TABLE 1.
Cause | Histological features | Animal models |
Group 1: Pulmonary arterial hypertension (PAH) | ||
Idiopathic PAH | Pulmonary artery intimal proliferation | Su-Hx rat/mouse |
Heritable PAH | Pulmonary artery medical hypertrophy | MCT rat |
Drugs/Toxin/Others | Plexiform lesions | Su-Hx-Normoxia |
Group 2: Pulmonary hypertension with left heart disease | ||
Left-sided heart disease | Pulmonary medical hypertrophy | SU5416/Obese ZSF1 rat |
Pulmonary vein arterialization | ||
Pulmonary interstitial edema | ||
Group 3: Pulmonary hypertension associated with lung disease | ||
and/or hypoxemia | ||
High altitudes | Hypoxic pulmonary vasoconstriction | Su-Hx rat/mouse |
COPD/Pulmonary fibrosis | Muscularization of arterioles | Hypoxia rat/mouse |
Obstructive sleep apnea | ||
Group 4: Pulmonary hypertension due to chronic thrombotic and/or | ||
embolic disease | ||
Pulmonary emboli | Thrombi or embolism | Vena cava ligation |
Other clotting disorders | Recanalized organized thrombi | |
Group 5: Pulmonary hypertension triggered by other health conditions | ||
Heterogeneous | Heterogeneous |