Heterotaxy syndrome

Case contributed by Dr Vincent Tatco


Cyanosis since birth

Patient Data

Age: 15
Gender: Female
  • levopositioned heart with the cardiac apex directed towards the left
  • large atrioventricular septal defect
  • pulmonary atresia with small confluent pulmonary arteries
  • all pulmonary veins drain into the left-sided atrium 
  • aorta arising from the morphologic right ventricle with left-sided arch and descending segment
  • major aortopulmonary collateral arteries (MAPCA's)
  • interrupted inferior vena cava with hemiazygos continuation
  • hepatic veins and suprahepatic inferior vena cava draining into the left-sided atrium
  • right-sided superior vena cava draining into the right-sided atrium
  • smaller left-sided superior vena cava draining into the left-sided atrium 
  • bilateral hyparterial bronchi 
  • midline liver
  • right-sided stomach and spleen

Case Discussion

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

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Case information

rID: 41972
Published: 30th Dec 2015
Last edited: 30th Dec 2015
Inclusion in quiz mode: Included

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