Unilateral absence of the pulmonary artery

Case contributed by Dr George Harisis

Presentation

Pre-operative assessment for repair of congenital bicuspid aortic valve. History of normal pressure hydrocephalus.

Patient Data

Age: 45 years
Gender: Female
X-ray

Chest X-ray

Frontal chest radiograph demonstrates asymmetric absence of the right pulmonary artery shadow associated with mild rightwards mediastinal shift and elevation of the right hemidiaphragm.  The heart size is within normal limits. The lungs and pleural spaces are clear. Very subtle inferior notching of the right posterior 4th to 6th ribs is noted. 

CT

CT Chest

There is unilateral absence of the right pulmonary artery with no pulmonary arterial blood flow to the right lung. The right lung is supplied by prominent intercostal collaterals originating from the descending thoracic aorta and a dilated right internal mammary artery. VP shunt tubing is noted in the left anterior chest wall.

Case Discussion

This is an interesting case of unilateral absence of a pulmonary artery (UAPA), a rare entity that can present with non-specific symptoms such as chest pain and shortness of breath, or be entirely asymptomatic 1.

In many cases, UAPA is associated with other congenital abnormalities such as aortic coarctation or tetralogy of Fallot 2.  

In this case, several subtle findings on the chest x-ray hint at the diagnosis. 

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Case information

rID: 58966
Published: 15th Mar 2018
Last edited: 16th Jul 2018
System: Chest
Inclusion in quiz mode: Included

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