Copyright © 2007, The European Society of Cardiology
Papillary fibroelastoma of the aortic valve: A rare cause of stroke
aDepartment of Cardiology, Ziekenhuis Walcheren, Vlissingen, The Netherlands
bDepartment of Neurology, Ziekenhuis Walcheren, Vlissingen, The Netherlands
Received 9 June 2006; received in revised form 13 July 2006; accepted after revision 26 July 2006.
* Corresponding author. Department of Cardiology, Ziekenhuis Walcheren, Koudekerkseweg 88, Postbus 3200, 4380 DD Vlissingen, The Netherlands. Tel.: +31 118 425000; fax: +31 118 425331. walpot{at}zwv.nl
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Abstract |
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We report a case of a 59-year-old woman with recurrent cerebrovascular insults caused by a papillary fibroelastoma of the aortic valve. Primary cardiac tumors are rare. Papillary fibroelastoma (PFE) is the most common valvular tumor and the second cardiac benign tumor after myxoma. The clinical presentation of PFE varies from asymptomatic to severe embolic complications. The tumor was surgically removed to avoid new embolic events.
Keywords: Papillary fibroelastoma; Cardiac tumor; Stroke; Transesophageal echocardiography
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Case presentation |
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A 59-year-old woman, with a medical history of stroke in 1997, arterial hypertension and dyslipidemia, was re-evaluated because of two episodes of transient ischemic attack (TIA). She was treated with aspirin, dipyridamol, metoprolol and a statin. She was in sinus rhythm. Transthoracic echocardiography (TTE) showed a sclerotic aortic valve without stenosis. A small mass, attached to the aortic valve, was suspected. Additional transesophageal echocardiography (TEE) confirmed a tumorous structure, attached to the non-coronary cusp of the aortic valve (Figs. 1 and 2
). The mass was surgically removed. An attempt to repair the aortic valve was unsuccessful because of severe residual aortic regurgitation during peri-operative TEE and necessitated replacement by a mechanical prosthetic valve.
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Histologically a papillary fibroelastoma (PFE) was observed.
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Discussion |
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Primary cardiac tumors are rare and the relative incidence of PFE among these tumors is low. In a 1957–1991 Mayo Clinic1 series of 110 primary cardiac tumors that were surgically excised, there were 80 myxomas and 7 PFEs (6.4%). PFE is the most common valvular tumor.
The clinical presentation of PFE varies widely from asymptomatic to severe embolic complications.2 In the series of primary valve tumors compiled by Ryan et al.,3 66% of the patients with PFE were asymptomatic. In a meta-analysis of 725 patients with a cardiac PFE, TIA or stroke occurred in 120 cases.4
Most investigators recommend an aggressive approach to PFE, especially for lesions of the left side of the heart. Surgical excision of the PFE is considered to be curative and, in most cases, the tumor can easily be removed because of its pedunculation. In the series of Gowda et al.,4 a valve sparing procedure was performed in 90% of the patients, referred for surgical excision of the cardiac PFE.
Long-term oral anti-coagulation is recommended for the patients who are not considered for surgical resection of the PFE. However, there are no randomized controlled data available, proving the efficacy of anti-coagulation in preventing embolic events in patients with PFE.
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References |
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- Tazelaar H.D., Locke T.J., Mc Gregor C.G. Pathology of surgically excised primary 2cardiac tumors. Mayo Clin Proc (1992) 67:965–975.
- Shahian D.M. Papillary fibroelastoma. Semin Thorac Cardiovasc Surg (2000) 12(2):101–110.[Medline]
- Ryan P.E. Jr., Obeid A.I., Parker F.B. Jr. Primary cardiac valve tumors. J Heart Valve Dis (1995) 4:222–226.[Medline]
- Gowda R.M., Khan I.A., Nair C.K., Metha N.J., Vasavada B.C., Sacchi T.J. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J (2003) 146:404–410.[CrossRef][Web of Science][Medline]
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