Chomette G, Auriol M, Labrousse F, Tranbaloc P
Service d'anatomie pathologique, hôpital de la Pitié, Paris.
Arch Mal Coeur Vaiss. 1990 Jun;83(7):929-33.
The authors studies 154 cases of degenerative aortic regurgitation which presented macroscopically with atrophic changes of the valve and sometimes with hypertrophy and calcification. Histologically, the essential abnormality was the finding of mucopolysaccharide deposits dissociating the corpus spongiosa from the corpus fibrosa. Depending on the importance of these lesions, three degrees of severity can be defined, the most extensive (84% of our patient population) appearing to be typical of the disease. In addition, mild mitral valve prolapse (5%) and medial necrosis of the aortic wall (80% of patients undergoing aortic biopsy) were observed. These morphological features are on the whole quite different to those of other aortic valve pathologies (rheumatic, endocarditis). However, the border line with other pathologies with a similar anatomopathological substratum is less clearly defined: genetic abnormalities (Marfan's syndrome, Lobstein's disease, etc...) or age-related degenerative disease. The pathogenesis is not clearly understood but could be related to regional disturbances in collagen metabolism with collagenolysis predominating.
作者研究了154例退行性主动脉瓣关闭不全病例,这些病例在宏观上表现为瓣膜萎缩性改变,有时伴有肥厚和钙化。组织学上,主要异常是发现粘多糖沉积物使海绵体与纤维体分离。根据这些病变的严重程度,可以定义三个等级,最广泛的病变(占我们患者群体的84%)似乎是该疾病的典型表现。此外,还观察到轻度二尖瓣脱垂(5%)和主动脉壁中层坏死(接受主动脉活检的患者中有80%)。这些形态学特征总体上与其他主动脉瓣病变(风湿性、心内膜炎)有很大不同。然而,与具有相似解剖病理学基础的其他病变的界限定义不太明确:遗传异常(马凡综合征、洛布斯坦病等)或与年龄相关的退行性疾病。发病机制尚不清楚,但可能与以胶原溶解为主的胶原代谢局部紊乱有关。