KEGG   DISEASE: 修正大血管転位症
エントリ  
H01786                                                             
名称    
修正大血管転位症
概要    
Congenitally corrected transposition of the great arteries is a rare cardiac defect characterized by the combination of atrioventricular (AV) discordance and ventriculo-arterial (VA) discordance. The morphologic left ventricle and mitral valve supply the pulmonary circulation and the morphologic right ventricle and tricuspid valve supply the systemic circulation. The anatomy is described as congenitally 'corrected' because the flow of blood is normal with the deoxygenated systemic venous blood being pumped to the lungs and the well oxygenated pulmonary venous blood being pumped to the body. More than 90% of patients have additional anatomical abnormalities. The most frequent are ventricular septal defects, left ventricular outflow tract obstruction, and abnormalities of the tricuspid valve.
カテゴリ  
先天奇形
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  主に1つの体系に影響する構造的発達異常
   循環器系の構造的発達異常
    心臓または大血管の構造的発達異常
     LA85  房室または心室大血管結合の先天異常
      H01786  修正大血管転位症
指定難病 [jp08407.html]
 H01786
リンク   
ICD-11: LA85.0
MeSH: C535426
文献    
  著者
Hornung TS, Calder L
  タイトル
Congenitally corrected transposition of the great arteries.
  雑誌
Heart 96:1154-61 (2010)
DOI:10.1136/hrt.2008.150532
文献    
  著者
McEwing RL, Chaoui R
  タイトル
Congenitally corrected transposition of the great arteries: clues for prenatal diagnosis.
  雑誌
Ultrasound Obstet Gynecol 23:68-72 (2004)
DOI:10.1002/uog.896
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