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J Thorac Cardiovasc Surg 1996;112:199-201
© 1996 Mosby, Inc.

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TRANSAORTIC VIDEO-ASSISTED EXCISION OF A LEFT VENTRICULAR PAPILLARY FIBROELASTOMA

Indianapolis, Ind., and Chicago, Ill.

Received for publication August 22, 1995 Accepted for publication Sept. 13, 1995. Papillary fibroelastomas are rare intracardiac tumors that can arise from any endocardial surface. Before the advent of echocardiography, diagnosis was primarily an incidental finding at autopsy or operation. Despite the diminutive size of papillary fibroelastomas, surgical excision is generally indicated to prevent life-threatening coronary, cerebral, and systemic emboli. This article reports the first video-assisted removal of an intracardiac tumor.

A 57-year-old white man was admitted for evaluation of recurrent atrial fibrillation. Previous operations included myomectomy for idiopathic hypertrophic subaortic stenosis 10 years before this admission. History, results of physical examination, and laboratory profile were unremarkable. Despite appropriate medical therapy, the patient remained in atrial fibrillation.

Before elective cardioversion, a two-dimensional echocardiographic scan unexpectedly demonstrated a stalked 1.5 x 1.5 cm mass arising from the posterior medial papillary muscle. Echocardiographic diagnosis was consistent with a small myxoma or papillary fibroelastoma. Cardiac catheterization demonstrated normal coronary anatomy and no evidence of left ventricular outflow tract obstruction. Surgical excision was recommended in consideration of the known potential for coronary, cerebral, and systemic embolization.

Sternotomy was uneventful; bicaval and ascending aortic cannulation were accomplished and total cardiopulmonary bypass was initiated with systemic cooling to 30° C. The small left atrial size made the standard left atrial approach less desirable; a transaortic approach was chosen to avoid a ventricular incision, with its potential complications.

With the patient in cardioplegic arrest, a hockey-stick incision was created in the ascending aorta; however, transvalvular inspection of the left ventricular cavity did not demonstrate the mass. With standard videothorascopic equipment, a viewing scope that allowed simultaneous visualization and biopsy was passed through the aortic valve and into the left ventricle. A pedicled mass with delicate papillary fronds resembling a sea anemone was seen arising from the endocardial surface at the base of the posterior medial papillary muscle. The stalk of the mass was easily grasped with the biopsy portion of the viewing scope, allowing excision of the entire tumor (Fig. 1). Intraoperative transesophageal echocardiography demonstrated no residual mass.

View larger version (98K): [in this window] [in a new window]   Fig. 1. Gross appearance of the excised papillary fibroelastoma with its attached stalk (arrow).

The postoperative course was uncomplicated, with the exception of recurrent atrial fibrillation necessitating cardioversion. Patient follow-up at 20 months demonstrated no evidence of tumor recurrence.

Papillary fibroelastomas are rare, characteristically small, nonneoplastic tumors representing 1% to 4% of all intracardiac masses. ¹ Although infrequently seen in patients younger than 50 years, papillary fibroelastomas are the most common type of tumor arising from cardiac valves and have been reported to arise from any endocardial surface. ²

Before the advent of echocardiography, incidental diagnosis was occasionally made at the time of heart operations; however, such tumors were usually pathologic curiosities discovered at autopsy. The techniques of two-dimensional echocardiography and, more recently, transesophageal echocardiography provide both a definitive preoperative diagnosis and a means of accurate postoperative follow-up. ³ Cardiac catheterization is still recommended to exclude associated coronary artery disease; however, left ventriculography should be avoided to prevent iatrogenic tumor embolization.

Grossly, these tumors resemble sea anemones, with numerous villuslike projections extending from a central stalk. Histologically, the papillary fronds typically have three distinct architectural zones: (1) a central core consisting of dense connective tissue, (2) a peripheral loose myxomatous zone with a rich mucopolysaccharide matrix, and (3) an overlying layer of hyperplastic endothelium. This last layer of endothelium is contiguous with the endothelial cells lining the endocardium or cardiac valves.

Despite their small size, papillary fibroelastomas have been reported to cause angina and sudden death from either coronary emboli or direct ostial occlusion by prolapsing tumor. ⁴ In addition, cerebral embolization and infarction have been reported to be caused by tumor particles or, more likely, fragmentation of thrombus, which has a propensity toward accumulation on the extensive surface area provided by the tumor fronds. ^3,5 Although papillary fibroelastomas are considered "benign," their clinical course is deceptive. In light of their unpredictable nature, papillary fibroelastomas should be resected even in the patient who has no symptoms.

Footnotes

From the Department of Cardiovascular-Thoracic Surgery, St. Vincent Medical Center, Indianapolis, Ind.,^aand the Departments of Cardiovascular-Tthoracic Surgery,^b and Cardiology,^c Rush–Presbyterian–St.. Luke's Medical Center, Chicago, Ill.

References

1. McAllister HA, Fenogolio JJ. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978:20-5.
   
2. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127-31.[Abstract]
   
3. Ong LS, Nanda NC, Barold SS. Two-dimensional echocardiographic detection and diagnostic features of left ventricular papillary fibroelastoma. Am Heart J 1982;103:917-8.[Medline]
   
4. Boone S, Higginson LA, Walley VM. Endothelial papillary fibroelastomas arising in and around the aortic sinus, filling the ostium of the right coronary artery. Arch Pathol Lab Med 992;116:135-7.
   
5. McFadden PM, Lacy JR. Intracardiac papillary fibroelastoma: an occult cause of embolic neurologic deficit. Ann Thorac Surg 1987;43:667-9.[Abstract]
   

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