Diagnostic classification of pulmonary hypertension
| Pulmonary arterial hypertension |
| Idiopathic |
| Familial |
| Associated with: |
| Connective tissue diseases |
| Congenital left-to-right shunt |
| Portal hypertension |
| HIV infection |
| Drugs and toxins |
| Other conditions# |
| Substantial venous or capillary involvement |
| Pulmonary veno-occlusive disease |
| Pulmonary capillary haemangiomatosis |
| Persistent pulmonary hypertension of the newborn |
| Pulmonary hypertension with left heart disease |
| Left-sided atrial or ventricular heart disease |
| Left-sided valvular heart disease |
| Pulmonary hypertension associated with lung disease or hypoxaemia or both |
| 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 or both |
| Thromboembolic obstruction of proximal pulmonary arteries |
| Thromboembolic obstruction of distal pulmonary arteries |
| Nonthrombotic pulmonary embolism (tumour, parasites, foreign material) |
| Miscellaneous |
| Sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, and compression of pulmonary vessels (adenopathy, tumour, and fibrosing mediastinitis) |
#: these conditions include thyroid disorders, type-1 glycogen storage disease, Gaucher’s disease, hereditary haemorrhagic telangiectasia, haemoglobinopathies, myeloproliferative disorders and splenectomy. Reproduced from [1] with permission from the publisher.