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Pulmonary trunk hypoplasia

MedGen UID:: 576694
•Concept ID:: C0345017
•: Congenital Abnormality

Synonym:	Pulmonary Trunk Hypoplasia
SNOMED CT:	Pulmonary trunk hypoplasia (253622001)

Definition

Underdeveloped pulmonary arteries. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPulmonary trunk hypoplasia

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Congenital malformation of the respiratory system
        Congenital anomaly of lung
        Pulmonary trunk hypoplasia

Professional guidelines

PubMed

Congenital Bilateral Branch Pulmonary Artery Hypoplasia: Prenatal Diagnosis and Parental Counseling.

Habib PA, Bae S, Turan S, Sundararajan S
Neoreviews 2023 Dec 1;24(12):e829-e835. doi: 10.1542/neo.24-12-e829. PMID: 38036449

Pulmonary arterial hypertension: pathogenesis and clinical management.

Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357 Free PMC Article

Alagille syndrome: pathogenesis, diagnosis and management.

Turnpenny PD, Ellard S
Eur J Hum Genet 2012 Mar;20(3):251-7. Epub 2011 Sep 21 doi: 10.1038/ejhg.2011.181. PMID: 21934706 Free PMC Article

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