01.01.40 + 07.13.04 – Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis

IPCCC Term DOUBLE OUTLET RIGHT VENTRICLE WITH SUBAORTIC VENTRICULAR SEPTAL DEFECT WITHOUT PULMONARY STENOSIS

IPCCC Code 01.01.40 + 07.13.04

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation that is a variant of double outlet right ventricle with concordant atrioventricular connections, a subaortic ventricular septal defect, and unobstructed pulmonary outflow tract.

  • 01-01-40 and 07-13-04 This heart is viewed anteriorly and has been sectioned in a long axis plane. Both arterial trunks take origin predominantly from the right ventricle in the setting of a subaortic interventricular communication and an unobstructed pulmonary outflow tract. The defect, which opens to the right ventricle between the limbs of the septal band, is restrictive and perimembranous, since there is fibrous continuity between the leaflets of the aortic and tricuspid valves. The aortic valve is also in fibrous continuity with the anterior leaflet of the mitral valve.
  • Content for Echocardiogram
  • Content for Computerized Axial Tomography
  • MRI
  • Content for Angiography
  • Content for Intraoperative Videos
  • Other