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Tex Heart Inst J. 2009; 36(2): 158–159.
PMCID: PMC2676614

Papillary Fibroelastoma of the Aortic Valve as a Cause of Transient Ischemic Attack

Abstract

Diagnostic evaluation of embolic neurologic events requires the consideration of cardiac causes. We recently encountered a case that emphasizes the importance of cardiac tumor as a source of embolic events. We present herein the case of a 42-year-old woman who suffered a transient ischemic attack caused by a papillary fibroelastoma that originated from the aortic valve.

Key words: Aortic valve/pathology, echocardiography, transesophageal, heart neoplasms/pathology, heart valve diseases/ultrasonography, heart valves/pathology, fibroma/complications/diagnosis/therapy/ultrasonography, transient ischemic attack

Diagnostic evaluation of embolic neurologic events requires the consideration of cardiac causes. We recently encountered a case that emphasizes the importance of cardiac tumor as a source of embolic events. Primary cardiac tumors are rare: their prevalence in a series of multiple autopsies1 was only 0.02%. Cardiac papillary fibroelastoma, the 2nd most common primary benign cardiac tumor after cardiac myxoma, accounts for 10% of all cardiac tumors and is the most common tumor of the heart valves. Papillary fibroelastoma occurs slightly more often in men than in women (55% vs 45%), and the mean age of patients is 60 years.2 Although some authors associate papillary fibroelastoma with an underlying risk of heart disease, no risk factors have been established, and the histogenesis of these tumors remains a controversial topic.3,4 Concomitant valvular dysfunction is attributed only rarely to these tumors.5 Clinical manifestations of papillary fibroelastoma include cerebrovascular accidents, myocardial infarction, pulmonary embolism, congestive heart failure, and sudden cardiac death. Most patients, however, remain asymptomatic or display symptoms incidental to the tumor itself.2,3

Case Report

In July 2006, a 42-year-old woman was referred to our department for evaluation and surgical treatment of an aortic valve tumor. The tumor was discovered with the aid of transthoracic echocardiography (TTE) during a diagnostic evaluation after a transient ischemic attack (TIA) that had been characterized by left-sided weakness. The patient's medical history was unremarkable, except for the recent TIA.

Upon admission to our cardiovascular surgery service, the patient had no symptoms of heart failure. Preoperative transesophageal echocardiography (TEE) confirmed the presence of a mobile mass (3–4 mm in diameter) on the leading edge of the right coronary cusp (Fig. 1). The aortic valve appeared to be structurally normal. The patient's left ventricular ejection fraction was 0.53.

figure 18FF1
Fig. 1 Preoperative transesophageal echocardiogram confirms the presence of a 3–4 mm mobile mass on the leading edge of the right coronary cusp.

The patient was taken to the operating room, where cardiopulmonary bypass was initiated after ascending aortic and central venous cannulation. A transverse aortotomy was performed. The 3-mm papillary fibroelastoma was then excised from the leaflet edge of the right coronary cusp, which spared the aortic valve. The other leaflets were carefully inspected, and no other tumors were found. Postoperative TEE showed no evidence of aortic stenosis or regurgitation (Fig. 2). Subsequent histopathologic examination confirmed the diagnosis of a benign papillary fibroelastoma of the aortic valve.

figure 18FF2
Fig. 2 Postoperative color-flow Doppler transesophageal echocardiogram shows no evidence of aortic stenosis or regurgitation.

The patient was discharged from the hospital with no neurologic or cardiovascular symptoms.

Discussion

The case illustrates the importance of echocardiography in considering a cardiac source, such as papillary fibroelastoma, as the cause of unexplained embolic events.4 The medical literature describes several cases of recurrent cerebrovascular accidents associated with permanent neurologic damage, the source of which was recurrent embolization from papillary fibroelastoma.6 Even very small papillary fibroelastomas (in our patient's case, 3 mm in diameter) can lead to neurologic damage.7,8 The transient nature of our patient's cerebrovascular accident makes her case highly unusual.

Transthoracic echocardiography and TEE have proved to be sensitive tools for the diagnosis of rare cardiac tumors, and the echocardiographic characteristics of each variety of tumor have been established.5

According to Sun and colleagues,4 sensitivity in the detection of papillary fibroelastomas is 61.9% for TTE and 76.6% for TEE; sensitivity increases considerably when tumors smaller than 2 mm are excluded from the study (88.9% for TTE). Papillary fibroelastomas are usually mobile and are attached to the endocardium by a stalk. Findings in histologic examination of these tumors include a matrix consisting of mucopolysaccharides, varying layers of elastic fibers, and rare spindle cells covered by a single hyperplastic endothelial layer.3

Papillary fibroelastomas occur predominantly on valvular surfaces (77%)—most often the aortic valve (44%), followed by the mitral valve (35%), the tricuspid valve (15%), and the pulmonary valve (8%). More than 95% of these tumors are found in the left side of the heart, and most of them are small (< 1 cm) and solitary. However, there have been reports of patients with multiple tumors at various locations within the heart, which emphasizes the need for careful intraoperative inspection.2,4,9

In the patient described herein, the tumor was excised using a valve-sparing technique, which is the treatment of choice in symptomatic patients. This technique yields excellent outcomes and is usually curative, although we recommend annual follow-up of these patients to check for reoccurrence. If the valve has been mechanically compromised, however, repair or replacement may be necessary. Thorough inspection of the other leaflets and valves should be undertaken to rule out multiple tumors. In symptomatic patients who have surgical contraindications, anticoagulation therapy may be an option.2

Footnotes

Address for reprints: Mehmet H. Akay, MD, 2475 Underwood, Apt. #172, Houston, TX 77025. E-mail: moc.liamtoh@yakahm

References

1. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996;77(1):107. [PubMed]
2. Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003;146(3):404–10. [PubMed]
3. Burke A, Virmani R. Tumors of the heart and great vessels. Fascicle 16, 3rd Series. In: Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1996. p. 231.
4. Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001;103(22):2687–93. [PubMed]
5. Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll Cardiol 1997;30(3):784–90. [PubMed]
6. Dehnee AE, Brizendine S, Herrera CJ. Recurrent strokes in a young patient with papillary fibroelastoma: a case report and literature review. Echocardiography 2006;23(7):592–5. [PubMed]
7. Grinda JM, Couetil JP, Chauvaud S, D'Attellis N, Berrebi A, Fabiani JN, et al. Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;117(1):106–10. [PubMed]
8. Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;59(2):538–41. [PubMed]
9. Lichtenstein HL, Lee JC, Stewart S. Papillary tumor of the heart: incidental finding at surgery. Hum Pathol 1979;10(4): 473–5. [PubMed]

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