01.01.24 – Double outlet right ventricle with intact ventricular septum

IPCCC Term DOUBLE OUTLET RIGHT VENTRICLE WITH INTACT VENTRICULAR SEPTUM

IPCCC Code 01.01.24

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation that is a variant of double outlet right ventricle that is associated with an intact ventricular septum.

  • Image #1 shows an anterior view of a heart with double outlet right ventricle that has been sectioned along the long axis. The pulmonary trunk is dilated and there is aortic stenosis (red arrow – pulmonary outflow and yellow arrow – aortic outflow). A portion of the tricuspid valve is attached to the caudal limb of the septal band in the subaortic region. The interventricular septum is intact. Image #2 shows the heart viewed from the posterior aspect, with windows removed from the left atrium and ventricle. Both left chambers are hypoplastic with absence of the left atrioventricular connection (yellow bar). The views from the left confirm the integrity of the ventricular septum.
  • Content for Echocardiogram.
  • Content for Computerized Axial Tomography.
  • Content for Angiography.
  • Content for Intraoperative Videos.