Tokunaga K, Furuta S, Machii K, Takanashi R
J Cardiogr. 1985 Sep;15(3):829-46.
Recently, the frequency of nonrheumatic aortic regurgitation (AR) has apparently increased, accompanied by a decrease in frequency of rheumatic fever. The purpose of the present study was to ascertain the echocardiographic features of nonrheumatic AR. We had 24 surgically- or autopsy-proven cases of nonrheumatic AR admitted during a two year period. These were 10 cases of infective endocarditis (IE), five with ventricular septal defect of type I, three with syphilis, and two with prosthetic valve malfunctions, and the remainder five were difficult to diagnose clinically. These five were three men and two women, whose ages ranged from 40 to 67 years and averaged 50 years, and their final diagnoses were annulo-aortic ectasia (AAE), Behcet's disease, and the aortitis syndrome (Takayasu's arteritis), and two other cases were of unknown etiology. The echocardiographic manifestations were compared with the operative, autopsy, and pathological findings. Echocardiographically, there were few or no increased intensities of aortic valvular echoes, and aortic roots had a tendency to dilate, leading to the failure of coaptation of valve leaflets, for a relative lack of valvular surface area to cross-sectional area of the aortic ring. Three of the five had flail aortic valves and three had associated MVP. Three were diagnosed as floppy aortic valves at the time of surgery. Excised valves revealed little hyperplasia or sclerosis grossly. Fibrinoid necrosis or mucoid degeneration were noted by light microscopy. Some specimens of aortic walls also revealed cystic medial necrosis or disruption of elastic fibers. All these findings were based on degenerative processes of connective tissue, and not on inflammatory processes. These pathological findings and the coexistence of mitral valve prolapse (MVP), which were not regarded as coincidental, suggest that connective tissue fragility--congenital or acquired--may play an important role in the genesis of nonrheumatic AR.
最近，非风湿性主动脉瓣关闭不全（AR）的发病率明显上升，同时风湿热的发病率有所下降。本研究的目的是确定非风湿性AR的超声心动图特征。我们有24例经手术或尸检证实的非风湿性AR病例，这些病例是在两年期间收治的。其中10例为感染性心内膜炎（IE），5例为I型室间隔缺损，3例为梅毒，2例为人工瓣膜功能障碍，其余5例临床诊断困难。这5例患者中3例为男性，2例为女性，年龄在40至67岁之间，平均年龄为50岁，最终诊断为主动脉瓣环扩张（AAE）、白塞病和主动脉炎综合征（高安动脉炎），另外2例病因不明。将超声心动图表现与手术、尸检和病理结果进行了比较。超声心动图显示，主动脉瓣回声强度很少增加或无增加，主动脉根部有扩张趋势，导致瓣叶对合不良，这是由于瓣膜表面积与主动脉环横截面积相比相对不足。5例中有3例有连枷样主动脉瓣，3例合并二尖瓣脱垂（MVP）。3例在手术时被诊断为松弛性主动脉瓣。切除的瓣膜大体上显示增生或硬化不明显。光镜下可见纤维素样坏死或黏液样变性。一些主动脉壁标本还显示中层囊性坏死或弹性纤维断裂。所有这些发现均基于结缔组织的退行性变过程，而非炎症过程。这些病理发现以及二尖瓣脱垂（MVP）的并存并非偶然，提示先天性或后天性结缔组织脆弱性可能在非风湿性AR的发病机制中起重要作用。
