Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases
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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