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Ann Thorac Surg 2002;74:1252-1254
© 2002 The Society of Thoracic Surgeons
a Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
Accepted for publication May 19, 2002.
* Address reprint requests to Dr Orszulak, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905 USA
e-mail: orszulak.thomas{at}mayo.edu
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An 86-year-old woman had progressive dyspnea, chronic cough, and lower extremity edema, and presented with a syncopal episode. At admission, she was hypotensive and in acute renal failure requiring hemodialysis. She was in atrial fibrillation and had diffuse pulmonary crackles. Chest roentgenogram demonstrated bilateral pulmonary infiltrates. On transesophageal echocardiogram, she had a normal left ventricular size with mildly decreased ejection fraction. A 4.0-cm mobile mass was seen in the right atrium, attached to the right atrial wall above the tricuspid anulus and below the right atrial appendage (Fig 1). Surgery was performed for diagnosis of the mass and pulmonary infiltrates. Under cardiopulmonary bypass with bi-caval cannulation, a 4-cm gelatinous pedunculated mass based on a small stalk on the inferior posterior border of the fossa ovalis was found and excised (Fig 2). There were no other tumors. Grossly, the mass appeared as a myxoma, however, pathologic examination of the atrial mass revealed a papillary fibroelastoma (Fig 3). A right lung biopsy was also performed that was read as acute and organizing pneumonia. Postoperatively, the patient’s hemodynamic status and renal and respiratory function improved. The chest roentgenogram remained unchanged.
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Papillary fibroelastomas are located with equal frequency in both sides of the heart [1]. They originate from the aortic valve in 29% of the cases, mitral in 25%, tricuspid in 17%, and the pulmonary valve in 13% [2]. PFE originate in other endocardial surfaces in less than 15% of cases, as in this report. They have been described in the left ventricular septum, the papillary muscles, the chordae, the mural endocardium, and in the right and left atrium [2].
They are usually singular in number ranging in size from a few millimeters to 2 cm [2]. More than 80% are less than 1 cm in diameter [4]. Only a few cases of PFE greater than 3 cm have been described [4]. Large PFE are usually located on the right side of the heart, as in this case.
Papillary fibroelastomas may cause embolic or obstructive symptoms. Embolization may occur from fragments of the papillary fronds or from thrombus that forms around the tumor [1, 2]. Embolization from tumors on the left side of the heart cause neurological symptoms in the majority of cases (transient ischemic attack or stroke). Myocardial infarction, angina, syncope, limb ischemia, and sudden death have also been described. If large enough, PFE can cause cardiac obstructive symptoms or intermittent coronary ostial obstruction [2].
Right-sided tumors remain predominantly asymptomatic until they are large enough to alter hemodynamic function by causing tricuspid obstruction, intermittent partial right ventricular outflow obstruction, or arrhythmias [1, 2]. A few cases of pulmonary embolism caused by PFE have been described [5]. Other symptoms included dyspnea, chest discomfort, and palpitations. In spite of being intimally associated with the valvular endocardium, they rarely cause permanent valvular dysfunction requiring valve replacement or repair during excision [1–3].
Pulmonary infiltrates are not associated with PFE. However, pulmonary infiltrates have been described in association with angiosarcomas of the right heart [6].
Histologically, the papillary fronds consist of a central core of dense connective tissue surrounded by a layer of loose connective tissue covered by hyperplastic endothelial cells (Fig 3) [2]. The fronds are enveloped by a layer of gelatinous material that dissolves when the tumor is placed in physiologic saline solution achieving its characteristic appearance of a sea anemone [3].
The exact nature of PFE is still controversial. They may represent a neoplasia, a degenerative process associated with trauma or repetitive hemodynamic stress to the endocardium or even a chronic form of viral endocarditis [3].
Before the advent of echocardiography, PFE were usually diagnosed at autopsy or during cardiac operations for other reasons. Currently, echocardiography can reliably diagnose PFE preoperatively in 88% of the cases [1, 3]. Its echocardiographic characteristics included a small mass attached to the valvular endocardium by a stalk with independent mobility. The fronds can be seen as strand-like projections specked with echolucency near the edges of the tumor. The gelatinous envelope can obscure these fronds making the differential diagnosis from myxoma difficult [3]. The differential diagnoses of PFE include myxoma, fenestrations, strands, Lambl’s excrescences, vegetation, and thrombus [1].
The identification of a PFE in a good-to-moderate surgical-risk patient is an indication for surgical removal to prevent its potentially life threatening systemic or pulmonary embolic complications [1–3]. Surgical excision of the tumor under cardiopulmonary bypass is the preferred treatment [1]. Surgical excision can be accomplished with preservation of the native valve in most cases. Care should be taken during manipulation of the heart, before aortic cross-clamping, to prevent fragmentation of the tumor and systemic embolization [1]. Intraoperatively transesophageal echocardiogram and videoscopic techniques play an essential role in identifying the number and location of the tumors, their anatomic attachment, and in assessing the adequacy of surgical resection [1]. Once these tumors are diagnosed, prophylactic anticoagulation should be initiated [3] while the patient awaits definitive treatment. If surgery is contraindicated, chronic anticoagulation should be continued [3]. Chronic anticoagulation alone is ineffective in preventing embolization [1]. The prognosis after surgical resection is good, with no reports of recurrence [2].
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This article has been cited by other articles:
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  C. Yerebakan, A. Liebold, G. Steinhoff, and C. A. Skrabal Papillary Fibroelastoma of the Aortic Wall With Partial Occlusion of the Right Coronary Ostium. Ann. Thorac. Surg., June 1, 2009; 87(6): 1953 - 1954. [Abstract] [Full Text] [PDF]
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 A. Le Guyader, K. Nubret, N. Darodes, and M. Laskar Removal of a ventricular mitral valve fibroelastoma by an aortic approach Interactive CardioVascular and Thoracic Surgery, December 1, 2006; 5(6): 785 - 787. [Abstract] [Full Text] [PDF]
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D. L. Ngaage, C. J. Mullany, R. C. Daly, J. A. Dearani, W. D. Edwards, H. D. Tazelaar, C. G.A. McGregor, T. A. Orszulak, F. J. Puga, H. V. Schaff, et al. Surgical Treatment of Cardiac Papillary Fibroelastoma: A Single Center Experience With Eighty-Eight Patients Ann. Thorac. Surg., November 1, 2005; 80(5): 1712 - 1718. [Abstract] [Full Text] [PDF]
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