Asian Cardiovasc Thorac Ann 2002;10:111-114
© 2002 Asia Publishing EXchange Pte Ltd
Clinical Features and Surgical Treatment of Cardiac Myxoma: Report of 18 Cases
Kadir Durgut, MD,
Niyazi Görmüs, MD,
Mehmet Ozulku, MD,
Ufuk Özergin, MD,
Cevat Özpinar, MD
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Department of Cardiovascular Surgery University of Selcuk School of Medicine Konya, Turkey
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Kadir Durgut, MD Tel: 90 332 323 2600 Fax: 90 332 323 2643 email: kdurgut{at}selcuk.edu.tr Department of Cardiovascular Surgery, University of Selcuk School of Medicine, Akyokus, Meram, Konya 42080, Turkey.
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ABSTRACT
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Myxomas are the most common benign primary cardiac tumors. As many patients with cardiac myxoma suffer from cerebral or systemic embolism, which are serious complications, diagnosis of the tumor is vital. Between 1990 and 2000, 18 patients (6 males, 12 females), aged 24 to 73 years (mean, 55.3 years), were operated on for cardiac myxoma. The most common location of the myxoma was the left atrium (78%), and the transseptal surgical approach was preferred (78%) as it allows total resection of the left atrial myxoma along with its pedicle. Carney complex, a familial autosomal dominant form of atrial myxoma, was not found in any of the patients. There had been no operative or postoperative mortality and morbidity. The mean postoperative follow-up period was 5 years (mean, 1 to 10 years). No recurrence had been seen. We believe that the transseptal approach, in allowing total resection of the myxoma, prevents recurrence.
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INTRODUCTION
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Intracardiac tumors are rare. Myxoma is the most common primary cardiac tumor (45%), followed by lipoma (20%), papillary fibroelastoma (15%), and angioma (5%). Cardiac myxomas are benign mesenchymal tumors, and they are usually polypoid myxomatous or pedicled with a diameter of 5 to 6 cm. They are most frequently located in the left atrium (90%).1
In this report, we emphasize the importance of the diagnosis of these benign tumors, which is usually made after pulmonary, cerebral, or systemic complications; and we discuss diagnostic tools and surgical treatment for these tumors.
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PATIENTS AND METHODS
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Cases of intracardiac tumors that were surgically removed on cardiopulmonary bypass between 1990 and 2000 in our department were reviewed retrospectively. During this period, 18 patients were operated on for intracardiac masses that were histopathologically diagnosed as cardiac myxomas. Twelve of them were female (67%) and 6 were male (33%). They were aged 24 to 73 years (mean, 55.3 years). The myxoma was located in the left atrium in 14 cases (78%) and in the right atrium and the right ventricle in 2 cases each (together 22%). The most common symptoms were dyspnea (95%), tachycardia (92%), fever (84%), weight loss (70%), and syncope (60%). We describe here some of the cases.
In a patient with left atrial myxoma, a huge mass protruded into the left ventricle and mimicked mitral stenosis on the transthoracic echocardiogram (Figure 1
). Two other cases were admitted to the neurology department with cerebral embolism. Transthoracic echocardiography revealed intracardiac masses. A 68-year-old male was admitted to the cardiology department complaining of angina pectoris. Transthoracic echocardiography revealed a left atrial myxoma and angiography showed 95% proximal stenosis of the left anterior descending coronary artery. The left internal mammary artery was anastomosed to the stenosed artery after resection of the myxoma by the transseptal approach. In a 41-year-old man, a left atrial myxoma was removed together with a wide cuff of normal left atrial tissue by a transseptal biatrial approach. He also had 85% distal stenosis of the circumflex coronary artery, but it could not be treated because the artery diameter was less than 1 mm. A 73-year-old female had a well-circumscribed, nonobstructive left atrial myxoma without a history of systemic embolism (Figures 2A and 2B).
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Figure 1. A huge left atrial myxoma on transthoracic echocardiography, protruding into the left ventricle and mimicking mitral stenosis. LV = left ventricle, RA = right atrium, RV = right ventricle.
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Figure 2. (A) Macroscopic specimens of a well-circumscribed left atrial myxoma in an elderly patient and (B) the usual appearance of a left atrial myxoma with irregular, heterogeneous, and polypoid features.
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In all cases, median sternotomy and extracorporeal circulation with core and topical hypothermia and cold crystalloid cardioplegia were used. All the myxomas were resected en bloc with their pedicle or with a wide cuff of normal tissue. All cardiac chambers were inspected to avoid missing any tumor emboli or an occasional multicentric lesion. In right-sided myxomas, right atri-otomy was performed, while in left atrial myxomas a transseptal approach after right atriotomy was used. A huge left atrial myxoma and adhesions necessitated a transseptal biatrial approach in 2 cases.
All the patients and their family members were examined physically and by echocardiography for the symptoms and signs of Carney complex, a familial autosomal dominant form of atrial myxoma. None were found.
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RESULTS
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The clinical and operative details of the patients are shown in Table 1. There was no operative or postoperative mortality or morbidity. All the patients were asymptomatic and in New York Heart Association functional class I in the early postoperative period up to midterm follow-up. The size of the resected tumors ranged from 2.5 x 4 x 2 cm to 18 x 7 x 5 cm. Mean postoperative hospitalization was 5 days (range, 4 to 6 days). Histological examination of the resected materials confirmed the echocardiographic and intraoperative diagnosis of cardiac myxoma in all cases.
Postoperatively, the mean follow-up period was 5 years (range, 1 to 10 years). No recurrence or distal metastasis had been seen.
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DISCUSSION
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The first resection of a left atrial myxoma was made by Crafoord using extracorporeal circulation in 1954.2 Primary tumors of the heart are very rare, and the majority of them are benign, with myxomas located in the left atrium being the most common form. Cardiac myxomas usually originate from the endocardium of the atrial septum, and 95% of them are located in the atria. They are frequently seen in females between the 3rd and 6th decades. A left atrial myxoma is exceptionally rare in the elderly. An autosomal dominant inherited disorder, Carney complex, is associated with atrial myxoma, spotty skin pigmentation, and endocrinopathy.3 If patients with cardiac myxoma show signs and symptoms of this disorder, their family members should be examined.
Palpitation, fever, and weight loss are the most frequent symptoms of cardiac myxomas, but cerebrovascular accident or peripheral arterial embolism can also be presented. Systemic embolism is seen in 30% to 45% of all cases of left atrial myxoma. In patients over 75 years of age, myxomas are usually seen as nonobstructive, well-circumscribed, calcified tumors, with a low risk of obstruction or embolism.4 It was reported that the only independent predictor of systemic embolism in left atrial myxoma is the polypoid nature.5 In our series, the left atrial myxomas in patients presenting with cerebrovascular accidents or peripheral embolism also had polypoid features.
At least 20% of ischemic strokes are cardioembolic, and cardiac myxoma is one of the rarest causes of cerebral embolism. It was reported that 25% to 45% of atrial myxomas cause cerebrovascular accidents, and recurrent cerebral embolism may be the main sign of atrial myxoma.6 Therefore, in the differential diagnosis of cerebral ischemic accident, left atrial myxoma should be suspected and transthoracic echocardiography should be performed. Surgical resection of cardiac myxomas is recommended as it prevents recurrent embolism. In 2 of our cases, left atrial myxoma was diagnosed by echo-cardiography after a cerebrovascular ischemic accident.
Although systemic embolism is a common complication of left atrial myxoma, coronary embolism leading to acute myocardial infarction is extremely rare. Two cases have been reported of coronary embolization from a left atrial myxoma that led to acute coronary complications.7,8
Right-sided cardiac myxomas are extremely rare, and they can cause pulmonary embolism before or during surgery. To prevent pulmonary embolism, the pulmonary artery can be crossclamped as soon as extracorporeal circulation is initiated.9 In addition, a single cannula in the superior vena cava can be used until fibrillation before another is inserted into the inferior vena cava.10
Transthoracic or transesophageal echocardiography and, more recently, magnetic resonance imaging are the most frequently used diagnostic tools for investigating intracardiac tumors. However, diagnosis can only be confirmed by histological examination of the excised tumor. Intraoperative transesophageal echocardiography was not performed in any of our cases as preoperative transthoracic 2-dimensional echocardiography studies were sufficient.
Liu and co-workers11 compared the use of computed tomography and echocardiography in the diagnosis of myxoma. They reported that the former is as effective as the latter because it displays a clear image, the precise location, and the movement of the tumor with the flowing bloodstream in the cardiac chamber, thus providing reliable indications for surgery.
Surgery is the choice of treatment. Different approaches have been described for removing left atrial myxomas: right lateral, transseptal, transseptal biatrial (Dubost), and superior transseptal. In our cases of left atrial myxoma, the transseptal approach was preferred, but a transseptal biatrial approach was needed in 2 cases. The superior transseptal approach has been reported as an excellent method in comparison with the others by providing a clear surgical view for resecting left atrial myxomas.12
Recently, it has been reported that a malignancy potential is associated with multiple distant metastasis of recurrent cardiac myxomas.13 Therefore, long-term follow-up is indicated in these patients. All our patients had been followed up for a mean period of 5 years, with no recurrence or distant metastasis with malignancy potential.
In conclusion, myxomas should be suspected in any cases with peripheral arterial embolism, and embolic fragments should be examined histopathologically. We suggest the transseptal approach as an easy and safe method for removing left atrial myxomas.
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ABSTRACT
INTRODUCTION
