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.
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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. |
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