MAP >20 mmHg at rest, recorded during right heart catheterisation.
often incidentally detected by echocardiography.
Aetiology
- pulmonary arterial hypertension (PAH) (group 1)
- idiopathic PAH
- heritable PAH
- drug- and toxin-associated PAH
- PAH associated with connective tissue disease, HIV infection, portal hypertension, congenital heart disease or schistosomiasis
- PAH with features of venous or capillary involvement
- persistent pulmonary hypertension of the newborn
- pulmonary hypertension due to left heart disease (group 2)
- pulmonary hypertension due to lung diseases and/or hypoxia (group 3)
- pulmonary hypertension due to pulmonary artery obstruction (group 4)
- chronic thromboembolic pulmonary hypertension (CTEPH)
- other pulmonary artery obstructions
- pulmonary hypertension with unclear and/or multifactorial mechanisms (group 5)
Clinical Features
- dyspnoea
- chest pain
- fatigue
- presyncope
- limitation of activities
Management