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A case of left atrial myxoma with unusual tumor vascularity Mahmoud Mohammadzadeh Shabestari, Afsoon Fazlinezhad, Zahra Moravvej, Mohammad Abbasi Tashnizi, Ali Azari and Leila Bigdelu Asian Cardiovascular and Thoracic Annals published online 26 November 2013 DOI: 10.1177/0218492313513776 The online version of this article can be found at: http://aan.sagepub.com/content/early/2013/11/26/0218492313513776

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Case Study

A case of left atrial myxoma with unusual tumor vascularity

Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313513776 aan.sagepub.com

Mahmoud Mohammadzadeh Shabestari1, Afsoon Fazlinezhad2,3, Zahra Moravvej3,4, Mohammad Abbasi Tashnizi5, Ali Azari1,3,6 and Leila Bigdelu1,2,3

Abstract A 69-year-old woman presented with a 2-week history of chest pain and mitral and aortic valve replacement 15 and 5 years earlier. Transthoracic echocardiography revealed a well-seated monoleaflet mitral valve and a bileaflet aortic valve with normal function. Cardiac catheterization showed 3-vessel coronary artery disease and an abnormal cluster of new vessels derived from the left atrial circumflex and posterior ventricle branches. Two- and 3-dimensional transesophageal echocardiography identified a semi-mobile oval left atrial mass with multiple internal cystic spaces, attached to the fossa ovalis region in the interatrial septum. The mass was resected. The pathological diagnosis was atrial myxoma.

Keywords Coronary angiography, coronary disease, heart atria, heart neoplasms, myxoma

Introduction Myxoma is the most common primary cardiac tumor. In some cases, myxomas have vascularity that might be observed in a coronary angiogram.1 Depending on the tumor location, both coronary arteries can supply tumor vascularity. We report an unusual case of left atrial myxoma in which tumor vascularity was derived from both coronary arteries in a woman with previous valve replacement surgeries.

murmur at the left sternal border, with no change on inspiration. An electrocardiogram demonstrated normal sinus rhythm with no ST-segment changes. Chest radiography and blood biochemistry were normal. Based on the report of a cardiologic workup in another center, 2-dimensional transthoracic echocardiography (Philips iE33) revealed a well-seated prosthetic monoleaflet mitral valve and a prosthetic 1

Case report A 69-year-old woman presented with a 2-week history of chest pain, dyspnea, and palpitations. She was admitted to the emergency department with a diagnosis of acute coronary syndrome. She had undergone aortic valve replacement with a bileaflet prosthesis 5 years earlier and also had a successful mitral valve replacement 15 years earlier. The primary cause of mitral and aortic valve dysfunction was rheumatic heart disease. She had no complaint until two weeks before admission. Physical examination indicated normal jugular venous pressure. Auscultation revealed metallic S1 and S2 sounds and a soft grade 2/6 systolic

Atherosclerosis Prevention Research Center, Imam Reza Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 2 Department of Cardiology, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 3 Cardiovascular Research Center, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 4 Students’ Research Committee, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 5 Department of Cardiac Surgery, Imam Reza Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran 6 Department of Cardiac Surgery, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Corresponding author: Leila Bigdelu, Department of Cardiology, Ghaem Hospital, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. Email: [email protected]

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Figure 1. (a) Selective left coronary artery injection in anterior oblique projection showing a prosthetic monoleaflet valve in the mitral position, a prosthetic bileaflet valve in the aortic position, and an abnormal cluster of new vessels from the left atrial circumflex branch (arrow). (b) Selective right coronary artery injection in anterior oblique projection showing the right coronary artery with 2 significant lesions in the mid and distal portions. Small branches of the posterior left ventricular artery (arrow) supply neovascularity.

bileaflet aortic valve with normal transvalvular gradients, normal leaflet motion, and no paravalvular leakage. The left atrium was mildly enlarged. The size and function of the other cardiac chambers were normal. In our center, cardiac catheterization showed 3-vessel coronary artery disease with an abnormal cluster of new vessels derived from the left atrial circumflex branch and the posterior left ventricular branch (Figure 1). Normal motion of the prosthetic valves was confirmed by fluoroscopy. Subsequent 2- and 3-dimensional transesophageal echocardiography (Philips iE33) identified a 25  26-mm semi-mobile oval left atrial mass with multiple internal cystic spaces, which was attached to the fossa ovalis region in the interatrial septum by a broad-based stalk (Figure 2, Video 1, Supplementary material). These findings suggested cardiac myxoma. The mass was resected surgically, and the small branches supplying the tumor were ligated. Pathological analysis revealed it to be a 28  20  30-mm gelatinous mass attached to a 20  25  20-mm stalk of atrial septal tissue, and the diagnosis of myxoma was confirmed. A recent followup examination showed that the general status of the patient was satisfactory, and she is currently on treatment with warfarin.

Discussion Myxoma is the most common primary cardiac tumor and accounts for 30% to 50% of all primary cardiac tumors.2 Myxomas can occur in various ages but are frequent in adults 30 to 50 years of age; 70% of patients are women. Approximately 75% of these tumors arise from the left atrium, usually from the fossa ovalis region, and 18% arise from the right atrium. The few remaining tumors originate from atypical sites such as the left or right ventricle and valves.2 They are often

Figure 2. Two-dimensional transesophageal echocardiogram. A left atrial oval mass is visible on the interatrial septum in the fossa ovalis region, with multiple internal echo-free spaces (grape in heart).

solitary, but in the familial form, they can be multiple and in any intracardiac location. Cardiac myxoma may cause a variety of nonspecific constitutional symptoms including fatigue, fever, weight loss, and embolic and obstructive symptoms.3 The cerebral circulation is the most frequent site of embolic complications. Obstructive symptoms vary depending on tumor location and include dyspnea, syncope, and dizziness. Myxoma is not usually a highly vascularized mass, but in some cases it may have a rich blood supply. Van Cleemput and colleagues4 reviewed the angiographic findings of 19 patients: myxomas were located in the left atrium in 17, and 7 had highly vascular myxomas; the right coronary artery and left circumflex supplied the tumor in 4 and 3 cases, respectively. Our patient had an unusual myxoma that received blood from both coronary arteries. Left atrial myxoma blood supply from the left and right coronary arteries is an

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unusual finding and has, to our knowledge, only been reported in 2 patients.5,6 The role of coronary angiography in determination of a tumor is not well understood. However, it is currently a diagnostic tool for evaluation of concomitant coronary artery disease. In this case, the tumor was supplied by the left circumflex and right coronary artery; the mass had septations and echo-free spaces due to internal hemorrhage. According to some researchers, highly vascularized myxomas are prone to internal bleeding, resulting in rapid enlargement.1 Transthoracic echocardiography is the best initial tool for identifying a cardiac mass. In addition, it can provide information about tumor characteristics, but it may be suboptimal when a prosthetic mitral valve is present and its acoustic shadow interferes with good visualization of the left atrium. In such cases, 2- or 3-dimensional transesophageal echocardiography may play a better diagnostic role. The treatment of choice for myxoma is prompt surgical excision.2 Recurrence rates are 0% to 5% for sporadic myxomas and up to 20% for familial myxomas. Therefore, these patients must be followed up with serial echocardiograms.

References 1. Chow WH, Chow TC, Tai YT, Yip AS and Cheung KL. Angiographic visualization of ‘tumour vascularity’ in atrial myxoma. Eur Heart J 1991; 12: 79–82. 2. Reynen K. Cardiac myxomas. N Engl J Med 1995; 333: 1610–1617. 3. Amano J, Kono T, Wada Y, et al. Cardiac myxoma: its origin and tumor characteristics [Review]. Ann Thorac Cardiovasc Surg 2003; 9: 215–221. 4. Van Cleemput J, Daenen W and De Geest H. Coronary angiography in cardiac myxomas: findings in 19 consecutive cases and review of the literature. Cathet Cardiovasc Diagn 1993; 29: 217–220. 5. Alizade E, C¸akır H, Ac¸ar G and Pirmammadova C. Giant left atrial myxoma with left and right coronary system blood supply accompanying mitral stenosis; realtime three- dimensional echocardiography imaging. Anadolu Kardiyol Derg 2013; 13: 27–29. 6. Rasmussen KK, Peeples TC and Nellen JR. Unusual variant in tumor vascularity associated with left atrial myxoma. AJR Am J Roentgenol 1983; 141: 927–928.

Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest statement None declared.

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A case of left atrial myxoma with unusual tumor vascularity.

A 69-year-old woman presented with a 2-week history of chest pain and mitral and aortic valve replacement 15 and 5 years earlier. Transthoracic echoca...
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