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Pulmonary arterial hypertension

Case contributed by Varun Babu
Diagnosis certain

Presentation

Severe pulmonary hypertension. Was sent for ruling out pulmonary thromboembolism

Patient Data

Age: 40 years
Gender: Female

Chest (AP) radiograph

x-ray
  • cardiomegaly (CT ratio >50%) with elevated cardiac apex. 
  • enlarged right atrium
  • dense bilateral hilum with positive hilum convergence sign
  • obliteration of left pulmonary bay
  • incidental azygos lobe
  • dilated main pulmonary artery and both right and left pulmonary arteries. 
  • pruning of peripheral pulmonary vessels.
  • no acute or chronic pulmonary thromboembolism
  • right sided cardiomegaly with RV:LV ratio >1
  • dilated right atrium
  • straightened interventricular septum with mild bowing to left ventricle
  • incidental azygos lobe

Case Discussion

It is important to understand the pathophysiology involving pulmonary arterial hypertension (PAH) and how it reflects on various structures involving the pulmonary circulation. From early grades of simple dilatation of main pulmonary trunk to severe grades, as in our study wherein the high pulmonary arterial system pressure back reflects changes in the right side of the heart are key to document in imaging. Acute or chronic pulmonary thromboembolism is one of the causes of severe PAH and has to be ruled out. In this case it turned out to be negative. 

Parenchymal lung disease (not depicted) was also absent in our patient. 

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