迄今为止,风湿性心脏病 (RHD) 是美国和全世界三尖瓣狭窄 (TS) 最常见的病因。在发达国家,急性风湿热 (ARF) 在过去 40-50 年的相对发病率已显著降低;而在发展中国家 ARF 和风湿性心脏病的负担依然较高。美国 ARF 的年发病率估计值为每 10 万人 0.2-0.8 例,苏丹为每 10 万人 100 例,中国为每 10 万人 150 例。[4]World Health Organization. Rheumatic fever and rheumatic heart disease. Technical Report Series 923. February 2004. http://www.who.int/ (last accessed 6 June 2017).http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf[5]World Health Organization. Joint WHO/ISFC meeting on rheumatic fever/rheumatic heart disease control, with emphasis on primary prevention. Geneva: 7-9 September 1994. http://www.who.int/en/ (last accessed 6 June 2017).http://whqlibdoc.who.int/hq/1994/WHO_CVD_94.1.pdf慢性风湿性心脏病的总患病率在美国较低,据估计每 1000 人出现 0.05 例。社会经济学和环境因素,比如资源短缺、缺少医疗保健提供者以及认识水平低,都是发展中国家 ARF 和风湿性心脏病持续高发的原因。在诸如美国的发达国家中,据估计高达 50% 曾患 ARF 和心肌炎的人士会罹患慢性风湿性心脏病。[6]Horstkotte D, Niehues R, Strauer BE. Pathomorphological aspects, aetiology and natural history of acquired mitral valve stenosis. Eur Heart J. 1991;12(suppl B):55-60.http://www.ncbi.nlm.nih.gov/pubmed/1936027?tool=bestpractice.com[7]Majeed HA, Batnager S, Yousof AM, et al. Acute rheumatic fever and the evolution of rheumatic heart disease: a prospective 12 year follow-up report. J Clin Epidemiol. 1992;45:871-875.http://www.ncbi.nlm.nih.gov/pubmed/1624969?tool=bestpractice.com单纯三尖瓣受累的情况是极为罕见的,临床 TS 通常伴有二尖瓣和主动脉瓣疾病。[8]Hauck AJ, Freeman DP, Ackermann DM, et al. Surgical pathology of the tricuspid valve: a study of 363 cases spanning 25 years. Mayo Clin Proc. 1988;63:851-863.http://www.ncbi.nlm.nih.gov/pubmed/3045434?tool=bestpractice.com[1]Roguin A, Rinkevich D, Milo S, et al. Long-term follow-up of patients with severe rheumatic tricuspid stenosis. Am Heart J. 1998;136:103-108.http://www.ncbi.nlm.nih.gov/pubmed/9665226?tool=bestpractice.com[9]Raman SV, Sparks EA, Boudoulas H, et al. Tricuspid valve disease: tricuspid valve complex perspective. Curr Probl Cardiol. 2002;27:103-142.http://www.ncbi.nlm.nih.gov/pubmed/11979238?tool=bestpractice.com据估计,风湿性心脏病患者的 TS 总体发病率为 9%-15%,具有显著的临床意义和更为严重的情况仅占 5%。[1]Roguin A, Rinkevich D, Milo S, et al. Long-term follow-up of patients with severe rheumatic tricuspid stenosis. Am Heart J. 1998;136:103-108.http://www.ncbi.nlm.nih.gov/pubmed/9665226?tool=bestpractice.com[10]Essop MR, Nkomo VT. Rheumatic and nonrheumatic valvular heart disease: epidemiology, management, and prevention in Africa. Circulation. 2005;112:3584-3591.http://circ.ahajournals.org/cgi/content/full/112/23/3584http://www.ncbi.nlm.nih.gov/pubmed/16330700?tool=bestpractice.com[11]Goswami KC, Rao MB, Dev V, et al. Juvenile tricuspid stenosis and rheumatic tricuspid valve disease: an echocardiographic study. Int J Cardiol. 1999;72:83-86.http://www.ncbi.nlm.nih.gov/pubmed/10636636?tool=bestpractice.com[12]Yousof AM, Shafei MZ, Endrys G, et al. Tricuspid stenosis and regurgitation in rheumatic heart disease: a prospective cardiac catheterization study in 525 patients. Am Heart J. 1985;110:60-64.http://www.ncbi.nlm.nih.gov/pubmed/4013991?tool=bestpractice.com[13]Kitchin A, Turner R. Diagnosis and treatment of tricuspid stenosis. Br Heart J. 1964;26:354-379.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1018152/pdf/brheartj00344-0066.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/14156086?tool=bestpractice.com这引出了对美国风湿性 TS 患病率为每 1000 人 0.003 例的总体估计。ARF 的男性发病率和女性发病率持平;但女性更加通常倾向于发生严重的风湿性 TS。美国唯一表现出相对较高的 ARF 和风湿性心脏病发病率的种族是居住在夏威夷的波利尼西亚裔。
类癌心脏病的发病率约为每 30 万人 1 例。[14]Bassan MD, Ahlman H, Wangberg B, et al. Biology and management of the midgut carcinoid syndrome. Am J Surg. 1993;165:288-297.http://www.ncbi.nlm.nih.gov/pubmed/8427415?tool=bestpractice.com[15]Fox DJ, Khattar RS. Carcinoid heart disease: presentation, diagnosis, and management. Heart. 2004;90:1224-1228.http://www.ncbi.nlm.nih.gov/pubmed/15367531?tool=bestpractice.com
先天性 TS 的发病率很低,不到 0.1%。