Accepted Manuscript Neovascularization and associated fistula formation in a left atrial myxoma: Evaluation with multidetector computed tomography Jon Suh, MD, PhD Heon Lee, MD, PhD Keun Her, MD, PhD Eun Suk Koh, MD, PhD PII:

S0828-282X(14)00221-9

DOI:

10.1016/j.cjca.2014.03.035

Reference:

CJCA 1167

To appear in:

Canadian Journal of Cardiology

Received Date: 18 March 2014 Revised Date:

25 March 2014

Accepted Date: 26 March 2014

Please cite this article as: Suh J, Lee H, Her K, Koh ES, Neovascularization and associated fistula formation in a left atrial myxoma: Evaluation with multidetector computed tomography, Canadian Journal of Cardiology (2014), doi: 10.1016/j.cjca.2014.03.035. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Neovascularization and associated fistula formation in a left atrial myxoma:

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Evaluation with multidetector computed tomography

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Jon Suh MD, PhDa, Heon Lee MD, PhDb, Keun Her MD, PhDc, Eun Suk Koh MD, PhDd Department of aCardiology, bRadiology, cCardiovascular surgery, and dPathology

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Soonchunhyang University Hospital Bucheon,

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Bucheon, Republic of Korea

Heon Lee MD, PhD.

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Reprint request to

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Department of Radiology

Soonchunhyang University Hospital Bucheon 1174 Jung-Dong, Wonmi-Gu, Bucheon, 420-767 Republic of Korea TEL : 82-32-621-5851 Fax : 82-32-621-5874 Email : [email protected] The authors have no conflict of interest to declare. This work was supported by Soonchunhyang University Research Fund.

ACCEPTED MANUSCRIPT Abstract Although angiographically detectable neovascularity is being reported with increasing frequency in patients with cardiac myxoma, associated coronary fistula to the cardiac

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chamber has not been described. We report herein a 62-year-old woman in whom cardiac CT enabled the noninvasive diagnosis of a left atrial myxoma with neovascularization arising from the left circumflex artery and the formation of an unusual fistula into the left atrial

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cavity with concomitant evaluation of the coronary arteries. Careful suture ligation of a supplying coronary branch in the atrial septum was performed during tumor excision to

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prevent the development of an intra-atrial steal.

Keywords: Left atrial myxoma; Neovascularization; Multi-slice computed tomography;

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Vascular fistula

ACCEPTED MANUSCRIPT Brief summary We report a patient in whom cardiac CT enabled the noninvasive diagnosis of a left atrial myxoma with neovascularization and the formation of an unusual fistula into the atrial cavity

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with concomitant evaluation of the coronary arteries. Careful suture ligation of a coronary branch was performed during tumor excision to prevent the development of an intra-atrial

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steal

ACCEPTED MANUSCRIPT Introduction Primary tumors of the heart are quite rare, having been reported in approximately 0.03% of autopsy series. Of these, 80% are benign, and about half of benign tumors are myxomas (1).

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Although they are being reported with increasing frequency, tumor vascularity has been thought to be uncommon in cardiac myxomas (2). Furthermore, to the best of our knowledge, coronary to atrial fistula formation through the tumor has not been described in

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the literature. We report herein the case of a patient with a atrial myxoma with

fistula into the left atrial cavity.

Case report

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neovascularization arising from the left circumflex artery and the formation of an unusual

A 62-year-old female was hospitalized because of epigastric pain. She was referred for

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cardiac CT after abdominal CT had demonstrated an incidental hypodense mass in the left atrium. Cardiac CT showed no evidence of coronary artery disease (Figure 1A), but revealed

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a 2.8 x 2.4 cm lobulated mass in left atrium arising from the inter-atrial septum (Figure 1B, video 1). Cardiac CT also depicted intratumoral neovasculariztion with clusters of tortuous

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vascular channels, supplied from the atrial branch of the left circumflex artery (Figure 1C). A focal vascular opening into the left atrial cavity was suspected on the inferior surface of the mass, suggesting the drainage site of a coronary artery-left atrial fistula (Figure 1D), which was confirmed in the pathologic specimen (Figure 1E).

Discussion According to previous studies, tumor vascularity is thought to be rare in cardiac myxomas

ACCEPTED MANUSCRIPT since these tumors are composed of bradytrophic tissue (2, 3). However, angiographically detectable tumor vascularity is being reported with increasing frequency during preoperative coronary angiography performed to exclude coronary artery disease (2, 4).

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Nevertheless, tumor vascularity-associated fistula formation to the left atrium, as in the present case, has not been described in patients with cardiac myxomas but is usually associated with organized thrombi in patients with mitral stenosis (5). Although both

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coronary angiography and echocardiography have been recommended and widely used to evaluate cardiac myxomas (3, 4), cardiac CT, in this case, was useful for the diagnosis and

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evaluation of atrial myxoma, providing both angiographic visualization of the coronary arteries and tumor vasculature, and echocardiographic description of its size and location. In this patient, additional suture ligation was performed to make sure of complete tying off the supplying branch in the atrial septum during the careful patch closure of the septal defect.

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In conclusion, we report the case of a highly vascularized left atrial myxoma with the formation of an unusual fistula into left atrial cavity. Thus, additional suture ligation of a

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supplying coronary branch was performed during the patch closure of the septal defect after tumor excision. This case also shows the utility of cardiac CT for noninvasive diagnosis of

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complex intracardiac pathology with concomitant evaluation of the coronary arteries.

ACCEPTED MANUSCRIPT References

1.

Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996;77:107

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Reynen K, Kockeritz U, Taha M, Strasser RH. Neovascularization in left atrial myxoma. Z Kardiol 2004;93:69-71 Umeda Y, Matsuno Y, Imaizumi M, Mori Y, Iwata H, Takiya H. Right atrial myxoma with

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tumor vascularity originated from the left and right coronary arteries. Int J Cardiol 2009;131:e137-139 4.

Van Cleemput J, Daenen W, De Geest H. Coronary angiography in cardiac myxomas: Findings in 19 consecutive cases and review of the literature. Cathet Cardiovasc Diagn Sakamoto I, Hayashi K, Matsunaga N, Ogawa Y, Matsuoka Y, Okimoto T et al. Coronary angiographic finding of thrombus in the left atrial appendage. Acta Radiol 1996;37:749-

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1993;29:217-220

ACCEPTED MANUSCRIPT Figure legend Figure 1. A. Medial axial image of the coronary arteries showed no evidence of coronary artery disease. Note motion artifact (*) with an irregular heart rhythm ranging from 52 to 66

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beats per minute. B. Axial images revealed a lobulated mass (arrow) in left atrium arising from the interatrial septum and measuring 2.8 x 2.4 cm. C. MIP and volume-rendering images demonstrated marked intratumoral neovascularity with clusters of tortuous vascular

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channels supplied from the atrial branch of the left circumflex artery (arrows). D. A focal opening of the vascular channels into the left atrial cavity (arrows) was suspected on the

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tumor surface, suggesting the drainage site of a coronary artery-left atrial fistula. Figure 2. Gross specimen of left atrial myxoma. Note a prominent intra-tumoral vascular proliferation (arrows), intravascular thrombus and hemorrhage (*), and focal vascular

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opening on the surface of the tumor (open arrows).

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Video legend Video 1. Three-dimensional volume-rendered CT image demonstrates a highly

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vascularized left atrial myxoma attached on atrial septum, without evidence of wall

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motion abnormalities of heart chambers.

Neovascularization and associated fistula formation in a left atrial myxoma: evaluation with multidetector computed tomography.

Although angiographically detectable neovascularity is being reported with increasing frequency in patients with cardiac myxoma, associated coronary f...
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