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右位心

Dextrocardia

ORPHA:1666疾病

定义 英文原文（暂无中文）

A rare, congenital, non-syndromic, developmental defect during embryogenesis characterized by positioning of the heart in the right hemithorax, with the base and apex of the heart pointing caudally and to the right, due to abnormalities of embryologic origin that are intrinsic to the heart itself. Situs inversus or situs solitus may be associated, with extracardiac visceral transposition anomalies usually present in the former case and additional cardiac defects (e.g. septal defects, transposition of the great arteries, double-outlet right ventricle, anomalous pulmonary venous return, tetralogy of Fallot) frequently observed in both cases.

基本事实

发病年龄
婴儿期、新生儿期

临床表型 26

必现 100%1

  • 右位心 HP:0001651

极常见 99–80%3

  • 异常心电图 HP:0003115
  • 心脏形态异常 HP:0001627
  • T波倒置 HP:0010872

常见 79–30%2

  • 先天性大动脉畸形 HP:0011603
  • 全内脏反位 HP:0001696

偶见 29–5%9

  • 肺分叶异常 HP:0002101
  • 肾形态异常 HP:0012210
  • 生殖系统形态异常 HP:0012243
  • 腹部位置异常 HP:0011620
  • 肺位置形态学异常 HP:0011615
  • 肺动脉异常 HP:0004414
  • 脾脏异常 HP:0001743
  • 输尿管异常 HP:0000069
  • 肠旋转不良 HP:0002566

罕见 <4–1%11

  • 足部形态异常 HP:0001760
  • 肋骨形态异常 HP:0000772
  • 耳发育缺陷/不全 HP:0008771
  • 先天性髋关节脱位 HP:0001374
  • 全面发育迟缓 HP:0001263
  • 脑积水 HP:0000238
  • Meckel憩室 HP:0002245
  • 神经母细胞瘤 HP:0003006
  • 胰腺发育不良 HP:0002594
  • 耳前皮赘 HP:0000384
  • 蹼颈 HP:0000465

外部标识与链接

OrphanetMONDO:0015661GARD:1827ICD-10 Q24.0ICD-11 LA80.1ClinicalTrials.gov 检索

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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）