罕见病知识库 RareSeen

先天性左心室室壁瘤

Congenital left ventricular aneurysm

ORPHA:1055疾病

定义 英文原文(暂无中文)

A rare congenital non-syndromic heart malformation characterized by a bulging of the left ventricular wall, connected to the left ventricle by a wide neck (with a ratio of the connection to the body of the anomaly >1). The dimensions of aneurysms have been described as small as 0.5 cm in diameter and as big as 8x9 cm in size. Most frequent locations are the left ventricular apex and the perivalvular area. Aneurysms can be a- or dyskinetic or show almost normal contractility. Patients may remain asymptomatic or present with systemic embolization, congestive heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia, or sudden cardiac death.

基本事实

发病年龄
产前、新生儿期

临床表型 6

必现 100%1

  • 左心室形态异常 HP:0001711

常见 79–30%1

  • 充血性心力衰竭 HP:0001635

偶见 29–5%4

  • ST段异常 HP:0012249
  • T波异常 HP:0005135
  • 呼吸暂停 HP:0002104
  • 心律失常 HP:0011675

外部标识与链接

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本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)