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评估二尖瓣脱垂进展风险中的家族史。一个家族的报告。

Family history in assessing the risk for progression of mitral valve prolapse. Report of a kindred.

作者信息

Cooper M J, Abinader E G

出版信息

Am J Dis Child. 1981 Jul;135(7):647-9. doi: 10.1001/archpedi.1981.02130310051017.

Abstract

Mitral valve prolapse (MVP) consists of prolapse or billowing of the mitral leaflet(s) into the left atrium during ventricular systole associated with a mid-to-late systolic click and/or a late systolic murmur. Thoracic skeletal abnormalities, progressive mitral regurgitation, ventricular arrhythmia, and sudden death have been associated with an autosomal dominant "MVP syndrome." The discovery of MVP in an asymptomatic 8-year-old boy led to a family investigation with subsequent delineation of age-related, progressive, mitral valve disease through four generations of the kindred. Although generally considered a benign syndrome, obtaining a full family history is important in assessing the risk for progression of mitral valve disease in affected family members.

摘要

二尖瓣脱垂(MVP)是指在心室收缩期二尖瓣叶脱垂或膨入左心房,伴有收缩中期至晚期喀喇音和/或收缩晚期杂音。胸廓骨骼异常、进行性二尖瓣反流、室性心律失常和猝死与常染色体显性“MVP综合征”相关。在一名无症状的8岁男孩中发现MVP后,对其家族进行了调查,随后在该家族的四代人中明确了与年龄相关的、进行性的二尖瓣疾病。尽管MVP通常被认为是一种良性综合征,但全面了解家族病史对于评估受影响家庭成员中二尖瓣疾病进展的风险非常重要。

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