Heterotaxy syndrome (dextrocardia, atrioventricular discordance, pulmonary atresia)

Case contributed by Vincent Tatco
Diagnosis certain


Cyanosis since birth

Patient Data

Age: 3
Gender: Male
  • dextrocardia (morphologic right atrium on the right, morphologic left atrium on the left, morphologic right ventricle with moderator band on the left and morphologic left ventricle on the right) 
  • atrioventricular discordance (morphologic right atrium empties into the morphologic left ventricle while the morphologic left atrium empties into the morphologic right ventricle)
  • ventricular septal defect
  • pulmonary atresia with small confluent pulmonary arteries
  • all pulmonary veins drain into the morphologic left atrium 
  • aorta arising from the morphologic right ventricle with left-sided arch and descending segment
  • major aortopulmonary collateral arteries (MAPCA's)
  • right-sided superior and inferior venae cavae drain into the morphologic right atrium
  • right-sided liver
  • left-sided stomach and spleen

Case Discussion

This is a case of heterotaxy syndrome with complex congenital heart disease

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