Heterotaxy syndrome (left isomerism with Taussig-Bing anomaly)

Case contributed by Vincent Tatco
Diagnosis certain

Presentation

Cyanosis since birth

Patient Data

Age: 1 year
Gender: Male
  • mesocardia
  • morphologic right ventricle on the right and morphologic left ventricle on the left
  • left atrial isomerism with atrial septal defect
  • right pulmonary veins drain into the right-sided atrium
  • left pulmonary veins, left superior vena cava and left suprahepatic inferior vena cava drain into the left-sided atrium
  • double outlet right ventricle with subpulmonic ventricular septal defect (Taussig-Bing anomaly)
  • right-sided aorta
  • left-sided suprahepatic inferior vena cava and right-sided infrahepatic inferior vena cava 
  • bilateral hyparterial bronchi 
  • midline liver
  • polysplenia
  • left-sided stomach

Case Discussion

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

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