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.