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Dextrocardia

Disease definition

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.

ORPHA:1666

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Neonatal, Infancy
  • ICD-10: Q24.0
  • ICD-11: LA80.1
  • OMIM: -
  • UMLS: C0011813
  • MeSH: D003914
  • GARD: 1827
  • MedDRA: 10012592
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