Heterotaxy syndrome

Case contributed by Vincent Tatco
Diagnosis certain

Presentation

Cyanosis since birth

Patient Data

Age: 5
Gender: Female
ct
  • levopositioned heart with the cardiac apex directed towards the left
  • large atrioventricular septal defect
  • aorta and main pulmonary trunk are side by side and both are arising from the morphologic right ventricle (double outlet right ventricle)
  • interrupted inferior vena cava with azygos continuation
  • midline liver
  • polysplenia
  • right-sided stomach

Case Discussion

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

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