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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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version April 2024

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