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Double outlet right ventricle

Case contributed by Yaïr Glick
Diagnosis certain

Presentation

Congenital cardiac defect. Bronchoscopy revealed a tracheal narrowing inferior to the vocal cords - rule out vascular ring.

Patient Data

Age: 5 days
Gender: Male
  • enlarged heart, in particular, the right atrium and ventricle
  • aortic and main pulmonary artery origin from the right ventricle
  • large ventricular septal defect
  • right aortic arch with a right-sided descending aorta
  • common origin of the left pulmonary artery and the left brachiocephalic artery bifurcating into the left subclavian and left common carotid artery from the aortic arch
  • separate aortic origins of the right common carotid artery and right subclavian artery afterwards
  • patent ductus arteriosus
  • decreased diameter of the trachea at the left brachiocephalic trunk (2.7 mm vs 4.3 mm proximally and distally) indicating compression
  • normal lung configuration
  • normal drainage of pulmonary veins into the left atrium
  • Kerley B lines in both lungs, suggestive of left heart insufficiency
  • nutmeg appearance of the liver, suggestive of right heart insufficiency

Case Discussion

Double outlet RV, large VSDPDAright-sided aortic arch with mirror-image branching
and left pulmonary artery from the aortic arch.
Pressure exerted on the trachea from a branching vascular ring.

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