Orphanet: Absence of the pulmonary artery

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Absence of the pulmonary artery

Disease definition

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital great vessels anomaly that commonly presents by dyspnea, frequent respiratory infections, hemoptysis and high-altitude pulmonary edema. UAPA is often associated with congenital heart malformation (CHM, see this term). In the absence of associated cardiac malformation (isolated UAPA; IUAPA), the condition may be asymptomatic until adult age.


Classification level: Disorder
  • Synonym(s):
    • Aplasia of pulmonary artery
    • UAPA
    • Unilateral Pulmonary Artery Absence
    • Unilateral pulmonary artery agenesis
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: Q25.7
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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