01.01.56 – Tetralogy of Fallot with absent pulmonary valve

IPCCC Term TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE

IPCCC Code 01.01.56

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation that is a variant of tetralogy of Fallot in which the ventriculo-arterial junction of the right ventricle with the pulmonary trunk features an atypical valve with rudimentary leaflets (cusps) which do not coapt.

  • This heart demonstrates tetralogy of Fallot with absent pulmonary valve. In Image #1, the right ventricle has been opened in clam-shell fashion showing the right ventricular inlet and outlet components with the aortic valve overriding the perimembranous ventricular septal defect. Note the anterior malalignment of the outlet septum. In Image #2, the right ventricular outflow tract is viewed from the anterior aspect with an elongated outlet component secondary to the anterior deviation of the outlet septum. The pulmonary valve consists of rudimentary, nodular, valve tissue that fails to coapt. This so-called absent pulmonary valve produces varying degrees of both pulmonary stenosis and regurgitation, often with physiologic dilation of the pulmonary trunk and right and left pulmonary arteries. The arterial duct (not seen) in this heart is absent, which is often the case. The aortic arch extends to the right with mirror image branching of the brachiocephalic arteries. (yellow star – trachea)
  • Content for Echocardiogram.
  • Content for Computerized Axial Tomography.
  • Content for Angiography.
  • Content for Intraoperative Videos.