Ann Thorac Surg 2000;69:1590-1591
© 2000 The Society of Thoracic Surgeons
Case reports
Left ventricular outflow tract obstruction due to valve myxoma
Ingeborg Keeling, MDa,
Peter Oberwalder, MDa,
Helmut Schuchlenz, MDb,
Michael Anelli-Monti, MDa,
Bruno Rigler, MDa
a Department of Surgery, Karl Franzens University Graz, Graz, Austria
b Department of Medicine, Karl Franzens University of Graz, Graz, Austria
Address reprint requests to Dr Keeling, Division of Cardiac Surgery, Department of Surgery, Karl Franzens University of Graz, Auenbruggerplatz 1, 8010 Graz, Austria
e-mail: ingeborg.keeling{at}kfunigraz.ac.at
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Abstract
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A life-threatening left ventricular outflow tract obstruction developed in a 26-year-old man because of a very uncommon myxoma emerging from the ventricular side of the mitral valve. Immediate surgical treatment was indicated because of high-risk factors reported herein. After transaortic resection of a single myxoma, the progress of this patient has been excellent, especially as a familial myxoma could be excluded.
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Introduction
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Intracardiac myxoma is the most common tumor of the heart, with an estimated incidence of 0.5 per million population per year [1]. The symptoms vary greatly, depending on the size and localization of the tumor. Up to 80% of myxomas are localized in the left atrium, whereas myxomas emerging from valve tissue are rarely found [2, 3]. A case of a mitral valve myxoma is discussed.
A 26-year-old man was admitted after a cardiac myxoma had been detected during preventive medical checkup. The patient was completely asymptomatic. Physical examination showed a variable diastolic murmur that decreased in the supine position. Laboratory investigations showed normal levels with respect to coagulation variables, antinuclear factors, and cardiolipin antibodies. Electrocardiogram showed normal sinus rhythm at 70 beats/minute, indifference type, and a negative T wave in lead III. The result of a chest roentgenogram study was normal. Transesophageal echocardiography revealed an intracardiac tumor at the ventricular-sided basis of the anterior mitral valve leaflet resulting in a systolic prolapse through the aortic valve and a 50% obstruction of the left ventricular outflow tract (Fig 1). Magnetic resonance imaging of the neurocranium excluded asymptomatic microembolisms of the brain. A familial occurrence of the disease was excluded in first-grade relatives of the patient.
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Fig 1. (A, B) Preoperative transesophageal echocardiograms of a mitral valve myxoma at the ventricular-sided basis of the anterior mitral valve leaflet resulting in obstruction of the left ventricular outflow tract.
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A transaortic resection of a mitral valve myxoma, emerging from the anterior mitral valve leaflet, was performed under extracorporeal circulation and moderate hypothermia by way of a median sternotomy. Intraoperatively, after dissection of the left atrium and transverse aortotomy, the huge broad-based myxoma, 4 cm in diameter, became visible, as it nearly reached the supravalvular area of the ascending aorta. Transaortic inspection revealed that the relatively soft tumor arose with a broad basis from parts of the anterior papillary muscle and from the backside of the anterior mitral valve leaflet. The myxoma of this patient arose from the ventricular side of the mitral valve leaflet, which is extremely rare, and not as usual from the left atrial wall. Macroscopically, the gelatinous tissue appeared as yellow-brownish to yellow-greenish myxoid stroma. Tumor resection was followed by inspection of both atria for more myxoma tissue. Histopathologic examination revealed proliferations of capillaries, blood extravasations, and disseminated fibrin depositions. These findings were consistent with the diagnosis of a myxoma. Intraoperative echocardiography showed normal mitral valve function and a hemodynamically insignificant aortic insufficiency grade I. The postoperative course of the patient was uneventful, and after 13 months of follow-up he was free from evidence of recurrence of disease.
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Comment
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We report on a case of an asymptomatic patient with a myxoma that originated from the left-ventricular-sided aspect of the mitral valve and that required immediate resection. Furthermore, the myxoma required immediate resection as with future tumor growth this myxoma threatened valve destruction and a left ventricular outflow tract obstruction. The patient was not only at an increased risk for sudden cardiac death [4], but also at a higher risk of embolization, which occurs in 30% to 43% of these patients [2, 3]. This higher risk of embolization can be due to the motion of the valve leaflets or from the high pressure within the left ventricle during systole [3]. Cardiac myxomas seem to recur more often in young men, in patients with multifocal origins, and in up to 40% of patients who have a family history of the tumor [2, 5]. The prognosis for patients with solitary myxomas after surgical resection is excellent as evidenced by the low hospital mortality rate [2]. Late recurrences have been reported to occur in 0.4% to 5% of surgically treated patients 3 months to 22 years after operation [2]. In those rare instances in which the tumor arises from an atrioventricular valve, the valve occasionally requires valvuloplasty or even replacement. We decided to remove the tumor through the aortic root to avoid the higher risk of intraoperative embolization. This approach facilitated the exposure of the left-ventricular-sided aspect of the mitral valve apparatus. Despite the broad basis by which the myxoma originated from the mitral valve, the valve could be completely preserved. The transesophageal echocardiographic follow-up at 12 months revealed trivial mitral valve dysfunction without evidence of tumor recurrence. The course of our patient indicates that the many risks inherent in this disease may be kept low by aiming at early diagnosis with immediate surgical treatment. As this disease may mimic a great variety of other cardiac diseases, its possible presence should always be considered, as relatively low-risk cardiac surgical treatment can be performed with an excellent postoperative prognosis for most patients.
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References
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Chakfe N., Kretz J.G., Valentin P., et al. Clinical presentation and treatment options for mitral valve myxoma. Ann Thorac Surg 1997;64:872-877.[Abstract/Free Full Text]
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Vassiliadis N., Vassiliadis K., Karkavelas G. Sudden death due to cardiac myxoma. Med Sci Law 1997;37:76-78.[Medline]
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Accepted for publication September 29, 1999.
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Abstract
Introduction
