Diagnostic classification of pulmonary hypertension(Venice 2003)
| Pulmonary arterial hypertension |
| Idiopathic |
| Familial |
| Associated with: |
| Connective tissue disease |
| Congenital systemic to pulmonary shunts |
| Portal hypertension |
| HIV infection |
| Drugs and toxins |
| Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary haemorrhagic telangiectasia, haemoglobinopathies, myeloproliferative disorders or splenectomy) |
| Associated with significant venous or capillary involvement |
| Pulmonary veno-occlusive disease |
| Pulmonary capillary haemangiomatosis |
| Persistent pulmonary hypertension of the newborn |
| Pulmonary hypertension associated with left-sided heart disease |
| Left-sided atrial or ventricular heart disease |
| Left-sided valvular heart disease |
| Pulmonary hypertension associated with lung respiratory diseases or hypoxia |
| Chronic obstructive pulmonary disease |
| Interstitial lung disease |
| Sleep-disordered breathing |
| Alveolar hypoventilation disorders |
| Chronic exposure to high altitude |
| Developmental abnormalities |
| Pulmonary hypertension due to chronic thrombotic or embolic disease |
| Thromboembolic obstruction of proximal pulmonary arteries |
| Thromboembolic obstruction of distal pulmonary arteries |
| Nonthrombotic pulmonary embolism (tumour, parasites or foreign material) |
| Miscellaneous |
| Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumour or fibrosing mediastinitis) |