Presentation
Exertional dyspnoea
Patient Data
There is massive enlargement of the pulmonary trunk and the right and left main pulmonary arteries.
The pulmonary trunk shows delicate mural calcification.
There is massive enlargement of the pulmonary trunk and the right and left main pulmonary arteries with abrupt pruning of the peripheral pulmonary arteries. There is also mural calcification of the pulmonary trunk (best seen on the plain film), in keeping with chronic pulmonary hypertension. Bowing of the interventricular septum to the left.
Case Discussion
This patient has known idiopathic pulmonary hypertension, and her imaging shows well the typical appearances.
Idiopathic pulmonary arterial hypertension is essentially a diagnosis of exclusion. The differential is that of causes of secondary pulmonary arterial hypertension and includes among others:
- emphysema
- cystic fibrosis
- chronic pulmonary emboli
- chronic left to right shunt
For a more complete list, please refer to the article on causes of pulmonary arterial hypertension.
None of these were present in this patient.
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