|Year : 2016 | Volume
| Issue : 2 | Page : 114-116
A Rare case of single coronary artery with atherosclerotic lesions arising from the right sinus of Valsalva
Florian Blaschke1, Florian Krackhardt1, Bherous Kherad1, Burkert Pieske1, Wilhelm Haverkamp1, Matthias Rief2
1 Department of Cardiology, Campus Virchow Klinikum, Charité - University Hospital, Berlin, Germany
2 Department of Radiology, Campus Mitte, Charité - University Hospital, Berlin, Germany
|Date of Web Publication||23-Feb-2016|
Department of Cardiology, Campus Virchow-Klinikum, Charité - Universitaetsmedizin Berlin, Augustenburger Platz 1, Berlin - 13353
Source of Support: None, Conflict of Interest: None
Context: Congenital coronary anomalies, including anomalous origin, distribution, intercoronary communications, and coronary fistulae occur at a rate of approximately 1% in the general population and are the most incidental findings. Case Report: A 49-year-old male patient presented to the emergency department with exercise-induced dyspnea and atypical angina pectoris. Coronary angiography (CAG) and contrast-enhanced 320-slice multidetector cardiac computed tomography with subsequent three-dimensional reconstructions revealed a single coronary artery (SCA) arising from the right sinus of Valsalva with a proximal branch giving rise to the left anterior descending coronary artery. The left anterior descending coronary artery shows severe atherosclerotic lesions and it is occluded afterwards. Adenosine stress perfusion cardiac magnetic resonance imaging (MRI) revealed a stress myocardial ischemia at the anterior wall without signs of fibrosis, scar, or necrosis. Conclusion: We present an extremely rare case of a SCA, with the solitary vessel arising from the right sinus of Valsalva. In our patient's case, the atherosclerotic lesions and occlusion in the branch supplying the anterior wall were considered eligible for neither percutaneous intervention nor bypass graft surgery.
Keywords: Cardiac magnetic resonance imaging (MRI), congenital coronary anomalies, coronary heart disease, multidetector cardiac computed tomography, single coronary artery (SCA)
|How to cite this article:|
Blaschke F, Krackhardt F, Kherad B, Pieske B, Haverkamp W, Rief M. A Rare case of single coronary artery with atherosclerotic lesions arising from the right sinus of Valsalva. North Am J Med Sci 2016;8:114-6
|How to cite this URL:|
Blaschke F, Krackhardt F, Kherad B, Pieske B, Haverkamp W, Rief M. A Rare case of single coronary artery with atherosclerotic lesions arising from the right sinus of Valsalva. North Am J Med Sci [serial online] 2016 [cited 2020 Apr 10];8:114-6. Available from: http://www.najms.org/text.asp?2016/8/2/114/177345
| Introduction|| |
Congenital coronary anomalies, including anomalous origin, distribution, intercoronary communications, and coronary fistulae, are relatively common, occurring at a rate of approximately 1% in the general population.  However, a single coronary artery (SCA), defined as a single aortic orifice, providing blood supply to the entire heart, is an extremely rare congenital anomaly with an incidence of only 0.024-0.066% in the general population. ,, SCA has been reported in association with and without atherosclerotic lesions or in association with other congenital anomalies such as coronary arteriovenous fistulas, bicuspid aortic valve, and hypertrophic cardiomyopathy. ,,
| Case Presentation|| |
A 49-year-old male presented to the emergency department with exercise-induced dyspnea and atypical angina pectoris. He reported a history of reduced exercise capacity since 5 months. On physical examination, his blood pressure was 135/85 mmHg and his heart rate was 82 beats per min. A resting 12-lead electrocardiogram (ECG) displayed sinus rhythm without any ST-segment abnormalities and a complete right bundle branch block [Figure 1]a. Transthoracic echocardiography showed normal biventricular function (left ventricular ejection fraction by biplane Simpson's was 60%) without regional wall motion abnormalities. Mitral valve insufficiency (grade I) and mild aortal and tricuspid regurgitation was noted without signs of increased pulmonary artery pressure. Laboratory testing revealed slightly increased C-reactive protein levels (14.7 mg/L). Cardiac enzymes (troponin T and creatinkinase) and other routine biochemistry test values were within the normal range. His cardiovascular risk-factor profile included untreated hypertension, hyperlipidemia, and a history of cigarette smoking (60 pack years). His family history was negative for heart disease.
|Figure 1: (a) Electrocardiogram (50 mm/s) showing normal sinus rhythm without any ST-segment abnormalities and a complete right bundle branch block. (b) Coronary angiography showing a single coronary artery arising from the right sinus of Valsalva. (c) Aortography in the left anterior oblique view ruled out the presence of any other coronary ostia. RCC: Right coronary cusp. RCA: Right coronary artery|
Click here to view
The patient underwent coronary angiography (CAG) that revealed an SCA originating from the right sinus of Valsalva [Figure 1]b and c. An aortic root angiography was performed to rule out the presence of any other coronary ostia [Figure 1]c. Contrast-enhanced 320-row multidetector cardiac computed tomography with subsequent three-dimensional reconstructions confirmed the diagnosis of a solitary vessel arising from the right sinus of Valsalva. The single vessel courses within the right atrioventricular groove and shows mild luminal irregularities. The left anterior descending (LAD) coronary artery, which emerges as a proximal branch from the solitary vessel, shows significant atheroscerotic lesions and is occluded in the course [Figure 2]. Adenosine stress and rest perfusion cardiac magnetic resonance imaging (MRI) revealed stress-induced perfusion defect at the anterior wall. No significant delayed enhancement as evidence of myocardial fibrosis, scar, or necrosis was observed.
|Figure 2: (a) Cardiac computed tomography angiography showing the rise of the single coronary artery from the right sinus of Valsalva (*) with proximal run of the right coronary artery (RCA). The distal parts of the RCA are not shown in this projection. The single coronary artery crosses the right ventricle and the root of the pulmonary artery (RV/PA) anteriorly and gives rise to the heavily calcified left anterior descending (LAD) coronary artery and further the left circumflex coronary artery (LCX). Furthermore, the left atrium (LA) and the superior vena cava (SVC) are shown. (b) Volume rendering reconstruction of contrast-enhanced 320-row multidetector cardiac computed tomography depicting the single coronary artery. The RCA gives rise to a branch that runs anterior to the pulmonary artery and supplies the LAD and LCX. Furthermore, the Ramus interventricularis posterior (RIVP) and a posterolateral branch (PL) originate from the RCA. PV: Pulmonary vein|
Click here to view
Treatment options such as coronary angioplasty with stenting or coronary artery bypass graft surgery were discussed within the Heart Team. The Heart Team considered the atheroscerotic lesions and occlusion in the branch supplying the anterior wall eligible for neither percutaneous intervention nor bypass graft surgery. Thus, the patient was scheduled for intensified medical therapy to which the patient consented. The patient was treated with acetylsalicylic acid (ASS), angiotensin II type 1 (AT1) receptor blocker, diuretic hydrochlorothiazide, statins, and nitrates and routine cardiac checkups were recommended once in a year.
| Discussion|| |
We present an extremely rare case of an SCA, with the solitary vessel arising from the right sinus of Valsalva. Lipton et al.  classified the SCA in nine patterns according to the origin, anatomical course, and termination of the anomalous vessel. The case presented here enters into the R-IIA pattern, where R implies that the ostium is located in the right sinus of Valsalva. SCA is commonly associated with other congenital anomalies, such as transposition of the great vessels, coronary arteriovenous fistula, or bicuspid aortic valve. However, in our depicted case no other associated coronary anomalies were found. The spectrum of clinical manifestations of coronary anomalies is very broad from clinically benign to severe symptoms including arrhythmias, syncope, myocardial infarction, or sudden death. Invasive CAG is considered an adequate technique for the diagnosis of coronary anomalies. However, the conventional CAG is technically limited to two-dimensional projections and may not be sufficient to classify the anomaly correctly and delineate the precise course of the anomalous vessel. Thus, noninvasive techniques, such as computed tomography or magnetic resonance CAG, which offer three-dimensional reconstructions are necessary to visualize the coronary anatomy accurately. ,
The greatest clinical challenge presented by coronary anomalies is the decision about the treatment. Functional tests such as cardiac MRI or stress echocardiography to detect effort-induced ischemia and myocardial scars are helpful to direct the appropriate treatment. According to the American College of Cardiology/American Heart Association guideline, a surgical revascularization of coronary anomalies with a documented ischemia is recommended.  However, previous studies revealed that standard clinical stress tests often fail to detect myocardial ischemia resulting from coronary anomalies. 
In our patient's case, adenosine stress and rest perfusion cardiac MRI revealed stress induced perfusion defect at the anterior wall. However, as the atherosclerotic lesions and occlusion in the branch supplying the anterior wall were considered eligible for neither percutaneous intervention nor bypass graft surgery, the patient was scheduled for intensified medical therapy and routine cardiac checkups were recommended once in a year.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21:28-40.
Lipton MJ, Barry WH, Obrez I, Silverman JF, Wexler L. Isolated single coronary artery: Diagnosis, angiographic classification, and clinical significance. Radiology 1979;130:39-47.
Desmet W, Vanhaecke J, Vrolix M, Van de Werf F, Piessens J, Willems J, et al
. Isolated single coronary artery: A review of 50,000 consecutive coronary angiographies. Eur Heart J 1992;13:1637-40.
Türkay C, Gölbasi I, Bayezid O. A single coronary artery from the right sinus of valsalva associated with atherosclerosis. Acta Cardiol 2002;57:377-9.
Ogden JA, Goodyer AV. Patterns of distribution of the single coronary artery. Yale J Biol Med 1970;43:11-21.
Hillestad L, Eie H. Single coronary artery. A report of three cases. Acta Med Scand 1971;189:409-13.
Schmid M, Achenbach S, Ludwig J, Baum U, Anders K, Pohle K, et al
. Visualization of coronary artery anomalies by contrast-enhanced multi-detector row spiral computed tomography. Int J Cardiol 2006;111:430-5.
Mandal S, Tadros SS, Soni S, Madan S. Single coronary artery anomaly: Classification and evaluation using multidetector computed tomography and magnetic resonance angiography. Pediatr Cardiol 2014;35:441-9.
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: 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;118:2395-451.
Angelini P, Velasco JA, Flamm S. Coronary anomalies: Incidence, pathophysiology, and clinical relevance. Circulation 2002;105:2449-54.