Heterotaxy syndrome (dextrocardia, atrioventricular discordance, pulmonary atresia)

Case contributed by Dr Vincent Tatco

Presentation

Cyanosis since birth

Patient Data

Age: 10 months
  • 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
  • small confluent pulmonary arteries supplied by a patent ductus arteriosus and major aortopulmonary collateral arteries (MAPCA's)
  • all pulmonary veins drain into the morphologic left atrium 
  • aorta arising from the morphologic right ventricle with left-sided arch and descending segment
  • right-sided superior and inferior venae cavae drain into the morphologic right atrium
  • right-sided liver
  • left-sided stomach and spleen
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Case Information

rID: 41981
Case created: 30th Dec 2015
Last edited: 3rd Jan 2016
System: Paediatrics
Inclusion in quiz mode: Included

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