Double inlet left ventricle (DILV) with d-TGA

Case contributed by Hidayatullah Hamidi

Presentation

Cough, sputum, fever, and hemoptysis for one year.

Patient Data

Age: 25 years
Gender: Female
  • dominant subpulmonary ventricle connected to both atrioventricular valves
  • anteriorly located small subaortic outlet chamber with no atrioventricular connection
  • ventriculoarterial discordance with the aorta arising from the small subaortic outflow chamber and the dilated pulmonary trunk from the dominant ventricle
  • ventricular septal defect with a connection of the dominant ventricle and the hypoplastic anterior chamber
  • hypoplastic aorta
  • dilatation of the pulmonary trunk (49 mm) and bilateral pulmonary arteries as well as the lobar and segmental branches
  • significant dilatation of the normally located right atrium and left atrium
  • early reflux of contrast is seen in the IVC and hepatic veins

Case Discussion

Single ventricle physiology in keeping with a double inlet left ventricle and transposition of the great arteries (d-TGA) and associated Eisenmenger syndrome.

A case like this poses many diagnostic difficulties:

The smooth septal side of the single-chamber suggests the double inlet left ventricle, which gives rise to the dilated pulmonary artery and a connection to the small subaortic accessory chamber representing the hypoplastic right ventricle. The latter is positioned anteriorly (right) as opposed to posteriorly (left) suggesting a d-loop configuration.

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