09.10.71 – Congenital pulmonary arterial (branch) hypoplasia

IPCCC Term CONGENITAL PULMONARY ARTERIAL (BRANCH) HYPOPLASIA

IPCCC Code 09.10.71

ICD-11 Code PENDING

Synonyms PENDING

Abbreviations PENDING

IPCCC Definition A congenital cardiovascular malformation in which there is diffuse luminal narrowing of a pulmonary arterial branch (below the lower limit of normal adjusted for body size).

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The right and left pulmonary arteries in this heart are hypoplastic bilaterally and along their entire length. Image #1 is a left lateral view showing the pulmonary trunk, the hypoplastic left pulmonary artery, the arterial duct (black star) and the aortic arch. The hypoplastic left pulmonary arterial wall is very thin, and it is stenotic along its entire length. In Image #2, the arterial trunks are folded anteriorly over the ventricular mass, showing the uniform hypoplasia of both the right and left pulmonary arteries as they extend to the hilar regions of the lungs.

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