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Complex congenital heart disease with heterotaxy

Case contributed by Dr Mohammad A. ElBeialy


Follow-up of a post-operative case of congenital heart disease.

Patient Data

Age: 5 years
Gender: Female


  • left atrial isomerism, apex to the right
  • bronchi have left bronchus pattern; both lungs are bilobed
  • atrioventricular concordance
  • large VSD
  • single atrium
  • both atria attached to the right and left ventricles by a single valve
  • right atrial appendage to the left of common atrium while left atrial appendage to the right of common atrium
  • aorta arises from the RV just anterior and to the right


  • aortic root appears to be rotated clockwise
  • left sided aortic arch gives three main branches
  • descending aorta is seen running in the left paravertebral course
  • no evident abdominal aortic coarctation nor PDA
  • minute MAPCAs to both lungs
  • RCA arises from the left side of the aorta, and the LM arises from the right side of the aorta; both vessels run a normal course

Pulmonary arteries and veins

  • confluent pulmonary artery branches
  • atretic main pulmonary artery
  • left SVC supplies both RPA and LPA; the azygos vein supplies the LPA only
  • LPA has a patent stent starting from its origin in the main pulmonary artery (MPA) to the first segmental branch. It includes the site of anastomosis of the left SVC with the LPA and azygos vein insertion in LPA
  • normal pulmonary veins drain into the left side of the common atrium

Venae cava and innominate veins

  • left SVC only (absent right SVC)
  • IVC is interrupted
  • hepatic veins drain in the right of the common atrium separately

Abdominal: situs ambiguous and polysplenia

Case Discussion

Complex congenital heart disease with heterotaxy: left isomerism, IVC interruption, pulmonary atresia, absent right SVC, persistent left SVC, left sided Kawashima, successfully dilated main pulmonary arteries.

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Case information

rID: 42318
Published: 3rd Jul 2017
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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