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Ann Thorac Surg 1997;64:872-877
© 1997 The Society of Thoracic Surgeons

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Current review

Clinical Presentation and Treatment Options for Mitral Valve Myxoma

Departments of Cardiovascular Surgery and Thoracic Surgery, Les Hopitaux Universitaires de Strasbourg, Strasbourg, France

	Abstract

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 
Myxomas rarely are located on the mitral valve. We report the case of a 44-year-old man operated on successfully with mitral valve conservation, which is the 21st case of mitral valve myxoma reported in the western literature. Among the cases reported in the literature, the diagnosis was made at the time of autopsy in 6 cases, with premortem heart failure in 1 case. A clinical diagnosis was made in 15 cases on the basis of the following symptoms: peripheral embolism in 9 cases, cardiac signs in 4 cases, and constitutional manifestations in 2 cases. The clinical presentation of mitral valve myxoma differs slightly from that of other cardiac myxomas in that it has a lower incidence of constitutional manifestations. The gold standard for noninvasive diagnosis and localization of cardiac myxomas is transesophageal echocardiography. It allows for the detection of early, small valvular tumors and may help to characterize better their location and echostructure, facilitating the choice of an optimal surgical approach through preoperative study of the integrity and mobility of the valve. Conservative operative treatment by resection of the area of implantation of the myxoma followed by suture repair of the valve and annuloplasty may be recommended as the most appropriate treatment option for this rare condition.

	Introduction

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 
Seventy-five percent of primary cardiac tumors are benign, and myxomas are the most frequent variety [1]. Myxomas usually develop on the interatrial septum, and occur in the left atrium in more than 75% of cases. Myxomas rarely may be encountered at other sites within the cardiac cavities, such as the ventricles, and exceptionally may develop on a cardiac valve. The most common presenting symptoms of cardiac myxomas are obstructive symptoms, such as mitral stenosis, systemic embolism, and constitutional symptoms [2]. Recently, the diagnosis of mitral myxomas has been improved greatly by the use of transesophageal echocardiography. This investigation also allows preoperative planning of the most appropriate treatment, namely valve replacement or valvuloplasty. We report the 21st published case of mitral valve myxoma and review the world literature on this rare and harmful cardiac disease, evaluating particularly its clinical presentation and treatment options.

	Case Report

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 
A 44-year-old man was referred to our institution 2 hours after the acute onset of pain in both legs. The patient was previously healthy and without a significant medical history. Physical examination revealed a normally developed man with stable vital signs. The heart rate was 96 beats/min and regular. No abnormal heart sound was heard. Both femoral and leg pulses were absent. The legs were cold and discolored below the knees, with normal skin sensitivity. The diagnosis of embolism of the aortic bifurcation was made and emergency surgical embolectomy was planned.

The electrocardiogram showed a normal sinus rhythm, and the chest roentgenogram was normal. Digital subtraction angiography disclosed a rounded intraluminal mass at the level of the aortic bifurcation. The right iliac artery was occluded with contrast reinjection of the common femoral artery. The origin of the left iliac artery was partially occluded. No angiographic sign of atherosclerosis was observed in the aorta or in the visceral and peripheral arteries. There was no sign of a distal embolism (Fig 1).

View larger version (105K): [in this window] [in a new window]   Fig 1. . Digital subtraction angiogram performed through a humeral catheterization shows a rounded intraluminal mass at the level of the aortic bifurcation. The right iliac artery is occluded and the distal runoff is reinjected at the level of the common femoral artery. The origin of the left iliac artery is partially occluded. There is no angiographic sign of atherosclerosis.

View larger version (133K): [in this window] [in a new window]   Fig 2. . Macroscopic view of the operative specimen. It is round, firm, and encapsulated.

View larger version (89K): [in this window] [in a new window]   Fig 3. . Transthoracic echocardiography showing a regular mass measuring 1.4 x 1.7 cm, located on the atrial side of the anterior mitral leaflet.

View larger version (76K): [in this window] [in a new window]   Fig 4. . Transesophageal echocardiography providing an improved and more precise description of the mass than transthoracic echocardiography. The mass measured 2.2 x 1.8 cm, prolapsed into the left ventricle (VG) during diastole, and returned to the left atrium (OG) during systole.

View larger version (99K): [in this window] [in a new window]   Fig 5. . Magnetic resonance imaging. Note the rounded mass attached to the anterior mitral valve leaflet.

View larger version (82K): [in this window] [in a new window]   Fig 6. . (A) Operative view. Note the rounded mass attached to the anterior leaflet by a short stalk. (B) Macroscopic view of the mass attached to the anterior mitral valve leaflet.

The patient's postoperative recovery was uneventful. No mitral insufficiency was noted by transthoracic echocardiography. In May 1996, transthoracic echocardiography demonstrated normal function of the mitral valve without any sign of tumor recurrence.

	Comment

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 
We reviewed the literature on mitral valve myxoma to study clinical presentations and treatment options for this rare condition. The present case appears to be the 21st case of mitral valve myxoma reported in the literature (Table 1). Mitral valve myxoma affected men and women with the same frequency (10 men and 11 women). The mean patient age was 41.8 ± 20.5 years (range, 5 to 83 years).

Embolization is a frequent complication of cardiac valve myxomas, and it was described in 9 cases. The site of the embolism was a cerebral artery in 8 cases and the aortic bifurcation in our case. Embolizations of fragments of cardiac myxomas are not uncommon, and are expected in about 40% of patients [2, 20]. Embolization has been reported in 45% to 60% of left-sided and in 8% to 10% of right-sided cardiac myxomas [21]. Most embolic events related to cardiac myxomas involve the cerebral arteries [21–23]. Emboli also may involve other sites, such as the renal, coronary, or lower limb arteries [22, 24–28]. Embolism of the aortic bifurcation has been described infrequently [20, 22, 29–31], mainly because it requires embolization of a large tumor mass, exceeding 2 cm in diameter. In our case, a direct surgical approach to the aortic bifurcation was necessary to retrieve the embolized tumor, because its size and firmness made it resistant to fragmentation by the Fogarty embolectomy catheter.

There are two types of cardiac myxomas: the polypoid type and the round type. The polypoid type of tumor is semitransparent and gelatinous, and is associated with a higher incidence of embolization than the round type [32]. It may cause multiple emboli because the tumor is fragile and may be fragmented further in the arteries. This tumor material may be undetected if it is associated with a thrombus, and it sometimes shows malignant behavior with infiltration and destruction of the arterial wall of pulmonary, cerebral, coronary, and other systemic vessels giving rise to myxomatous pseudoaneurysms [23]. Therefore, any embolectomy material or specimens should be analyzed histologically to exclude an occult myxoma [23]. In contrast, emboli related to the round type of myxoma are rare. Their retrieval may be impossible by embolectomy catheter, and a direct approach to the site of embolism may be required as described in our case. Because of its morphology, the tumoral origin of the embolus is recognized easily and multiple emboli are rare. Unfortunately, the macroscopic characteristics of the mitral valve myxomas were not indicated precisely in the articles we reviewed. However, we emphasize that mitral valve myxomas present a major risk of embolization because of the high mobility of their area of implantation (ie, the mitral valve) and the high pressure inside the left ventricle.

Cardiac signs were reported in 4 cases; the myxomas were a fortuitous finding of an abnormal cardiac murmur in 1 case, a complaint of palpitations in 1 case, and a complaint of dyspnea in 2 cases. Constitutional manifestations were reported in 2 cases: arthralgias and fever in the first case and asthenia in the second case. The literature shows that about 60% of patients with cardiac myxomas had congestive heart failure and 65% had constitutional manifestations [2, 20]. The clinical presentation of mitral valve myxoma was slightly different from that of other cardiac myxomas, because constitutional manifestations were found in only 2 cases. This incidence may be explained by the fact that myxomas in the mitral valve location produce early symptoms, mainly embolization, at a relatively smaller size than myxomas that develop in cardiac cavities. Heart failure was found with a similar frequency, in most cases diagnosed at autopsy; the cause of death probably was related to cardiac manifestations.

Any clinical presentation suggesting a cardiac tumor should lead to an early diagnostic procedure because of the risk of embolization. Echocardiography is considered to be the gold standard for noninvasive diagnosis and localization of cardiac myxomas. Most myxomas arise from the interatrial septum in the vicinity of the fossa ovalis, and they occur in the left atrium in more than 75% of cases and in the right atrium in about 20% of cases [33]. Myxomas on rare occasions may be encountered in other locations within the cardiac chambers, such as the right [14] and left ventricles [25, 30, 34], and they exceptionally may arise from a cardiac valve. The tricuspid valve is the most frequent location of these valvular myxomas [35], followed by the mitral valve, with 16 reported cases [3–19], and the pulmonary and aortic valves [36, 37]. Mitral valve myxomas were located mainly on the atrial side of the valve, with an equivalent distribution between the anterior and posterior leaflets. The size of the myxomas ranged between 0.5 and 3 cm in diameter. Transthoracic echocardiography may miss a tumor measuring less than 5 mm in diameter, suggesting that transesophageal echocardiography is necessary to perform a precise cardiac examination when a cardiac tumor is suspected. Transesophageal echocardiography allows the early detection of small valvular tumors and may help to characterize better the location and echostructure of these lesions. In addition, transesophageal echocardiography can guide the surgical approach by revealing the integrity and mobility of the valve before operation.

Thirteen cases of mitral valve myxoma were treated surgically and 6 cases were found at autopsy. One patient died of tumor embolization during operation, and information was not available in 1 case. The preferential surgical treatment used in 6 cases was removal of the tumor without valve excision. The follow-up data for this option were given in only 3 cases, and no recurrence was observed after 1.5, 6, and 6 months, respectively. In 2 cases, the treatment combined excision of the mass with mitral valve replacement. In 2 cases, the myxoma was removed with its insertion area on the mitral valve without valvuloplasty. No further information was provided in 1 case, but the follow-up was uneventful after 11 months in the second case. In our case, we believed, like two other authors, that it was preferable to extend the resection through the leaflet around the tumor, followed by direct suture repair of the leaflet and annuloplasty, to avoid local recurrence from infiltrating myxoid stroma. Follow-up was not available in the other 2 cases. However, conservative leaflet resection was successful in our patient, with no recurrence or mitral insufficiency 2 years after operation. It may be recommended as an appropriate treatment option for this rare lesion.

	Acknowledgments

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 
We are indebted to Henri Gallin for the illustrations.

	Footnotes

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 
Address reprint requests to Dr Chakfé, Department of Cardiovascular Surgery, Les Hopitaux Universitaires de Strasbourg, BP no. 426, 67091, Strasbourg Cedex, France.

	References

Top Footnotes Abstract Introduction Case Report Comment Acknowledgments References

 

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Jean-Georges Kretz Sorin Popescu Gilbert Massard Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Chakfé, N. Articles by Massard, G. Search for Related Content PubMed PubMed Citation Articles by Chakfé, N. Articles by Massard, G.