PAH |
IPAH |
FPAH |
APAH |
Collagen vascular disease |
Congenital systemic-to-pulmonary shunts |
Portal hypertension |
HIV infection |
Drugs and toxins |
Other (thyroid disorders, glycogen storage disease, Gaucher's disease, hereditary haemorrhagic telangiectasia, haemoglobinopathies, myeloproliferative disorders, splenectomy) |
Associated with significant venous or capillary involvement |
PVOD |
PCH |
Persistent PH of the newborn |
PH with left heart disease |
Left-sided atrial or ventricular heart disease |
Left-sided valvular heart disease |
PH associated with lung diseases and/or hypoxaemia |
COPD |
ILD |
Sleep-disordered breathing |
Alveolar hypoventilation disorders |
Chronic exposure to high altitude |
Developmental abnormalities |
PH due to chronic thrombotic and/or embolic disease |
Thromboembolic obstruction of proximal pulmonary arteries |
Thromboembolic obstruction of distal pulmonary arteries |
Nonthrombotic pulmonary embolism (tumour, parasites, foreign material) |
Miscellaneous |
Sarcoidosis |
Histiocytosis X |
Lymphangiomatosis |
Compression of pulmonary vessels (adenopathy, tumour, fibrosing mediastinstis) |