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未经矫治的法洛四联症:61岁成人病例报告

Uncorrected tetralogy of Fallot: adult presentation in the 61st year of life.

作者信息

Fairley Sarah L, Sands Andrew J, Wilson Carol M

出版信息

Int J Cardiol. 2008 Aug 1;128(1):e9-e11. doi: 10.1016/j.ijcard.2007.04.155. Epub 2007 Aug 8.

DOI:10.1016/j.ijcard.2007.04.155
PMID:17689735
Abstract

Tetralogy of Fallot (TOF) is the commonest form of cyanotic congenital heart defect after infancy [Brickner ME, Hillis LD, Lange RA. Congenital Heart Disease in Adults-Second of Two Parts. NEJM 2000; 342(5):334-342.]. There are few studies assessing the risk of surgical correction in adult patients and long-term survival into the fourth decade of life is rare. The case history is described of a 61-year old female presenting with probable viral myocarditis. Subsequent investigations revealed an underlying diagnosis of tetralogy of Fallot. The patient remains asymptomatic despite persistent hypoxia. Potential factors contributing to longevity in this case are relatively good pulmonary blood flow via large branch pulmonary arteries, and the possible gradual development of right ventricular outflow tract obstruction over a long time period.

摘要

法洛四联症(TOF)是婴儿期后最常见的青紫型先天性心脏病[布里克纳ME,希利斯LD,兰格RA。成人先天性心脏病——两部分中的第二部分。《新英格兰医学杂志》2000年;342(5):334 - 342。]。评估成年患者手术矫正风险的研究较少,活到四十多岁的长期生存情况很罕见。本文描述了一名61岁女性的病例史,该女性最初表现为可能的病毒性心肌炎。后续检查发现其潜在诊断为法洛四联症。尽管持续存在缺氧情况,但该患者仍无症状。该病例中有助于长寿的潜在因素包括通过大的肺分支动脉有相对良好的肺血流量,以及右心室流出道梗阻可能在很长一段时间内逐渐发展。

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