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Papillary fibroelastomas : systemic embolism and other clinical features


Sang Thrombose Vaisseaux. Volume 12, Number 5, 296-304, Mai 2000, Mini-revues

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Author(s) : Claude Gully, Mohamed Mounir Benghanem, Driss Mouhoub, Ariel Cohen, Christine Sagan

Summary : These benign tumours of the endocardium may be distinguished from Lambl’s excrescences by their localisation and size. Previous reports suggest that they might correspond to giant Lambl’s excrescences and are always present after 10 years of age. Papillary fibroelastoma is usually detected by echocardiography and there have been about 60 case reports in the literature. They are essentially cardiac valve tumours (73% of valve tumours) and may give rise to severe symptoms, such as sudden death by migration or coronary obstruction and systemic embolism, especially from left heart lesions. However, they can be located at any point of the endocardium. The diagnosis of a valvular or an endocardial tumour is based on echocardiography and especially on transoesophagial echocardiography which often provides a more accurate aetiological diagnosis. In symptomatic tumours, surgery (usually simple ablation) is indicated with a low operative risk and complete resolution of symptoms. Tumours discovered by chance pose difficult problems of management and may lead to diagnostic or preventive surgery.

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