Tricuspid valve myxoma in a patient with congestive heart failure
© Vizzardi et al; licensee BioMed Central Ltd. 2010
Received: 4 November 2009
Accepted: 12 January 2010
Published: 12 January 2010
Myxomas are the most frequent benign primary cardiac tumours (50% of benign heart tumours). This kind of tumour is most likely to be localized in the left atrium, followed by the right atrium, right ventricle and left ventricle. Quite exceptional is the presence of a myxoma originating from the tricuspid valve or from the Eustachian valve. We describe the case of a woman with moderate dyspnoea of unknown origin and the presence of tricuspid myxoma who underwent tricuspid valve curettage.
Myxomas are the most frequent benign primary cardiac tumours (50% of benign heart tumours). They are most commonly detected in people between 30 and 60 years of age, although published findings report congenital myxomas, tumours developed during childhood and even in extremely old patients. This kind of tumour is most likely to be localized in the left atrium (75%), followed by the right atrium (18%), right ventricle (4%) and left ventricle (3%) [1, 2]. Quite exceptional is the presence of a myxoma originating from the tricuspid valve [3, 4] or from the Eustachian valve .
Surgical removal with tricuspid valve curettage was performed to improve the symptoms of congestive heart failure and prevent pulmonary embolism. Histological examination of the mass confirmed the diagnosis of myxoma and its benign nature. The postoperative course was uneventful and follow-up examination showed complete normalization of the Doppler echocardiographic parameters and the absence of clinical symptoms (i.e. dyspnoea).
Myxomas are rarely localized in the right heart originating from the right atrium or from the tricuspid valve; they usually present symptoms related to low cardiac output or to pulmonary arterial hypertension with hepatomegaly, oedema, ascites and cyanosis. This clinical picture varies, however, with the position of the tumour. The literature reports patients who were symptomatic for syncopes and dyspnoea [1, 2] or embolic manifestations that usually involved the lungs [6, 7]. In the case of friable polypoid myxomas there may also be multiple pulmonary embolism , frequently mistaken for a septic embolus, which can lead to severe pulmonary hypertension and sometimes also to aneurisms of the pulmonary arteries . Lastly, in the case of a right-to-left shunt at an atrial level, paradoxical embolism of the brain and kidneys and aortic bifurcation may occur . Resection of the tumour constitutes the definitive treatment for cardiac myxomas, using different techniques based on the location of the tumour in order to control embolization. In the case of tricuspid valve myxomas, tumour excision, tricuspid valvuloplasty or valve replacement may also be necessary. Surgery mortality is around 1% and relapses are possible (1-5% of all cases). Recurrences are usually due to metastatic dissemination or incomplete resection of the lesion and arise within 48 months of the operation [11–13]. Our clinical case highlights once more the absence and the non-specificity of the clinical manifestation of cardiac myxomas, especially when located in the right heart. Furthermore, as well established in the literature, this case stresses the importance and reliability of two-dimensional echocardiograpy for diagnosis of the tumour mass thanks to direct visualization of the tricuspid leaflets and normalization of the haemodynamic parameters.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by The Editor-in-Chief of the journal
- Hall RJ, Denton AC, McAllister HA, Frazier HO, O'Rourke R: Neoplastic heart disease. Heart. Edited by: Alexander RW, Fuster V, O'Rourke R, Hurst. Mc Graw-Hill, 2526-2523. 11Google Scholar
- Sabatine M, Colucci W, Schoen F: Primary tumors of the heart. Heart Disease. Edited by: Braunwald E, Libby P, Zipes D, Bonow RO Braunwald. Elsevier Masson, 1745-1750. 7Google Scholar
- Cordier G, Kieny R, Eisenmann B, Grison D, Hessel F, Ameur C, Chemouny JP, Legutke D, Laval G: Echocardiography of an operated case of myxoma of the tricuspid valve. Arch Mal Coeur Vaiss. 1981, 74 (6): 747-54.Google Scholar
- Kuroda H, Nitta K, Ashida Y, et al: Right Atrial Myxoma Originating from the Tricuspid valve. J Thora Cardiovasc Surg. 1995, 109: 1249-1250. 10.1016/S0022-5223(95)70211-3.View ArticleGoogle Scholar
- Teoh KH, Mulji A, Tomlinson CW, et al: Right Atrial Myxoma originating from the Eustachian valve. Can J Cardiol. 1993, 9: 441-443.PubMedGoogle Scholar
- Keenan DJM, Morton P, O'Kane H: Right atrial myxoma and pulmonary embolism. Br Heart J. 1982, 48: 510-2. 10.1136/hrt.48.5.510.PubMed CentralView ArticlePubMedGoogle Scholar
- Parsons AM, Detterdach FC: Multifocal right atrial myxoma and pulmonary embolism. Ann Thorac Surg. 2003, 75: 1323-1324. 10.1016/S0003-4975(02)04563-0.View ArticlePubMedGoogle Scholar
- Idir M, Osvel N, Guibaud JP, Labouvrie E, Roudat R: Fragmentation of a right atrial myxoma presenting as a pulmonary embolism. J Am Soc Echocardiogr. 2000, 13 (1): 61-3.View ArticlePubMedGoogle Scholar
- Heck HA, Gross CM, Houghton JL: Long-term severe pulmonary hypertension associated with right atrial myxoma. Chest. 1992, 102: 301-303. 10.1378/chest.102.1.301.View ArticlePubMedGoogle Scholar
- Murayama H, Tamaki S, Okada M, et al: Right Atrial Myxoma Associated with Atrial Septal Defect: A case report and review of the literature. Ann Thorac Cardiovasc Surg. 2001, 7 (3): 166-169.PubMedGoogle Scholar
- Shaff HV, Mullany CJ: Surgery for Cardiac Myxomas. Semin Thorac Cardiovasc Surg. 2000, 12: 77-88.View ArticleGoogle Scholar
- Asmar B, Acker M, Couetil JP, Penther PH, Carpentier A: Tricuspid valve myxoma: a rare indication for tricuspid valve repair. Ann Thorac Surg. 1991, 52 (6): 1315-6. 10.1016/0003-4975(91)90020-Q.View ArticlePubMedGoogle Scholar
- Almdahl SM, Myrmel T, Gjestvang FT: Surgical treatment of a tricuspid valve myxoma. Tidsskr Nor Laegeforen. 1996, 116 (18): 2175.PubMedGoogle 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.