Pulmonary arterial hypertension (PAH)

Pulmonary arterial hypertension (PAH) is a progressive condition characterised by elevated blood pressure in the pulmonary arteries, leading to right heart strain and potential failure. It matters clinically as it can significantly impair exercise capacity and quality of life, and if untreated, it can be fatal. A key association is connective tissue diseases, such as systemic sclerosis, which can lead to the development of PAH due to vascular changes and inflammation.