Asian Cardiovasc Thorac Ann 2006;14:e53-e54
© 2006 Asia Publishing EXchange Ltd
Papillary Fibroblastoma of Tricuspid Valve With Pulmonary Embolization
Pasquale Mastroroberto, MD,
Silvio Olivito, MD,
Francesco Onorati, MD,
Antonio di Virgilio, MD,
Salvatore Merola, MD,
Attilio Renzulli, MD
Cardiovascular Surgery Unit, University Magna Graecia, Catanzaro, Italy
For reprint information contact: Pasquale Mastroroberto, MD Tel: 39 09 6171 2308 Fax: 39 09 6171 2307 Email: mastroroberto{at}unicz.it, Cardiovascular Surgery, Medical School, Via T.Campanella, Catanzaro 88100, Italy.
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ABSTRACT
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A 53-year-old man was diagnosed with a papillary fibroelastoma of the tricuspid valve with pulmonary embolization and associated coronary artery disease. He underwent excision of the tumor and coronary bypass grafting. Due to potential embolization, it is recommended that all such fibroelastomas be excised.
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INTRODUCTION
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The incidence of primary cardiac tumors is reported to be less than 0.3% in autopsy series, and approximately 83% of these neoplasms are benign; 50% are cardiac myxomas.1,2 Papillary fibroelastoma is the third most common primary tumor and generally involves the cardiac valves. Most papillary fibroelastomas do not cause symptoms and are incidental findings at autopsy, at surgery, or during echocardiography. Early diagnosis of this pathology is very important to avoid systemic emboli, as previously documented.3 We report a case of concomitant right-sided embolizing fibroelastoma and coronary artery disease.
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CASE REPORT
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A 53-year-old man was urgently admitted with a history of left-sided chest pain relieved by sublingual administration of nitroglycerin, accompanied with dyspnea. An electrocardiogram revealed normal sinus rhythm with poor R-wave progression from precordial leads to V1 V6. Chest radiography indicated chronic obstructive pulmonary disease. Cardiac enzymes and troponin were essentially within normal limits. Echocardiography showed a 2.0–3.0 cm mass on one of the tricuspid leaflets (Figure 1
). A ventilation-perfusion lung scan was also performed and was read as significant right pulmonary emboli without signs of deep venous thrombosis. A coagulation profile was normal. There were no signs of local or systemic infection. Cardiac catheterization with coronary angiography and ventriculography revealed significant stenosis of the left anterior descending, first obtuse marginal, and interventricular posterior branches. A cardiac right-sided tumor was strongly suspected, associated with severe coronary disease, so the patient was advised to undergo surgery. A 3.5 x 2.5 x 2.0cm mass attached to the septal tricuspid leaflet was excised through a median sternotomy, and triple coronary bypass was performed. Microscopy revealed the characteristics of papillary fibroelastoma (Figure 2).
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Figure 1. Two-dimensional echocardiogram (apical 4-chamber view) showing a mass arising from the tricuspid valve. PFE = papillary fibroelastoma, TV = tricuspid valve.
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Figure 2. Microscopy showing hyperplastic endothelial cells covering a collagen core and surrounded by elastic fibers (hematoxylin and eosin stain, original magnification x 200).
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DISCUSSION
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The term "fibroelastoma" describes the gross and microscopic characteristics of this tumor, which consist of multiple papillary fronds with a collagen core surrounded by elastic fibers and covered with endocardial cells. Symptoms related to this neoplasm are often absent because of its small size. The mitral and aortic valves are the most common sites of papillary fibroelastoma in adults, whereas the tricuspid valve is most commonly involved in children. Typical echocardiographic features are pedunculated finger-like mobile excrescences on one or more valves, ranging in size from a few millimeters to a few centimeters, and attached to the endocardial portion of an intracardiac native valve by small stalks located on the midportion or body of the valve. This neoplasm has a more sharply defined acoustic interface than vegetation, and its typical locations are the atrial surface of the atrioventricular valves and the ventricular surface of the semilunar valves.
Our case demonstrates that a right-sided tumor is potentially as dangerous as a left-sided one, so it is reasonable to perform surgical resection even if the patient is asymptomatic. In a review of the literature, Yee and colleagues4 suggest that a papillary fibroelastoma should be removed if the patient needs open heart surgery for other reasons, or in the case of a left-sided symptomatic tumor. In patients with left-sided asymptomatic tumors, anticoagulant or antiplatelet therapy or surgery should be performed, whereas a surgical approach for right-sided fibroelastoma may be performed if symptomatic. This statement is contradicted by this report, and also by others with the conviction that, after careful evaluation, all types of fibroelastoma should be surgically removed along with valve replacement or valvuloplasty if the native valve is involved.5 Echocardiography remains the gold-standard for diagnosis of a cardiac tumor. Transesophageal echocardiography is superior because of its sensitivity in revealing the site of origin, the number of masses, the mobility of the tumor, and its differentiation from thrombus or valve vegetation.4
It was concluded from this experience that despite its benign pathology, excision of cardiac papillary fibroelastoma is preferable, with a conservative approach to the involved valve. A careful follow-up is warranted to evaluate the possibility of recurrence which is as yet unknown.
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REFERENCES
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- McAllister HA Jr. Primary tumors and cysts of the heart and pericardium. Curr Probl Cardiol 1979;4:1–51.[Medline]
- Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg 1990;38(Suppl 2):183–91.
- Gorton ME, Soltanzadeh H. Mitral valve fibroelastoma. Ann Thorac Surg 1989;47:605–7.[Abstract]
- Yee HC, Nwosu JE, Lii AD, Velasco M, Millman A. Echocardiographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol 1997;80:811–4.[Medline]
- Neerukonda SK, Jantz RD, Vijay NK, Narrod JA, Schoonmaker FW. Pulmonary embolization of papillary fibroelastoma. Arising from the tricuspid valve. Tex Heart Inst J 1991;18:132–5.[Medline]
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ABSTRACT
INTRODUCTION
