Gully C, Benghanem M M, Motebassem R, Sagan C
Service de cardiologie, Centre hospitalier départemental, La Roche-sur-Yon.
Arch Mal Coeur Vaiss. 1998 Jun;91(6):777-82.
The authors review the literature of the clinical features of papillary fibroelastomas in the light of a new case. These benign tumours of the endocardium may be distinguished from Lambl's vegetations by their site and size. Some workers suggest that they correspond to giant Lambl's vegetation and could be a form of "aging" of the valvular endocardium. Nevertheless, Lambl's vegetations are always present after 10 years of age but the papillary fibroelastoma is rarely detected by echocardiography and there have been few case reports. They are essentially cardiac valve tumours (73% of valvular tumours) and may give rise to serious clinical symptoms, sudden death by migration or coronary obstruction, systemic embolism, especially from left heart lesions. However, they can be situated at any point of the endocardium. The diagnosis of a valvular or an endocardial tumour is based on echocardiography which, though not always accurate, gives a better aetiological diagnosis. In cases of symptomatic tumours, surgery (usually simple ablation) is indicated with a low operative risk and cure of symptoms. Tumours discovered by chance pose very difficult problems of management and may lead to diagnostic or preventive surgery.
作者结合一个新病例回顾了乳头肌弹性纤维瘤临床特征的文献。这些心内膜良性肿瘤可通过其部位和大小与兰布尔赘生物相鉴别。一些研究者认为它们相当于巨大的兰布尔赘生物，可能是瓣膜心内膜“老化”的一种形式。然而，兰布尔赘生物在10岁以后总是存在，但乳头肌弹性纤维瘤很少通过超声心动图检测到，病例报告也很少。它们主要是心脏瓣膜肿瘤（占瓣膜肿瘤的73%），可能会引起严重的临床症状，因迁移或冠状动脉阻塞导致猝死、系统性栓塞，尤其是来自左心病变。然而，它们可位于心内膜的任何部位。瓣膜或心内膜肿瘤的诊断基于超声心动图，虽然并不总是准确，但能给出更好的病因诊断。对于有症状的肿瘤，建议进行手术（通常为单纯切除术），手术风险低且症状可治愈。偶然发现的肿瘤带来了非常棘手的管理问题，可能导致诊断性或预防性手术。
