Pulmonary arterial hypertension - primary

Case contributed by A.Prof Frank Gaillard


Increasing shortness of breath.

Patient Data

Age: 30 years
Gender: Female


Annotated image

Annotated images

The enlargement main pulmonary artery is visible on frontal chest x-rays as a prominent middle 'mogul' between the aortic arch above and left atrial appendage below. 

On CT, the pulmonary trunk diameter (yellow line) should be less than approximately 30mm (reported figures vary), and should be smaller than the adjacent ascending aorta (red line). 

At a lower level, the inter-ventricular septum is reversed, bowed towards the left ventricle. 

CT demonstrates micronodules that are thought to represent enlarged pre-cap arterioles and are consistent with the diagnosis of primary idiopathic pulmonary arterial hypertension or pulmonary capillary haemangiomatosis.

Note the massive right heart strain, straight / reversed septum, enlarged RV and RA and pulmonary arteries.

Case Discussion

This case demonstrate typical appearances of pulmonary arterial hypertension, in this case due to pulmonary capillary haemangiomatosis. 

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Case information

rID: 8707
Published: 23rd Feb 2010
Last edited: 14th Aug 2019
System: Vascular, Chest
Inclusion in quiz mode: Included

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