Key result
Rare pulmonary valve papillary fibroelastomas present incidentally or cause dyspnea, emboli, or RVOT obstruction.
Why the study?
Pulmonary valve papillary fibroelastoma is rare and its demographic characteristics, clinical presentation, management, and outcomes are not well characterized.
Systematic Review
This systematic review aims to characterize the demographics, presentation, management, and outcomes of rare pulmonary valve papillary fibroelastomas.
Answers are grounded in this paper and cite their sources.
Warrants consideration of pulmonary valve papillary fibroelastoma in dyspnea or embolic events; extends systematic echocardiographic data on these rare tumors.
The pulmonary valve is the least affected site for valvular papillary fibroelastoma. With increasing use of routine echocardiography and other modalities of imaging, pulmonary valve papillary fibroelastomas (PVPFE) are being recognized more frequently. PVPFE is more often an incidental diagnosis and symptomatic patients usually present with shortness of breath. Embolic phenomena and right ventricular outflow tract obstruction are the most serious complications of PVPFE. Since PVPFE is rare, the purpose of this systematic review is to address demographic characteristics, the clinical presentation, management, and outcome of this benign tumor of the pulmonary valve.
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Hakim et al. (2013) conducted a systematic review in Pulmonary valve papillary fibroelastoma. Pulmonary valve papillary fibroelastoma was evaluated on Demographic characteristics, clinical presentation, management, and outcome. Pulmonary valve papillary fibroelastomas are rare, often incidentally diagnosed benign tumors that can present with shortness of breath, embolic phenomena, or right ventricular outflow tract obstruction.
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