A giant myxoma originating from the aortic valve causing severe left ventricular tract obstruction: a case report and literature review
© Prifti et al.; licensee BioMed Central. 2015
Received: 15 November 2014
Accepted: 6 April 2015
Published: 16 April 2015
The left ventricular localization of a myxoma is very rare, usually arising from the interventricular septum close to the left ventricular outflow tract, the mitral valve, the ventricular wall and extremely rarely the aortic valve.
A 13-year-old male was admitted due to dyspnea and angina. Transesophageal echocardiography revealed left ventricular outflow tract obstruction with a mean gradient of 58 mmHg, and a mobile mass measuring 65 × 25 mm originating from the ventricular surface of the aortic valve was identified. The patient underwent urgent surgical excision and aortic valve replacement. Histopathological examination of the mass confirmed the diagnosis of a myxoma.
In conclusion, a myxoma originating from the aortic valve remains a very rare localization. Total resection associated with aortic valve replacement seems to offer an excellent outcome.
Most cardiac myxomas are located in the left atrium, attached to the interatrial septum. The left ventricular localization of a myxoma is very rare, usually arising from the interventricular septum close to the left ventricular outflow tract [1-3], the mitral valve [4,5], the left ventricular wall  and extremely rarely the aortic valve . Here we report a case of an aortic valve myxoma undergoing successful resection.
Myxomas of the left ventricle are very rare. Recently, a thorough literature review demonstrated only 71 cases with a reported left ventricular myxoma usually originating from the interventricular septum . Myxomas of the cardiac valves are very unusual, especially those of the aortic valve. To our knowledge, this is the tenth reported case of an aortic valve myxoma. The first was described as a post-mortem finding . However, the patient in the present case is the youngest amongst them.
The shape, the extension, the site of attachment, the involvement of valve leaflets and the functional obstruction of the LV outflow tract could promptly and easily be assessed by echocardiography. Transesophageal echocardiography enables the detection of an aortic valve myxoma; however, histopathology remains the gold standard method of diagnosis. Most of the tumours were larger than 1 cm; however, the present case had the greatest mass dimensions amongst the reported cases with an aortic valve myxoma.
Clinical presentations of the reported cases and the present case
Kennedy et al. 
RCC and LCC
Watarida et al. 
1.1 × 1 cm
Ramsheyi et al. 
Okamoto et al. 
1 × 1 cm
Dyk et al. 
4 × 1 cm
Koyalakonda et al. 
1 × 1 cm
Kim et al. 
Shortness of breath
1.5 × 0.8 cm
Fernandez et al. 
1.5 × 0.7 cm
RCC and LCC
Javed et al. 
1.8 × 1.2 cm
Dyspnea and angina
60 × 22 mm
RCC and LCC
Differential diagnosis of an aortic valve myxoma includes vegetations, papillary fibroelastoma and Lambl’s excrescences. Microscopic and immunohistochemical characteristics allow the distinction between these entities. As we have observed, aortic valve myxomas are a potential source of emboli; therefore, surgical removal should be indicated as soon as the diagnosis is confirmed. Surgical excision should include not only the tumour but also the implantation site to minimize the risk of local recurrence. Tumour resection with conservation of the native valve should be intended, but sometimes due to a big tumour size and/or structural valve degeneration, replacement of the aortic valve may become necessary as in our case. These patients should be followed carefully due to a high probability of distal tumour growth at the site of previous embolization as well as local recurrence of the tumour.
In conclusion, a myxoma originating from the aortic valve remains a very rare left ventricular localization of such a tumour. Total resection associated with aortic valve replacement seems to offer an excellent outcome.
Written informed consent was obtained from the patient’s legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Natale E, Minardi G, Casali G, Pulignano G, Musumeci F. Left ventricular myxoma originating from the interventricular septum and obstructing the left ventricular outflow tract. Eur J Echocardiogr. 2008;9(1):84–5.PubMedGoogle Scholar
- Rao RV, Walsh S, Chan V, Stadnick E, Sohmer B, Veinot JP, et al. Unusual cause of an ejection murmur: myxoma in the left ventricular outflow tract. Can J Cardiol. 2013;29(12):1742.e13-5.View ArticlePubMedGoogle Scholar
- Kumar P, Garg A. Left ventricular myxoma in a child: a case report. Eur J Echocardiogr. 2011;12(3):E23.View ArticlePubMedGoogle Scholar
- Keeling IM, Oberwalder P, Schuchlenz H, Anelli-Monti M, Rigler B. Left ventricular outflow tract obstruction due to valve myxoma. Ann Thorac Surg. 2000;69:1590–1.View ArticlePubMedGoogle Scholar
- Choi BW, Ryu SJ, Chang BC, Choe KO. Myxoma attached to both atrial and ventricular sides of the mitral valve: report of a case and review of 31 cases of mitral myxoma. Int J Cardiovasc Imaging. 2001;17:411–6.View ArticlePubMedGoogle Scholar
- Abad C, Novoa J, Delgado A, Alonso A. Myxoma of the left ventricle. Tex Heart Inst J. 2014;41(4):395–400.View ArticlePubMed CentralPubMedGoogle Scholar
- Javed A, Zalawadiya S, Kovach J, Afonso L. Aortic valve myxoma at the extreme age: a review of literature. BMJ Case Rep. 2014;18:2014.Google Scholar
- Wold LE, Lie JT. Cardiac myxomas: a clinico-pathologic profile. Am J Pathol. 1980;101:219–33.PubMed CentralPubMedGoogle Scholar
- Watarida S, Katsuyama K, Yasuda R, Magara T, Onoe M, Nojima T, et al. Myxoma of the aortic valve. Ann Thorac Surg. 1997;63:234–6.View ArticlePubMedGoogle Scholar
- Ramsheyi A, Deleuze P, D’Attelis N, Bical O, Lefort JF. Aortic valve myxoma. J Card Surg. 1998;13:491–3.View ArticlePubMedGoogle Scholar
- Okamoto T, Doi H, Kazui T, Suzuki M, Koshima R, Yamashita T, et al. Aortic valve myxoma mimicking vegetation: report of a case. Surg Today. 2006;36:927–9.View ArticlePubMedGoogle Scholar
- Dyk W, Konka M. Images in cardiothoracic surgery: unusual complication of aortic valve grape-like myxoma. Ann Thorac Surg. 2009;88:1022.View ArticlePubMedGoogle Scholar
- Kennedy P, Parry AJ, Parums D, Pillai R. Myxoma of the aortic valve. Ann Thorac Surg. 1995;59:1221–3.View ArticlePubMedGoogle Scholar
- Fernandez AL, Vega M, El-Diasty MM, Suárez JM. Myxoma of the aortic valve. Interact Cardiovasc Thorac Surg. 2012;15:560–2.View ArticlePubMed CentralPubMedGoogle Scholar
- Koyalakonda SP, Mediratta NK, Ball J, Royle M. A rare case of aortic valve myxoma: an unusual cause of embolic stroke. Cardiology. 2011;118:101–3.View ArticlePubMedGoogle Scholar
- Kim HY, Kwon SU, Jang WI, Kim HS, Kim JS, Lee HS, et al. A rare case of aortic valve myxoma: easy to confuse with papillary fibroelastoma. Korean Circ J. 2012;42:281–3.View ArticlePubMed CentralPubMedGoogle Scholar
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