5 clinical subgroups
Categorisation is based on similar pathological mechanisms, clinical presentation, haemodynamic characteristics and therapeutic management.
There are many causes, but examples include:
Group
Examples
1. PAH
Idiopathic, heritable, connective tissue disease, drug and toxin-induced, portal hypertension, HIV
2. PH-LHD
Left ventricular systolic or diastolic impairment, valvular heart disease
3. PH-LD
Obstructive, restrictive, hypoxia without lung disease
4. PH due to pulmonary artery obstruction
Chronic thromboembolic PH (CTEPH)
5. PH with unclear / multifactorial mechanisms
Sarcoid, haematological, metabolic
PAH – pulmonary arterial hypertension
PH-LHD – PH due to left heart disease
PH-LD – PH due to lung disease
