Unilateral pulmonary artery atresia

Unilateral pulmonary artery atresia (UPAA) or unilateral absence of the pulmonary artery (UAPA) is a variant of pulmonary artery atresia

The estimated prevalence is around 1 in 200,000 young adults. The reported frequency on the right side is slightly greater for some reason 10.

Can be variable and include:

  • asymptomatic: adult patients with UPAA are often asymptomatic 8
  • chest pain
  • pleural effusion
  • recurrent chest infections: lack of arterial blood flow to the affected lung may result in poor delivery of inflammatory cells to sites of inflammation and impaired ciliary function
  • dyspnoea
  • reduced exercise intolerance
  • pulmonary hypertension
  • haemoptysis
  • high-altitude pulmonary oedema

It commonly occurs on the side opposite to the side of the aortic arch.

The distal intrapulmonary branches of the affected artery usually remain intact and can be supplied by collateral vessels from other vessels such as bronchial, intercostal, internal mammary, subdiaphragmatic, subclavian, or even coronary arteries.

Associations

It occurs in association with other cardiac anomalies  in approximately 60% of cases. They include:

In around 40% of cases they occur in isolation, where it is then termed as:

Features can vary dependent on which side is affected.

Plain radiograph

May be seen as volume loss to the ipsilateral lung and overinflation +/- herniation across the midline of the contralateral lung 4.

CT

Allows direct visualisation of the absence of the affected pulmonary artery. Volume loss to the affected lung is also shown. Extensive collaterals can be visualised. May allow recognition of associated cardiac anomalies.

Recognised complications include:

Treatment is often around management of complications in asymptomatic individuals. There is often no commonly accepted consensus on a particular treatment strategy.

It is thought to have been first described by O Frantzel in 1868 3


Congenital heart disease

There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorised, it is helpful to have a working understanding of normal and fetal circulation, as well as an understanding of the segmental approach to imaging in congenital heart disease.

Share article

Article Information

rID: 31904
Systems: Vascular, Chest
Synonyms or Alternate Spellings:
  • Unilateral absence of pulmonary artery (UAPA)
  • Unilateral absence of the pulmonary artery
  • Unilateral absence pulmonary artery (UAPA)
  • Unilateral absence of the pulmonary artery (UAPA)
  • Unilateral pulmonary artery atresia (UPAA)
  • UPAA
  • UAPA

Support Radiopaedia and see fewer ads

Cases and Figures

  • Drag
    Case 1: left PA atresia
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.