01.01.07 – Pulmonary atresia with intact ventricular septum

IPCCC Term PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

IPCCC Code 01.01.07

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there are normally aligned great arteries, no opening between the morphologically right ventricle and the pulmonary trunk, and no ventricular level communication.

  • Image # 1: This specimen demonstrates pulmonary atresia-intact septum and is viewed in anatomic position with the free wall of the right atrium and ventricle removed. The cavity of the right ventricle is bipartite, being represented only by its inlet and outlet component due to mural overgrowth of the apical trabecular component. This produces a less favorable type of pulmonary atresia-intact septum within the spectrum of this malformation. The outflow tract is patent, but extremely stenotic. At the distal aspect of the outflow tract there is pulmonary atresia with a tiny remnant of an imperforate valve at the ventriculo-arterial junction. Image #2: This close-up view of the atretic pulmonary valve is viewed from above and demonstrates triradiating sinuses peripherally with a tiny, imperforate valve centrally. The dilated sinuses of Valsalva are easily appreciated.
  • Content for Echocardiogram.
  • Content for Computerized Axial Tomography.
  • Content for Angiography.
  • Content for Intraoperative Videos.

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