Bellitti R, Caruso A, Festa M, Mazzei V, Iesu S, Falco A, Cotrufo M, Agozzino L
Int J Cardiol. 1985 Dec;9(4):399-412. doi: 10.1016/0167-5273(85)90234-7.
A clinico-pathologic study was performed in 25 patients undergoing aortic valve replacement because of regurgitation, caused by myxoid degeneration of the valve leaflets. Associated cardiac anomalies were floppy mitral valve (2 cases), floppy mitral valve and idiopathic hypertrophic subaortic stenosis (1), left atrial myxoma (1), and aortic coarctation at the isthmus (1). Three patients died (2 immediately and 1 on the 30th postoperative day). Pathological studies of the explanted valves showed deformities characterized by redundant thin leaflets which appeared soft and gelatinous. On histologic examination the fibrous layer of the leaflets was seen to be infiltrated by myxomatous tissue. Echocardiography showed the aortic root to be dilated in 13 patients and normal in the others. In those with normal aortic root, the histological examination of aortic wall disclosed minimal cystic medial necrosis in two cases. In contrast, more severe forms of cystic medial necrosis were evident in all patients having a dilated aortic root. Aortic valve replacement was performed in all cases. It was accompanied by a Bentall procedure (1 case), repair of ascending aorta dissection (2), replacement of the ascending aorta (1), mitral valve replacement (2), mitral valve replacement and apico-ascending aorta conduit (1) and excision of a left atrial myxoma (1). Our experience suggests that prolapse of the aortic valve due to floppy leaflets is a common degenerative disease which is generally associated with noninflammatory aortic root degeneration. This, together with aortic root dilatation, contributes to valve insufficiency. Nevertheless, the disease, when isolated (with normal aortic root), is liable in itself to produce aortic regurgitation. The need for early diagnosis is stressed, so as to be able to perform valve replacement.
对25例因瓣叶黏液样变性导致反流而接受主动脉瓣置换术的患者进行了临床病理研究。相关的心脏异常包括二尖瓣脱垂（2例）、二尖瓣脱垂合并特发性肥厚性主动脉瓣下狭窄（1例）、左房黏液瘤（1例）以及峡部主动脉缩窄（1例）。3例患者死亡（2例术中即刻死亡，1例术后第30天死亡）。切除瓣膜的病理研究显示，瓣膜畸形表现为瓣叶冗长、菲薄，质地柔软、呈胶冻状。组织学检查发现瓣叶纤维层被黏液瘤样组织浸润。超声心动图显示，13例患者主动脉根部扩张，其余患者正常。主动脉根部正常的患者中，2例主动脉壁组织学检查显示有轻微的囊性中层坏死。相反，所有主动脉根部扩张的患者囊性中层坏死更为严重。所有病例均行主动脉瓣置换术，同时进行了1例Bentall手术、2例升主动脉夹层修复、1例升主动脉置换、2例二尖瓣置换、1例二尖瓣置换及心尖 - 升主动脉管道手术以及1例左房黏液瘤切除术。我们的经验表明，瓣叶脱垂导致的主动脉瓣脱垂是一种常见的退行性疾病，通常与非炎性主动脉根部退变有关。这与主动脉根部扩张一起，导致瓣膜关闭不全。然而，该病单独存在（主动脉根部正常）时，本身也易导致主动脉反流。强调了早期诊断的必要性，以便能够进行瓣膜置换术。
