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European Journal of Echocardiography 2008 9(1):171-172; doi:10.1016/j.euje.2007.06.019
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org

‘Giant fleshy leaflet myxoma’

Ali Reza Moaref1, Reza Mollazadeh1,*, Ahmad Ali Amirghofran2, Bita Geramizadeh3 and Salma Sefidbakht3

1 Cardiology Department, Nemazee Hospital, Shiraz University of Medical Science, Zand Avenue, P.O. Box: 71435-1414, Shiraz, Iran
2 Cardiac Surgery Department, Nemazee Hospital, Shiraz University of Medical Science, Shiraz, Iran
3 Pathology Department, Shiraz Medical School, Shiraz University of Medical Science, Shiraz, Iran

Received 19 May 2007; accepted after revision 20 June 2007; online publish-ahead-of-print 10 September 2007.

* Corresponding author. Tel: +98 (0)917 313 3749; fax: +98 (0)711 6261089. E-mail address: mollazar{at}yahoo.com


    Abstract
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A 25-year-old woman was admitted to the neurology department because of sudden onset of weakness of her left upper and lower extremities. Cardiac auscultation revealed an extra sound. Transesophageal echocardiography showed a huge mobile fleshy mass on the atrial side of anterior mitral leaflet protruding into the left ventricle during diastole. A large irregularly shaped mass resembling a ‘cluster of grapes’ was found on the atrial side of anterior mitral leaflet. The mass was excised and mitral valve was repaired. Histology was diagnostic for myxoma. Six months clinical and echocardiographic follow-up was normal and did not show any recurrence of tumor.

Keywords: Myxoma; Leaflet; Cardiac; Atrial; Stroke


    Case presentation
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A 25-year-old woman was admitted to the neurology department because of sudden onset weakness of her left upper and lower extremities a few hours earlier. She mentioned that she had transient episodes of vertigo and blurred vision during the last week which spontaneously ameliorated. She denied any (cocaine or heroin) drug consumption. She has no family history of early onset stroke or coronary artery disease. She was afebrile and her blood pressure was normal. Her pupils' reaction to light was brisk bilaterally. Cardiac auscultation revealed an extra diastolic sound. She was well oriented to time, place and person. Motor power was 3 out of 5 at her left side. ECG showed tachycardia and was otherwise normal. Brain CT scan showed right hemisphere infarction in the parietal lobe. The patient was sent to the echocardiography laboratory for evaluation of the abnormal cardiac sound. Transthoracic echocardiography showed a large mass on the mitral valve. Transesophageal echocardiography delineates the mass as a large mass on the atrial side of anterior mitral leaflet (Figure 1, Movie clip 1 and 2) partially obstructing the mitral orifice during diastole (Movie clip 3). The patient underwent surgery and a large (2 x 2.5 cm) irregularly shaped mass resembling ‘cluster of grapes’ was found on the atrial side of anterior mitral leaflet. The mass was excised and mitral valve was repaired. Histologic examination revealed stellate tumor cells surrounded by a highly myxoid stroma diagnostic of myxoma (Figure 2). Postoperative course was uneventful and she was still well at six months follow-up.


Figure 1
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Figure 1 Transesophageal echocardiography in low esophageal level shows a huge fleshy mass on atrial side of anterior mitral leaflet.

 


Figure 2
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Figure 2 (A) Sections from tumor show low cellularity with concentric arrangement of tumor cells around vessels (arrow) (H&E x 100). (B) High power view of the stellate cell (arrow) (H&E x 400).

 
Myxomas are the most common primary cardiac tumor. They commonly occur in the left atrium and generally in the region of the fossa ovalis.1 Exceptionally they may arise from a cardiac valve. The tricuspid valve is the most frequent location of these valvular myxomas2 followed by mitral, aortic and pulmonary valves.3 Mitral valve myxomas were located mainly on the atrial side of valve (except one report on ventricular side4) with an equivalent distribution between the anterior and posterior leaflets.3 A review of the literature revealed that a diagnosis of left mitral myxoma was based on the findings of tumor embolization, suggesting that a myxoma in the mitral valve produces early embolization compared to other cardiac myxomas.3,5 Generally there are two types of atrial myxoma based on echo examination: (1) those that are well encapsulated; these are more likely to be encountered as incidental findings; (2) those that are highly mobile and amorphous (such as ours, Movie clip 2) which often results in symptomatic emboli (most commonly neurologic dysfunction).3 Diagnosis of this entity is generally made by echocardiography. Most cases can be diagnosed using transthoracic echocardiography but may be missed if it measures less than 5 mm in diameter. Transesophageal echocardiography allows the early detection of small valvular tumors and may help to characterize better the location and echo structure of these lesions. In addition, transesophageal echocardiography can guide the surgical approach by revealing the integrity and mobility of the valve before operation whether to perform mitral valve repair or replacement. Once a presumptive diagnosis of myxoma has been made on imaging studies, prompt resection is required because of the risk of embolization or cardiovascular complications.1


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Supplementary material associated with this article can be found in the online version.


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  1. Pucci A, Gagliardotto P, Zanini C. Histopathologic and clinical characterization of cardiac myxoma: a review of 53 cases from a single institution. Am Heart J (2000) 140:134.[CrossRef][Web of Science][Medline]
  2. Pessotto R, Santini F, Piccin C, Consolaro G, Faggian G, Mazzucco A. Cardiac myxoma of the tricuspid valve: description of a case and review of the literature. J Heart Valve Dis (1994) 3:344–6.[Medline]
  3. Chakfe N, Kretz JG, Valentin P, Geny B, Petit H, Popescu S, et al. Clinical presentation and treatment options for mitral valve myxoma. Ann Thorac Surg (1997) 64:872–7.[Abstract/Free Full Text]
  4. Gosse P, Herpin D, Roudaut R, Malergue MC, Longy M, Baudet E, et al. Myxoma of the mitral valve diagnosed by echocardiography. Am Heart J (1986) 111:803–5.[CrossRef][Web of Science][Medline]
  5. Chen MY, Wang JH, Chao SF, Hsu YH, Wu DC, Lai CP. Cardiac myxoma originating from the anterior mitral leaflet. Jpn Heart J (2003) 44:429–34.[CrossRef][Medline]

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This Article
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