Wigle E D, Rakowski H, Ranganathan N, Silver M C
Annu Rev Med. 1976;27:165-80. doi: 10.1146/annurev.me.27.020176.001121.
Mitral valve prolapse is a condition that is being recognized with increased frequency. It is not known whether its incidence is increasing, or whether we are better able to diagnose it today. In the idiopathic or familial variety, the mitral valve pathology is almost always that of myxomatous degeneration. Some authors have suggested the presence of a cardiomyopathy because of significant left ventricular dysfunction in many cases. Idiopathic prolapse occurs predominantly in females, often at a young age, and may be associated with chest pain, dyspnea, fatigue, presyncope, syncope, and/or sudden death. The clinical findings are variable and typically consist of a nonejection click and/or late systolic murmur, heard best at the cardiac apex. Diagnosis can be confirmed by echocardiography and/or ventricular cineangiography, the latter permitting accurate recognition of the anatomy of the prolapsed leaflets. The complications of infective endocarditis, severe mitral insufficiency, and life-threatening ventricular arrhythmias represent the major problems of management. It is important to distinguish the idiopathic form of mitral valve prolapse from that due to coronary artery disease and to realize that mitral valve prolapse may occur in Marfan's syndrome, Turner's syndrome, or in association with secundum atrial septal defect or ruptured chordae tendineae. Typical clicks and/or murmurs have also been described in patients with a history of rheumatic fever and in hypertrophic cardiomyopathy. Although much descriptive knowledge has accumulated over the past 15 years, many unanswered questions remain regarding the idiopathic type of prolapse. What is the nature and cause(s) of myxomatous degeneration? What is the relation of the valve pathology to the left ventricular dysfunction? What is the relation of both of these factors to disabling chest pain, electrocardiographic changes, and life-threatening arrhythmias? Hopefully, answers to these and other important questions regarding mitral valve prolapse will be forthcoming.
二尖瓣脱垂是一种被越来越频繁认识到的病症。目前尚不清楚其发病率是在上升,还是如今我们有了更强的诊断能力。在特发性或家族性类型中,二尖瓣病变几乎总是黏液样变性。一些作者认为,由于许多病例中存在明显的左心室功能障碍,所以存在心肌病。特发性脱垂主要发生在女性,通常是年轻时,可能伴有胸痛、呼吸困难、疲劳、先兆晕厥、晕厥和/或猝死。临床发现各不相同,典型表现为非喷射性喀喇音和/或收缩晚期杂音,在心尖处听得最清楚。超声心动图和/或心室电影血管造影可确诊,后者能准确识别脱垂瓣叶的解剖结构。感染性心内膜炎、严重二尖瓣关闭不全和危及生命的室性心律失常等并发症是主要的治疗问题。区分二尖瓣脱垂的特发性形式与冠状动脉疾病导致的脱垂很重要,并且要认识到二尖瓣脱垂可能发生在马方综合征、特纳综合征中,或与继发孔房间隔缺损或腱索断裂有关。有风湿热病史的患者以及肥厚型心肌病患者也有典型的喀喇音和/或杂音描述。尽管在过去15年里积累了很多描述性知识,但关于特发性脱垂类型仍有许多未解答的问题。黏液样变性的本质和原因是什么?瓣膜病变与左心室功能障碍有什么关系?这两个因素与致残性胸痛、心电图改变和危及生命的心律失常有什么关系?希望关于二尖瓣脱垂的这些及其他重要问题能得到解答。