with
normal FT3 and FT4 values.
Cardiac enzymes (troponin T,
CK) and other routine biochemistry
test values were within the normal
range. Past medical history revealed
hypertension, hyperlipidemia, and
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a history of cigarette smoking (50
pack years) as atherosclerotic risk
factors. Family history was negative for heart disease. The coronary
angiography showed a left anterior
descending coronary artery (LAD)
arising normally from the left coronary sinus without significant atherosclerotic lesions (Figure 1B, 1C,
and 1D).
The left circumflex artery (LCX)
was the dominant vessel with its terminal branch covering the territory
of the right coronary artery (RCA)
(Figure 1C, 1D). Since we were not
able to cannulate the right coronary
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CASE REPORT
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tion, intercoronary communications,
and coronary fistulae are relatively
common, occurring at a rate of approximately 1% in the general population.1 However, a single coronary
artery (SCA) is an extremely rare
congenital anomaly with an incidence
of only 0.024% to 0.066% in the general population.1-3 Lipton et al2 classified the SCA in 9 patterns according
to the origin, anatomical course, and
termination of the anomalous vessel.
The case presented here enters into
the L-I pattern, where L implies that
the ostium is located in the left sinus
of Valsalva. Single coronary artery is
commonly associated with other congenital anomalies, such as transposition of the great vessels, coronary arteriovenous fistula, or bicuspid aortic
valve.4 However, in our depicted case
no other associated cardiac anomalies
were found and coronary obstructive lesions were absent. Although
SCA is generally considered benign,
this anomaly has been associated with
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artery, aortic root angiography was
performed to exclude an atypical
take-off of the RCA. Aortography
(Figure 1B) and contrast-enhanced
320-slice multidetector cardiac computed tomography with subsequent
3-dimensional reconstructions confirmed the absence of the RCA and
an LCX supplying the RCA territory
(Figure 1E, 1F). Submaximal stress
echocardiography did not show signs
of exercise-induced myocardial ischemia. Due to the submaximal exercise
(maximal workload 75 Watt) and the
higher diagnostic accuracy of stressperfusion cardiac magnetic resonance
imaging (MRI), an adenosine stress
perfusion cardiac MRI was performed
which indicated no evidence of ischemia. In addition, late gadolinium enhancement did not reveal myocardial
scars or areas of fibrosis.
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Figure 1 A and 1B. Electrocardiogram (50 mm/sec) showing normal sinus rhythm with no ST-segment abnormalities (A). Aortography in the
left anterior oblique view showing absence of the right coronary ostium (B).
DISCUSSION
Congenital coronary anomalies, including anomalous origin, distribu-
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congestive heart failure, acute myocardial infarction, and sudden cardiac
death in the absence of atherosclerosis.5-7 Thus, Shirani et al8 reported that
15% of the patients with SCA had
myocardial ischemia as direct consequence of the anomaly. Various mechanisms, including anatomical features
such as the compression of the anomalous vessel along its course between
the aorta and the pulmonary artery
or sporadic spasm of the anomalous
coronary artery, have been postulated.
The greatest clinical challenge presented by coronary anomalies is the
decision about the treatment. Due to
the extremely rare variant of the SCA
described in our case, it is difficult to
predict whether patients with L-I type
SCA are at high risk or will have a benign course. According to an American College of Cardiology/American
Heart Association guideline, a surgical
revascularization of coronary anomalies with a documented ischemia is
recommended.9 However, previous
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CASE REPORT
Figure 1C-1F. Angiogram showing that the terminal branch of the left circumflex artery (LCX) is covering the territory of the right coronary
artery (RCA) (C, D). Contrast-enhanced 320-slice multidetector cardiac computed tomography showing absence of the RCA and an extended
LCX supplying the territory of the right coronary artery (E, F). LCC: left coronary cusp; RCC: right coronary cusp; LMCA: left main coronary
artery.
studies revealed that standard clinical
stress tests often fail to detect myocardial ischemia resulting from coronary
anomalies.10
CONCLUSION
We present an extremely rare case
of an SCA, with the RCA arising as a
terminal extension of the LCX. In our
patient’s case, coronary angiography re-
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vealed no significant atherosclerotic lesions or abnormally slow angiographic
filling to the RCA territory. In addition,
stress echocardiography and adenosine
stress MRI indicated no evidence of
exercise-induced ischemia. Thus, the
patient was discharged with beta blocker and ACE inhibitor for the treatment
of hypertension and routine cardiac
check-ups were recommended once a
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year. However, physicians must be aware
that subjects with SCA are particularly susceptible to deleterious effects of
atherosclerotic occlusive disease as the
heart and the conduction system entirely depend on the SCA for oxygenated
blood supply.
Editor’s Note: Disclosure: The authors have completed and returned the
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CLINICAL REVIEW
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1.Yamanaka O, Hobbs RE. Coronary artery
anomalies in 126,595 patients undergoing
coronary arteriography. Cathet Cardiovasc Diagn.
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Yonsei Med J. 2011;52(5):856-858.
8. S hirani J, Roberts WC. Solitary coronary ostium in the aorta in the absence of other major
congenital cardiovascular anomalies. J Am Coll
Cardiol. 1993;21(1):137-143.
9. Warnes CA, Williams RG, Bashore TM, et al.
ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease:
Executive Summary: a report of the American
College of Cardiology/American Heart Association Task Force on Practice Guidelines
(writing committee to develop guidelines for
the management of adults with congenital
heart disease). Circulation. 2008;118(23):23952451.
10. Angelini P,Velasco JA, Flamm S. Coronary anomalies: incidence, pathophysiology, and clinical relevance. Circulation.
2002;105(20):2449-2454.
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REFERENCES
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ICMJE Form for Disclosure of Potential
Conflicts of Interest. The authors report no
disclosures in relation to the content of this
manuscript.
Manuscript received May 3, 2013; provisional acceptance given May 22, 2013;
manuscript accepted July 17, 2013.
Address for correspondence: Florian
Blaschke, MD, Charite, Universitaetsmedizin Berlin, Campus Virchow-Klinikum,
Department of Cardiology, Berlin, Germany. Email: [email protected] n
CASE REPORT
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