07.12.08 – Doubly committed juxta-arterial outlet ventricular septal defect with posteriorly malaligned outlet septum

IPCCC Term DOUBLY COMMITTED JUXTA-ARTERIAL OUTLET VENTRICULAR SEPTAL DEFECT WITH POSTERIORLY MALALIGNED OUTLET SEPTUM

IPCCC Code 07.12.08

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiac malformation in which there is a ventricular septal defect that opens to the outlet of the right ventricle, with a muscular postero-inferior border when viewed from the right ventricle, and in which the fibrous outlet septum, or the area of continuity between the aortic and pulmonary valves, is malaligned in postero-caudal fashion relative to the apical part of the muscular septum such that there is obstruction to the left ventricular outflow tract.

  • In Image #1, the pulmonary outflow is viewed anteriorly, in anatomic position. There is a ventricular septal defect that opens to the outlet of the right ventricle, with a muscular postero-inferior border (red dots) when viewed from the right ventricle. The area of fibrous continuity between the aortic and pulmonary valves is malaligned in postero-caudal fashion along with the adjacent muscular portion of the outlet septum, such that there is obstruction to the left ventricular outflow tract. In Image #2 the left ventricular outflow of the same heart shown in the left hand image demonstrates the left ventricular outflow tract viewed posteriorly and in anatomic position. The muscular component of the outlet septum joins the area of fibrous continuity between the pulmonary and aortic valves and is deviated in a postero-caudal direction causing left ventricular outflow tract obstruction.
  • Content for Echocardiogram.
  • Content for Computerized Axial Tomography.
  • Content for Angiography.
  • Content for Intraoperative Videos.