This article has Open Peer Review reports available.
Giant biatrial myxoma nearly obstructing the orifice of the inferior vena cava
© Choi et al.; licensee BioMed Central Ltd. 2013
Received: 24 January 2013
Accepted: 3 June 2013
Published: 10 June 2013
Cardiac myxomas are the most common type of benign cardiac tumors and most of them occur in the left atrium but the biatrial myxoma is uncommon. We present a rare case of giant biatrial myxoma nearly obstructing the orifice of the inferior vena cava. A 58-year old woman presented with exertional dyspnea and intermittent chest discomfort. The non-pedunculated tumor involved most of the interatrial septum and extended from the orifice of the inferior vena cava to the displaced mitral annulus and the lower left pulmonary vein. The resected specimen weighed 76 gram and measured 80 × 40 × 30 mm. She did not complain of dyspnea or show any sign of recurrence by echocardiography during the 2-year follow-up period.
KeywordsHeart neoplasm Myxoma Biatrial
Cardiac myxoma is the most common type of primary cardiac neoplasm and accounts for 30% to 50% of all primary tumors of the heart with an annual incidence of 0.5 per million populations [1, 2]. Over 70% of all cardiac myxomas originate from the left and 18% from the right atrium. Biatrial myxomas account for less than 2.5% of all cardiac myxomas [3–5]. We present biatrial myxoma, which occupies both atria without pedunculating mass.
Cardiac myxoma is usually polypoid and pedunculated lesions, arising from endocardium . In contrast, other cardiac tumors, such as, lipoma and rhabdomyoma are typically not pedunculated. The majority of cardiac myxomas manifest as a gelatinous, smooth, round mass with a glistening surface . Imperio et al. reported that biatrial myxomas had two stalks arising from the same area of the interatrial septum and growing in opposite directions toward the right and left atria . Irani et al. reported that there was no evidence of extension across the interatrial septum, thereby favoring contemporaneous origin of two separate tumors , whereas Ha et al. described two distinct types of myxoma, that is, a round type, characterized by a solid texture and round shape with a non-mobile surface (52%); and a polypoid type, characterized by a soft texture and an irregular shape with a mobile surface (48%) . Right-sided myxomas are rare, but typically attach on a broad base and are more likely to be calcified than left-sided lesions [8, 9]. In our case, the biatrial myxoma was not pedunculated, was attached on a broad base, and its surface was hard and solid with calcifications. We speculated that the myxoma grew to nearly obstruct the orifice of the IVC and that its root grow predominantly toward the left-side. This speculation proved consistent with surgical findings, as two thirds of the left-side of the specimen was attached to the base of the left atrium. The symptom of right side heart failure might have been erroneous because the IVC was not entirely occluded. Budd-Chiari syndrome can rarely develop by hepatic inferior vena cava and portal vein thrombosis, caused by a large myxoma of the right atrium . The incidence of biatrial myxoma is rare but cardiopulmonary problems and neurologic complications can be prevented by surgical resection. Although the rate of recurrence is less than 5% after complete resection,  patients should be monitored routinely by cardiac echocardiography.
The Biatrial myxoma is uncommon and could be considered in the differential diagnosis of other cardiac tumors with several imaging modalities. Prognosis is excellent after surgical resection.
Written and informed consent obtained for publication of this case report and accompanying images. A copy of the written consent is available for review by editor-in- chief of this journal.
- McManus B: Primary tumors of the heart. Braunwald’s heart disease, a textbook of cardiovascular medicine. Edited by: Bonow RO, Mann DL, Zipes DP. 2012, Philadelphia: Elsevier Saunders, 1638-1650. 9View ArticleGoogle Scholar
- Burke A, Virmani R: Atlas of tumor pathology. Tumors of the heart and great vessels. Atlas of tumor pathology. Tumors of the heart and great vessels. Edited by: Rosa J. 1996, Washington: Armed Forces Institute of Pathology, 21-47. 3Google Scholar
- Irani AD, Estrera AL, Buja LM: Biatrial myxoma: a case report and review of the literature. J Card Surg. 2008, 23: 385-390. 10.1111/j.1540-8191.2007.00545.x.View ArticlePubMedGoogle Scholar
- Peachell JL, Mullen JC, Bentley MJ: Biatrial myxoma: a rare cardiac tumor. Ann Thorac Surg. 1998, 65: 1768-1769. 10.1016/S0003-4975(98)00206-9.View ArticlePubMedGoogle Scholar
- Ahmed AK, Rajendran R, Shaukat N: A rare case of atrial myxoma with biatrial extension. Int J Cardiol. 2008, 127: e50-e51. 10.1016/j.ijcard.2007.04.004.View ArticlePubMedGoogle Scholar
- Imperio J, Summers D, Krasnow N: The distribution patterns of biatrial myxomas. Ann Thorac Surg. 1980, 29: 469-473. 10.1016/S0003-4975(10)61682-7.View ArticlePubMedGoogle Scholar
- Ha JW, Kang WC, Chung N: Echocardiographic and morphologic characteristics of left atrial myxoma and their relation to systemic embolism. Am J Cardiol. 1999, 83: 1579-1582. 10.1016/S0002-9149(99)00156-3.View ArticlePubMedGoogle Scholar
- Murayama H, Tamaki S, Kato N: Right atrial myxoma associated with atrial septal defect: a case report and review of the literature. Ann Thorac Cardiovasc Surg. 2001, 7: 166-169.PubMedGoogle Scholar
- Chamsi-Pasha MA, Anwar AM, Nosir YF: Right atrial myxoma associated with an atrial septal defect by real-time three-dimensional echocardiography. Eur J Echocardiogr. 2009, 10: 362-364.View ArticlePubMedGoogle Scholar
- Anagnostopoulos GK, Margantinis G, Kostopoulos P: Budd-Chiari syndrome and portal vein thrombosis due to right atrial myxoma. Ann Thorac Surg. 2004, 78: 333-334. 10.1016/S0003-4975(03)01265-7.View ArticlePubMedGoogle Scholar
- Castells E, Ferran V, Octavio de Toledo MC: Cardiac myxomas: surgical management, long term results and recurrence. J Cardiovasc Surgery. 1993, 34: 49-53.Google Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.