Ann Thorac Surg 2003;75:1323-1324
© 2003 The Society of Thoracic Surgeons
Case report
Multifocal right atrial myxoma and pulmonary embolism
Alden M. Parsons, MDa,
Frank C. Detterbeck, MDa*
a Division of Cardiothoracic Surgery, Department of Surgery, University of North Carolina, Chapel Hill, North Carolina, USA
Accepted for publication September 27, 2002.
* Address reprint requests to Dr Detterbeck, Division of Cardiothoracic Surgery, CB# 7065, 108 Burnett-Womack Building, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599-7065, USA
e-mail: fdetter{at}med.unc.edu
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Abstract
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Multifocal cardiac myxoma with greater than two foci is rarely reported in the literature. We report a case of a 22-year-old woman who presented with profound right heart failure, and was found to have seven right atrial myxomas with bilateral pulmonary embolism, including near-complete occlusion of right pulmonary arterial flow. Multifocal atrial myxoma occurs most often in the familial setting, and often is associated with recurrence. Her disease was nonfamilial. She was successfully treated with surgical resection, and has had complete recovery with no evidence of recurrence over a 4-year period.
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Introduction
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Since the first successful resection of cardiac myxoma by Crafoord in 1954, this rare benign tumor has been well described, with an estimated incidence of 0.5 per million population per year [1]. The clinical presentation may vary widely depending on the intracardiac anatomic location. It is most commonly of left atrial origin (85% to 90%), less commonly right atrial (10% to 12%), and rarely ventricular (1% to 4%) [2, 3]. Multifocal intracardiac myxomatous disease, however, has not been well described, particularly with regard to its natural history, variations in clinical presentation, and patterns of recurrence.
A 22-year-old woman presented with severe dyspnea on exertion and pedal edema, increasing dramatically over a 6-month period. Her past medical history was negative for any cardiopulmonary disease, and she had no known family history of thromboembolic disorders or myxoma. An admission chest roentgenogram revealed ill-defined pleural based opacities. A chest computed tomography demonstrated multiple bilateral peripheral intraparenchymal densities as well as large bilateral pulmonary artery filling defects and a 2 x 3.5-cm right atrial mass. Thrombotic pulmonary emboli with peripheral lung infarction were suspected. The patient was anticoagulated, and a thromboembolic workup was initiated.
A lung ventilation-perfusion scan confirmed the diagnosis of pulmonary embolism and revealed two subsegmental perfusion defects on the left, and a perfusion defect to the entire right lung, with the exception of one segment of the right upper lobe (Fig 1). No deep venous thrombus was detected, and a hypercoagulable workup was negative. An echocardiogram revealed at least two large right atrial masses, with pulmonary artery pressures estimated to be 80 to 85 mm Hg, severely depressed right ventricular contractility, right ventricular enlargement, severe tricuspid regurgitation, and right atrial dilatation.
Given the possibility of myxomatous emboli, the patient was taken to the operating room and underwent pulmonary thromboendarterectomy and excision of right atrial myxomata by a median sternotomy and circulatory arrest. She was found to have seven distinct foci of right atrial tumor (Fig 2),
some of which were located on the atrial septum and some on the atrial free wall. The left atrium, left ventricle, and right ventricle were all carefully inspected, and no additional foci were detected. The myxomas were excised with either full-thickness or partial-thickness excisions of the atrial wall. Longitudinal arteriotomies were performed on both the left and right pulmonary arteries, revealing the myxomatous emboli, which were removed by en bloc thrombendarterectomies. Pathologic evaluation of the pulmonary arterial embolectomy specimens (Fig 3)
was consistent with myxoma.
Her postoperative course was uneventful. Repeat V/Q scan revealed normal right lung perfusion, with the exception of the anterior segment of the right upper lobe, and only a subsegmental perfusion defect in the lingula on the left. Her postoperative echocardiogram revealed improved right ventricular function, and her follow-up echocardiograms as an outpatient have normalized, without evidence of recurrence over a 4-year period.
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Comment
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This case is unique for several reasons. First, multifocal myxomas with more than two foci are rare. To our knowledge, there are no previously reported cases with greater than six foci of intracardiac myxoma, and there is only one reported case of a patient with six foci of atrial myxoma, and they were left atrial recurrences detected at autopsy [4]. Besides these cases and one additional case of four foci of tumor [7], all other reported cases of multifocal myxomas referred to two or three foci of tumor.
Second, this case presents several features that are uncharacteristic of a nonfamilial (sporadic) case. Multifocal myxoma is rarely reported in the literature in nonfamilial (sporadic) cases (6%) [8], whereas multifocal cardiac myxoma with at least two foci is relatively common in familial myxoma, occurring in approximately 33% of cases [8]. Sporadic myxoma, which accounts for 95% of cases [6], is more common in an older age group, and is more often left-sided (83% to 89% vs 62%) as compared with familial myxoma [2, 3, 6, 8]. There was no family history of myxoma in our patient despite specific questioning, nor did the patient have any stigmata of conditions or syndromes, such as the Carney syndrome, which are often associated with familial myxoma [8].
The embolization of the myxoma in this case does not appear to be unusual, although it resulted in a dramatic clinical presentation. The reported rate of left- or right-sided embolic phenomena associated with cardiac myxoma ranges from 21% to 33% [2, 3, 6]. There is a slight difference in the reported rates of embolism of right- and left-sided myxomas, with systemic emboli occurring in 21% to 33% of patients with left-sided cardiac myxoma, and pulmonary embolism occurring in 2% to 24% of patients found to have right-sided cardiac myxoma [2, 3, 6]. These numbers are likely confounded by the more clinically obvious and earlier presentation of peripheral and cerebral emboli compared with the more insidious pulmonary emboli. Whether embolism is more likely in cases of multifocal disease or familial cases has not been analyzed.
The likelihood of recurrence in our patient is difficult to determine given the multifocal yet nonfamilial nature of her disease. Recurrence of myxoma is most often reported in cases of multifocal, familial myxomatous disease. A review of reported cases of recurrent myxoma in the English language literature found an overall recurrence rate of 2% to 3% [5], but the recurrence rate ranges from 10% to 75% in reports of familial myxoma [5, 6]. In addition, multifocal myxoma is associated with a 33% rate of recurrence [5]. The interval from excision to recurrence is reported ranging from a few months to 8 years. The patient reported here has not developed any signs of recurrence, as demonstrated by surveillance with serial echocardiography over the course of 4 years.
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References
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- MacGowan S.W., et al. Atrial myxoma: national incidence, diagnosis and surgical management. Ir J Med Sci 1993;162:223-226.[Medline]
- Bortolotti U., et al. Surgical excision of intracardiac myxomas: a 20-year follow-up. Ann Thorac Surg 1990;49:449-453.[Abstract]
- Bjessmo S., Ivert T. Cardiac myxoma: 40 years’ experience in 63 patients. Ann Thorac Surg 1997;63:697-700.[Abstract/Free Full Text]
- Jugdutt, et al. An unusual case of recurrent left atrial myxoma. Can Med Assoc J 1975;112:1099–100
- Shinfeld A., et al. Recurrent cardiac myxoma: seeding or multifocal disease?. Ann Thor Surg 1998;66:285-288.[Abstract/Free Full Text]
- Bhan A., et al. Surgical experience with intracardiac myxomas: long-term follow-up. Ann Thor Surg 1998;66:810-813.[Abstract/Free Full Text]
- Liebler G.A., et al. Familial myxomas in four siblings. J Thor Cardiovasc Surg 1975;71:605-608.
- Carney J.A. Differences between nonfamilial and familial cardiac myxoma. Am J Surg Path 1985;9:53-55.[Medline]
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Abstract
Introduction
