Revised clinical classification of pulmonary hypertension(PH)
| 1. Pulmonary arterial hypertension (PAH) |
| 1.1. Idiopathic PAH |
| 1.2. Familial PAH |
| 1.3. Associated with PAH |
| 1.3.1. Collagen vascular disease |
| 1.3.2. Congenital systemic-to-pulmonary shunts |
| 1.3.3. Portal hypertension |
| 1.3.4. HIV infection |
| 1.3.5. Drugs and toxins |
| 1.3.6. Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary haemorrhagic telangiectasia, haemoglobinopathies, myeloproliferative disorders, splenectomy) |
| 1.4. Associated with significant venous or capillary involvement |
| 1.4.1. Pulmonary veno-occlusive disease |
| 1.4.2. Pulmonary capillary haemangiomatosis |
| 1.5. Persistent PH of the newborn |
| 2. PH with left heart disease |
| 2.1. Left-sided atrial or ventricular heart disease |
| 2.2. Left-sided valvular heart disease |
| 3. PH associated with lung diseases and/or hypoxaemia |
| 3.1. Chronic obstructive pulmonary disease |
| 3.2. Interstitial lung disease |
| 3.3. Sleep-disordered breathing |
| 3.4. Alveolar hypoventilation disorders |
| 3.5. Chronic exposure to high altitude |
| 3.6. Developmental abnormalities |
| 4. PH due to chronic thrombotic and/or embolic disease |
| 4.1. Thromboembolic obstruction of proximal pulmonary arteries |
| 4.2. Thromboembolic obstruction of distal pulmonary arteries |
| 4.3. Nonthrombotic pulmonary embolism (tumour, parasites, foreign material) |
| 5. Miscellaneous |
| Sarcoidosis, histiocytosis X, lymphangioleiomyomatosis, compression of pulmonary vessels (adenopathy, tumour, fibrosing mediastinitis) |
Reproduced from [1] with permission from the publisher.