Multimodality Imaging of a Giant Aortic Valve Papillary Fibroelastoma

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Abstract

Papillary fibroelastomas (PFEs) are benign cardiac tumors arising from endocardium. They are commonly found on valvular surfaces and average 1.0–1.5 cm in size. Though often asymptomatic, PFEs can lead to potentially severe complications, primarily due to their embolic potential. Surgical resection is recommended for all symptomatic or large PFEs. We report the case of a patient presenting with cardiovascular symptoms who was found to have a very large aortic valve PFE, as diagnosed by histopathologic examination following surgical resection. Multimodality cardiovascular imaging demonstrates the classic morphologic findings, including a pedunculated appearance and oscillating “frond-like” surface projections.

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Most cited references 8

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Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.

Ramesh Gowda, Ijaz Khan, Chandra Nair … (2003)

With the advent of echocardiography, cardiac papillary fibroelastoma (CPF) is being increasingly reported. The demographics, clinical characteristics, pathological features, treatment, and prognosis of CPF are examined. Cases, case series and related articles on the subject in all languages were identified through a comprehensive literature search. Seven hundred twenty-five cases of CPF were identified. Males comprised 55% of patients. Highest prevalence was in the 8th decade of life. The valvular surface was the predominant locations of tumor. The most commonly involved valve was the aortic valve, followed by the mitral valve. The left ventricle was the predominant nonvalvular site involved. No clear risk factor for development of CPF has been reported. Size of the tumor varied from 2 mm to 70 mm. Clinically, CPFs have presented with transient ischemic attack, stroke, myocardial infarction, sudden death, heart failure, presyncope, syncope, pulmonary embolism, blindness, and peripheral embolism. Tumor mobility was the only independent predictor of CPF-related death or nonfatal embolization. Symptomatic patients should be treated surgically because the successful complete resection of CPF is curative and the long-term postoperative prognosis is excellent. The symptomatic patients who are not surgical candidates could be offered long-term oral anticoagulation, although no randomized controlled data are available on its efficacy. Asymptomatic patients could be treated surgically if the tumor is mobile, as the tumor mobility is the independent predictor of death or nonfatal embolization. Asymptomatic patients with nonmobile CPF could be followed-up closely with periodic clinical evaluation and echocardiography, and receive surgical intervention when symptoms develop or the tumor becomes mobile.

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Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients.

Alan Cheng, Michael W. Stewart, J. Thomas … (2001)

Cardiac papillary fibroelastoma (CPF) is a primary cardiac neoplasm that is increasingly detected by echocardiography. The clinical manifestations of this entity are not well described. In a 16-year period, we identified patients with CPF from our pathology and echocardiography databases. A total of 162 patients had pathologically confirmed CPF. Echocardiography was performed in 141 patients with 158 CPFs, and 48 patients had CPFs that were not visible by echocardiography (<0.2 cm), leaving an echocardiographic subgroup of 93 patients with 110 CPFs. An additional 45 patients with a presumed diagnosis of CPF were identified. The mean age of the patients was 60+/-16 years of age, and 46.1% were male. Echocardiographically, the mean size of the CPFs was 9+/-4.6 mm; 82.7% occurred on valves (aortic more than mitral), 43.6% were mobile, and 91.4% were single. During a follow-up period of 11+/-22 months, 23 of 26 patients with a prospective diagnosis of CPF that was confirmed by pathological examination had symptoms that could be attributable to embolization. In the group of 45 patients with a presumed diagnosis of CPF, 3 patients had symptoms that were likely due to embolization (incidence, 6.6%) during a follow-up period of 552+/-706 days. CPFs are generally small and single, occur most often on valvular surfaces, and may be mobile, resulting in embolization. Because of the potential for embolic events, symptomatic patients, patients undergoing cardiac surgery for other lesions, and those with highly mobile and large CPFs should be considered for surgical excision.

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Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation.

Kyle Klarich, Maurice Enriquez-Sarano, George M. Gura … (1997)

We sought to determine the clinical and echocardiographic characteristics of papillary fibroelastoma (PFE). PFE is a rarely encountered cardiac tumor about which relatively little is known. Institutional records were reviewed for the years 1980 to 1995 for patients with pathologic or echocardiographic diagnosis of PFE. Group 1 included 17 patients with the pathologic diagnosis of PFE who also underwent echocardiography. Echocardiographic features of PFE were established in group 1. Group 2 included 37 patients with only echocardiographic evidence of PFE. In group 1, 7 (41.2%) of 17 patients had symptoms related to PFE. Neurologic events occurred in 5 (29.4%) of 17 patients. All patients had the tumor surgically removed. During follow-up, no new embolic events occurred. Echocardiographic characteristics of PFE included a small tumor (12.1 +/- 6.5 x 9.0 +/- 4.3 mm), usually pedunculated (14 [94%] of 17 patients) and mobile, with a homogeneous speckled pattern and a characteristic stippling along the edges. PFEs were most common on valvular surfaces (12 [60%] of 20 PFEs) but were not uncommon on other endocardial surfaces (8 [40%] of 20 PFEs). The tumor did not cause valvular dysfunction. In group 2, 16 (43%) of 37 patients were asymptomatic. Five patients (13.5%) had a previous neurologic event. During follow-up (mean 31 months, range 1 to 77), nine neurologic events occurred. PFEs are associated with embolism, can be diagnosed with echocardiography, are often an incidental clinical finding and do not cause valvular dysfunction.

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Author and article information

Journal

Journal ID (nlm-ta): Case Rep Med

Journal ID (iso-abbrev): Case Rep Med

Journal ID (publisher-id): CRIM.MEDICINE

Title: Case Reports in Medicine

Publisher: Hindawi Publishing Corporation

ISSN (Print): 1687-9627

ISSN (Electronic): 1687-9635

Publication date (Print): 2013

Publication date (Electronic): 28 July 2013

Volume: 2013

Electronic Location Identifier: 705101

Affiliations

¹Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA

²Department of Radiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA

³Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA

Author notes

*Nowell M. Fine: nowellfine@ 123456gmail.com

Academic Editor: Jagdish Butany

Article

DOI: 10.1155/2013/705101

PMC ID: 3745854

PubMed ID: 23983711

SO-VID: 1f7f884d-5430-4cd0-8f9e-17bf3301de16

License:

This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.