Marcus R H, Sareli P, Pocock W A, Barlow J B
University of Witwatersrand, Johannesburg, South Africa.
Ann Intern Med. 1994 Feb 1;120(3):177-83. doi: 10.7326/0003-4819-120-3-199402010-00001.
To describe the demographic, pathologic, and hemodynamic profiles of patients with severe rheumatic mitral valve disease in a developing country and to assess their relation to uncontrolled rheumatic disease activity.
Retrospective, cross-sectional, cohort study.
Tertiary medical center in Soweto, South Africa.
714 of 737 consecutive black patients, 4 to 73 years old, with pure mitral regurgitation, pure mitral stenosis, or mixed mitral disease who had mitral valve surgery and in whom preoperative and surgical data were concordant.
Valve lesions were evaluated on the basis of clinical, echocardiographic, hemodynamic, and surgical pathologic data. Active rheumatic carditis was diagnosed according to clinical evidence for concurrent acute rheumatic fever (Jones criteria), macroscopic appearances at surgery, and histologic findings.
219 patients had pure mitral regurgitation, 275 had pure mitral stenosis, and 220 had mixed lesions. Ongoing rheumatic activity was diagnosed in 106 patients with pure regurgitation (47%) and in only 5 patients with pure stenosis (2%). Pure regurgitation was the most common lesion in the first and second decades; the relative prevalence of pure stenosis increased with age. Purely regurgitant valves had pliable, unscarred leaflets (95%), dilated mitral annuli (95%), elongated chordae tendineae (92%), and anterior leaflet prolapse (81%). In contrast, purely stenotic valves had fused leaflet commissures (100%) and rigid leaflets (38%) but no evidence of prolapse.
The spectrum of rheumatic mitral valve disease that is hemodynamically severe in developing countries differs from that currently reported in the United States. Severe, pure rheumatic mitral regurgitation is as prevalent as pure stenosis but has an entirely different time course, surgical anatomy, and relation to disease activity, suggesting a separate pathophysiologic mechanism.
描述一个发展中国家严重风湿性二尖瓣疾病患者的人口统计学、病理学和血流动力学特征，并评估其与未控制的风湿疾病活动的关系。
回顾性横断面队列研究。
南非索韦托的三级医疗中心。
737例连续的4至73岁黑人患者中的714例，这些患者患有单纯二尖瓣反流、单纯二尖瓣狭窄或混合性二尖瓣疾病，接受了二尖瓣手术，且术前和手术数据一致。
根据临床、超声心动图、血流动力学和手术病理数据评估瓣膜病变。活动性风湿性心内膜炎根据并发急性风湿热的临床证据（琼斯标准）、手术时的宏观表现和组织学发现进行诊断。
219例患者为单纯二尖瓣反流，275例为单纯二尖瓣狭窄，220例为混合性病变。106例单纯反流患者（47%）被诊断为有持续的风湿活动，而单纯狭窄患者中只有5例（2%）。单纯反流是第一和第二个十年中最常见的病变；单纯狭窄的相对患病率随年龄增加。单纯反流的瓣膜有柔韧、无瘢痕的瓣叶（95%）、扩大的二尖瓣环（95%）、延长的腱索（92%）和前叶脱垂（81%）。相比之下，单纯狭窄的瓣膜有融合的瓣叶交界（100%）和僵硬的瓣叶（38%），但没有脱垂的证据。
在发展中国家，血流动力学严重的风湿性二尖瓣疾病谱与美国目前报道的不同。严重的单纯风湿性二尖瓣反流与单纯狭窄一样普遍，但有完全不同的病程、手术解剖结构以及与疾病活动的关系，提示存在单独的病理生理机制。
