1 PAH |
Idiopathic PAH |
Heritable PAH |
Drug- and toxin-induced PAH |
PAH associated with: |
Connective tissue disease |
HIV infection |
Portal hypertension |
Congenital heart disease |
Schistosomiasis |
PAH long-term responders to calcium channel blockers |
PAH with overt features of venous/capillaries (PVOD/PCH) involvement |
Persistent PH of the newborn syndrome |
2 PH due to left heart disease |
PH due to heart failure with preserved LVEF |
PH due to heart failure with reduced LVEF |
Valvular heart disease |
Congenital/acquired cardiovascular conditions leading to post-capillary PH |
3 PH due to lung diseases and/or hypoxia |
Obstructive lung disease |
Restrictive lung disease |
Other lung disease with mixed restrictive/obstructive pattern |
Hypoxia without lung disease |
Developmental lung disorders |
4 PH due to pulmonary artery obstructions |
Chronic thromboembolic PH |
Other pulmonary artery obstructions |
5 PH with unclear and/or multifactorial mechanisms |
Haematological disorders |
Systemic and metabolic disorders |
Others |
Complex congenital heart disease |