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Ann Thorac Surg 1995;60:1105-1107
© 1995 The Society of Thoracic Surgeons

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Case Reports

Radical Excisional Therapy and Total Cardiac Transplantation for Recurrent Atrial Myxoma

Department of Surgery, Division of Cardiothoracic Surgery, Columbia University, College of Physicians & Surgeons, New York, New York

Accepted for publication March 31, 1995.

	Abstract

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We describe the management of a recurrent atrial myxoma extending to the pulmonary veins and superior vena cava. Deep hypothermia and circulatory arrest with left atrial and caval excision was required to achieve complete resection of the tumor. The patient is alive and well without evidence of tumor recurrence 18 months after transplantation. Radical en bloc cardiac resection is feasible in selected cases of cardiac tumors.

	Introduction

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Primary cardiac tumors are rare. Myxomas, the most common, usually arise from the atrial septum and grow into the atrial cavities. Classically, they occur in otherwise healthy middle-aged women as an isolated tumor [1]. Since 1954, when Crafoord [2] first excised an atrial myxoma, these tumors have been routinely managed by surgical excision under cardiopulmonary bypass; the extent of the excision has been debated in the literature [3, 4]. It has been estimated that cardiac myxomas recur with an approximate incidence of 7% [5]; recurrences are usually managed by re-excision. Inadequate primary excision, tumor seeding at the time of operation, and multiple foci of tumor have all been proposed as explanations for recurrence [5]. We describe the clinical course of a patient who presented with an extensive, recurrent atrial myxoma and progressive mitral stenosis precluding excision. Orthotopic heart transplantation was performed and required modification of the standard technique for cardiectomy and implantation.

A 47-year-old woman presented to her ophthalmologist for evaluation of a 1-minute episode of monocular blindness. Ocular examination was unremarkable and she was referred for further work-up. Physical examination carotid Doppler echocardiography revealed no abnormalities. For the following 1 years she noted recurrent intermittent episodes of amaurosis fugax. In October 1992, she noted decreased exercise tolerance, palpitations, and shortness of breath. A stress test was unremarkable, and transesophageal echocardiography revealed a left atrial tumor consistent with a myxoma (Fig 1). In December of the same year, she underwent an exploratory operation, at which time two intraatrial masses were noted. A firm septal mass was found and shelled out. A second sessile mass located near the posterior annulus was excised, leaving a rim of normal-appearing tissue. The posteriomedial commissure and posterior mitral leaflet appeared to be involved with tumor. Frozen section revealed a myxoma. A specimen of tissue was carved off from the posteriomedial commissure and all loose debris was removed. Given the extent of the tumor, the presence of only mild to moderate mitral stenosis, and the potential difficulty of seating a prosthetic valve, the mitral valve was not replaced. She had an unremarkable postoperative course and had good exercise tolerance 6 weeks after operation.

View larger version (79K): [in this window] [in a new window]   Fig 1. . Transesophageal echocardiogram performed before primary excision of the tumor. Tumor is seen lining the left atrium (LA) and involving the posterior mitral leaflet and subchordal apparatus. (AV = aortic valve; LV = left ventricle; RA = right atrium; RV = right ventricle.)

View larger version (83K): [in this window] [in a new window]   Fig 2. . Transesophageal echocardiogram performed at the time of presentation for tumor recurrence. The tumor is seen coating the left atrial side of the mitral valve, extending to the subchordal apparatus. Functional mitral stenosis with a resultant mitral valve area of 1.5 cm2 was noted. Extension into the left upper pulmonary vein can be seen. (LA = left atrium; LUPV = left upper pulmonary vein; MV = mitral valve.)

Her postoperative course was unremarkable; she was weaned off pressors and extubated the first postoperative day. She remained in sinus rhythm. Because of concern about pulmonary vein thrombosis, she was given heparin and aspirin. Transesophageal echocardiography on the fifth postoperative day revealed normal ventricular function and no thrombus in the left atrium or pulmonary veins. After treatment for an early episode of rejection, she was discharged home 19 days after transplantation receiving triple-drug immunosuppression and subcutaneous daily heparin.

Magnetic resonance imaging at 1 year revealed no evidence of intracavitary recurrent tumor or thrombus with widely patent pulmonary veins. She is alive and well 18 months after transplantation.

On gross examination, two atrial masses were encountered. The first, measuring 2 x 1.5 x 1 cm arose from the atrial septum. The second, measuring 5 x 4 x 1.5 cm, was white-yellow and firm, and totally replaced the mitral valve. A few chordae tendineae were encased by the mass. Microscopically, the tumor showed extensive fibrosis and sclerosis in its central portion with densely cellular regions in the periphery. A few mitotic figures were found. The staining pattern was typical for myofibroblasts. No definite myocardial invasion was seen, and the margins were tumor-free. The cellularity, immunohistochemical profile, and cytologic atypia were consistent with a diagnosis of cardiac fibroma of uncertain malignant potential.

	Comment

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To our knowledge, only 5 patients have been reported to undergo cardiac transplantation for management of primary cardiac tumors [6–10]. Three of these patients were diagnosed with sarcomas (atrial angiosarcoma, ventricular neurofibrosarcoma and ventricular fibrosarcoma), 1 with a ventricular lymphoma, and 1 patient with a ventricular fibroma. Three patients (those with tumor limited to the heart) benefitted from long-term survival, whereas the remaining 2 patients (with evidence of extracardiac disease) succumbed to metastatic disease 14 and 15 months after transplantation. Although no definite conclusion can be made regarding the management of these patients, complete tumor resection afforded by cardiectomy and transplantation may provide long-term survival for patients with localized primary cardiac tumors.

This case illustrates the well-known fact that atrial myxomas can cause a variety of clinical signs and symptoms that often are disguised as other more common systemic diseases. Although advances in echocardiography have greatly enhanced diagnostic accuracy, index of suspicion is still of paramount importance in the diagnosis of primary cardiac tumors.

The pathologic findings are of interest; although the original tumor excision yielded a diagnosis of ``atrial myxoma,'' the final pathologic diagnosis after cardiectomy revealed a ``fibroma of uncertain malignant potential.'' This discrepancy is not easily explained. Fibromas are tumors that almost exclusively arise from ventricular myocardium and usually afflict children [11, 12]. Under light microscopy, calcification is commonly noted and a predilection to involve the conduction system exists [13]; the histologic criteria for diagnosis, however, remain uncertain [14]. When site of origin, patient age, absence of calcifications, and biological behavior of this tumor are taken together, a recurrent atrial myxoma-with possible malignant degeneration-appears to be the most plausible diagnosis.

Because of their potential to obstruct, embolize, and cause arrhythmias, all cardiac myxomas require an attempt at excision. In most cases, surgical resection leads to cure. In a small fraction of cases, metachronous recurrences or multiple tumors occur. Even under such circumstances, re-excision is associated with significant palliation or cure. This report describes an unusual case of recurrence in which re-excision was deemed impossible and transplantation became necessary.

Total cardiac transplantation including separate pulmonary venous and caval anastomoses was performed with the aid of deep hypothermia and circulatory arrest. The concern for pulmonary vein thrombosis was addressed acutely with intravenous heparin and chronically with low-dose subcutaneous heparin and aspirin. The potential cure afforded by cardiectomy and cardiac transplantation warrants an awareness of this modality for the treatment of extensive recurrent atrial myxomas.

	Footnotes

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Address reprint requests to Dr Michler, Cardiac Transplantation Service, 177 Fort Washington Ave, Milstein Hospital 7GN-435, New York, NY 10032.

	References

Top Footnotes Abstract Introduction Comment References

 

1. Peters MN, Hall RJ, Cooley DA, Leachman RD, Garcia E. The clinical syndrome of atrial myxoma. JAMA 1974;230:695–701.
2. Crafoord CL. Discussion on mitral stenosis and mitral insufficiency. In: Lam CR, ed. Proceedings of the International Symposium on Cardiovascular Surgery, Henry Ford Hospital, Detroit, Michigan, March 1955. Philadelphia: Saunders, 1955:202.
3. Donahoo JS, Weiss JL, Gardner TJ, Fortuin NJ, Brawley RK. Current management of atrial myxoma with emphasis on a new diagnostic technique. Ann Surg 1979;189:763–8.
4. Read RC, White HJ, Murphy ML, Williams D, Sun CN, Flanagan WH. The malignant potentiality of left atrial myxoma. J Thorac Cardiovasc Surg 1974;68:857–68.
5. Gray IR, Williams WG. Recurring cardiac myxoma. Br Heart J 1985;53:645–9.
6. Jamieson SW, Gaudiani V, Reitz B, et al. Operative management of an unresectable tumor of the left ventricle. J Thorac Cardiovasc Surg 1981;81:797–9.
7. Aravot DJ, Banner NR, Madden B, et al. Primary cardiac tumors: is there a place for cardiac transplantation? Eur J Cardiothorac Surg 1989;3:521–4.
8. Horn M, Phebus C, Blatt J. Cancer chemotherapy after solid organ transplantation. Cancer 1990;66:1468–71.
9. Aufiero T, Pae W, Clemson B, et al. Heart transplantation for tumor. Ann Thorac Surg 1993;56:1174–6.
10. Yuh DD, Kubo SH, Francis MD, et al. Primary cardiac lymphoma treated with orthotopic cardiac transplantation: a case report. J Heart Lung Transplant 1994;13:538–42.
11. Reul GJ, Howell JF, Rubio PA, Petersen PK. Successful partial excision of an intramural fibroma of the left ventricle. Am J Cardiol 1975;36:262–5.
12. McAllister HA Jr. Primary tumors and cysts of the heart and pericardium. In: Harvey WP, ed. Current problems in cardiology. Chicago: Year Book Medical, 1979.
13. Williams DB, Danielson GK, McGoon DC, Feldt RH, Edwards WD. Cardiac fibroma. Long term survival after excision. J Thorac Cardiovasc Surg 1982;84:230–6.
14. Colucci WS, Braunwald E. Primary tumors of the heart. In: Braunwald N, ed. Heart disease. A textbook of cardiovascular medicine. Philadelphia: Saunders, 1994.

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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 Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Daniel J. Goldstein Mehmet C. Oz Robert E. Michler Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Goldstein, D. J. Articles by Michler, R. E. Search for Related Content PubMed Articles by Goldstein, D. J. Articles by Michler, R. E.