Pulmonary arterial hypertension
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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