Pulmonary artery atresia

Pulmonary artery atresia (or sometimes known as pulmonary atresia) is one of congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow tract (RVOT) and the pulmonary trunk.

The estimated incidence is 1 in 10,000 births.

The term pulmonary atresia can cover a broad spectrum of abnormalities depending on the extent of the disruption.

Sub types

It can be classified into 3 sub-types 1:

Variants
Associations
  • tetralogy of Fallot: PA-VSD is considered by some authors as a severe from of tetralogy of Fallot
  • truncus arteriosus (Collett and Edwards type IV) or pseudotruncus: recognized to be a form of PA-VSD rather than truncus arteriosus 2
  • heterotaxy syndrome: pulmonary atresia is present in two-fifths of right isomerism cases 9
Plain radiograph
PA-VSD 3:
  • normal or mildly enlarged heart with poor / diminished pulmonary arterial vascular markings
  • asymmetrical vascular suggest stenosis within pulmonary arterial tree
  • there can be plethora due to horizontal arteries forming aorto-pulmonary collaterals
  • mottled appearance as the lung periphery may suggest pleuro-pulmonary collateral formation
PA-IVS: 
  • severe cardiomegaly from massive right atrial dilatation 10 
Echocardiography

Echocardiography can characterise intracardiac anatomy. Has limited role in assessing pulmonary artery anatomy. 

MDCT

MDCT is better than echocardiography for pulmonary artery anatomy.  It allows evaluation of the following 4:

  • length of pulmonary atresia
  • presence of pulmonary artery confluence
    • seagull appearance of the confluent pulmonary arteries and abbreviated pulmonary trunk
  • size of main, right, and left pulmonary arteries at the origin and at the hilum
  • presence of branch pulmonary artery stenosis
  • sources of pulmonary blood flow to each lung, including the number of bronchopulmonary segments supplied by native pulmonary arteries and the distribution of major aortopulmonary collateral arteries (MAPCAs)
Cardiac MRI

Allows direct visualisation of anomaly. Cine sequences may show a dilated non contracting right ventricle 5. MR angiography allows detection of aortopulmonary collaterals and patent ductus arteriosus (if present).

  • management varies depending on the presence of a VSD.
    • one surgical technique includes the unifocalisation procedure which can be performed in cases with complete pulmonary artery atresia with major aortopulmonary collateral arteries (MAPCAs).
  • prostaglandin E1 is used to keep the ductus open 6.

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.

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Article Information

rID: 8074
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Atresia of the pulmonary artery
  • Atresia of the pulmonary arteries
  • Pulmonary artery agenesis
  • Agenesis of the pulmonary artery
  • Pulmonary atresia

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    Pulmonary atresia
    Case 1
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    Case 2: with intact interventricular septum
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    Case 3: fetal echocardiogram
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    Case 4: CT
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    Case 5: in situs inversus
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    Case 6: with complex cardiac malformation
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    Case 7: in heterotaxy syndrome
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    Case 8: in heterotaxy syndrome
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