01.01.17 + 07.13.02 – Double outlet right ventricle with doubly committed ventricular septal defect & pulmonary stenosis

IPCCC Term DOUBLE OUTLET RIGHT VENTRICLE WITH DOUBLY COMMITTED VENTRICULAR SEPTAL DEFECT & PULMONARY STENOSIS

IPCCC Code 01.01.17 + 07.13.02

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 doubly committed ventricular septal defect with absence or deficiency of the conal septum, and pulmonary outflow tract obstruction.

  • 01-01-17 and 07-13-02 The right ventricle is opened in clam-shell fashion and viewed anteriorly to show both arterial trunks arising from the ventricle, producing the ventriculo-arterial connection of double outlet right ventricle. In this heart, the outlet septum (black asterisk) is fibrous rather than muscular, due to the presence of fibrous continuity between the leaflets of the pulmonary and aortic valves (yellow double headed arrow). In this case, there is also pulmonary stenosis. The interventricular communication lies between the limbs of the septal band, with fibrous continuity between the tricuspid and aortic valves, rendering the defect perimembranous. The defect is doubly committed, opening beneath both arterial outlets.
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