首页 > 期刊杂志 > 正文

Sudden cardiac death: a modern pathology approach to hypertrophic cardiomyopathy.

心源性猝死:用现代病理学方法研究肥厚型心肌病

Abstract

Hypertrophic cardiomyopathy is one of the most common causes of sudden cardiac death among young adults and adolescents. Unfortunately, the first manifestation of the condition may be sudden death during exertion, such as sporting activities. Other clinical symptoms include exertional dyspnea, angina, and syncope. Postmortem examination often reveals asymmetrical septal thickening and mural plaque formation in the left ventricular outflow tract. Histologic analysis shows cardiac myocyte hypertrophy, myofiber disarray, and interstitial and replacement fibrosis. Molecular analysis for known genetic abnormalities is essential to genetic counseling of living relatives of decedents to assess and reduce the risk of sudden cardiac death from hypertrophic cardiomyopathy.

摘要

肥厚型心肌病是年轻成年人和青少年心源性猝死的最常见原因之一。不幸的是,该病的首发表现可能是体育运动等剧烈活动时猝死。其它临床症状包括劳力性呼吸困难、心绞痛和和晕厥。尸检多见非对称性室间隔增厚和左心室流出道壁斑块形成。组织学分析显示心肌细胞肥大、肌纤维排列紊乱、间质纤维化和被纤维化所取代。为评估和减少死者亲属肥厚型心肌病所致心源性猝死的遗传咨询中,有必要对已知的遗传异常进行分子检测。

full text

我要评论

0条评论