Multiple ventricular septal defects - Goldenhar syndrome

Case contributed by Fabien Ho
Diagnosis certain


Known Goldenhar syndrome. Perimembranous VSD + multiple muscular VSDs. MPA strapping at 2 months of age. Single kidney.

Patient Data

Age: 18 years
Gender: Female

Multiple left to right shunts:

  • large perimembranous inlet VSD 21 x 17 mm
  • large muscular VSD ahead of the moderator band 24 x 21 mm
  • midventricular small VSD 5 x 5mm
  • midventricular small VSD 3mm

Enlarged hepatic veins with contrast reflux , enlarged right atrium and right ventricle: features of pulmonary hypertension, due to the shunts in this case.
Main pulmonary artery strapping with post-strapping dilation (29mm), enlarged left and right pulmonary arteries.

Case Discussion

MPA strapping was a temporary treatment to try to treshold pulmonary hypertension while waiting for surgery.

Goldenhar syndrome, also known as oculo-auriculo-vertebral spectrum (OAVS), usually features facial asymmetry due to unilateral abnormalities of the ears, eyes, and facial bones. 

Cardiac features are non specific for Goldenhar syndrome and are not part of the main diagnosis criteria for this syndrome.


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