Elsevier

American Heart Journal

Volume 146, Issue 3, September 2003, Pages 404-410
American Heart Journal

Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases

https://doi.org/10.1016/S0002-8703(03)00249-7Get rights and content

Abstract

Background

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.

Data collection

Cases, case series and related articles on the subject in all languages were identified through a comprehensive literature search.

Results and conclusions

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.

Section snippets

Data collection

A systematic search of the literature using MEDLINE database (National Library of Medicine, Bethesda, Md) was performed to identify all relevant articles using the following key words: cardiac papillary fibroelastoma, papillary fibroelastoma, cardiac valve tumors, primary cardiac tumors, papillary growths of heart, fibroelastoma, giant Lambl excrescences, and valvular excrescences. The references of the articles were examined for additional cases. All foreign language articles were translated

Results

All 725 cases described in this report had histopathological confirmation of CPF by complete excision or examination at autopsy. Sex data were accurately available in 517 patients. Among these, 286 patients (55%) were male and 231 were female (45%). Ten were children aged <10 years. Age prevalence was accurately available in only 296 patients, and it was highest in the 8th decade of life (Figure 1). The youngest patient reported was a newborn child and the oldest was 92 years of age. No clear

Historical perspective

Yater,6 in 1931, was the first to describe the valvular tumors, based on 25 previous reports and 2 new cases. In 1934, Campbell and Carling7 gave a description of a patient with sudden death related to the tumor located on the aortic valve. In 1975, Cheitlin et al 29 used the term ‘papillary fibroelastoma’ for the first time, and in the same year, Fishbein et al30 described ultrastructural and electron microscopic characteristics of CPF. Anderson et al,33 in 1977, reported the first and the

Conclusions

Although CPF is a histologically benign tumor, the clinical course could be devastating because of its strategic position, which includes systemic or pulmonary embolism and death. Echocardiography, particularly transesophageal echocardiography, complemented by cardiac magnetic resonance imaging, provides the degree of structural resolution necessary to ascertain the location and the extent of anatomic and hemodynamic involvement. The ultimate diagnosis is based on the characteristic

Acknowledgements

We thank Rachid Daoui, MD, Darmadi Khong, MD, Braulio Cosme-Thornmann, MD, Ketan Jani, MD, and Nilesh Patel, MD for translating the foreign language articles.

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