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WHO Group I Classification of PAH
Pulmonary Arterial Hypertension (PAH) represents Group 1 within the Pulmonary Hypertension (PH) WHO clinical classification system (Venice 2003 revision) and is one of five such groups. The groups are divided based on etiology.1
Group I. Pulmonary arterial hypertension (PAH)
- Idiopathic (IPAH)
- Familial (FPAH)
- Associated with (APAH):
- Connective tissue disease
- Congenital systemic-to-pulmonary shunts (e.g. Eisenmenger's)
- Portal hypertension
- 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
- Pulmonary veno-occlusive disease (PVOD)
- Pulmonary capillary haemangiomatosis (PCH)
- Persistent pulmonary hypertension of the newborn (PPHN)
Group II. Pulmonary venous hypertension with left heart diseases
Group III. Pulmonary hypertension associated with respiratory diseases and/or hypoxemia (including chronic obstructive pulmonary disease)
Group IV. Pulmonary hypertension due to chronic thrombotic and/or embolic disease
Group V. Miscellaneous group
- e.g. sarcoidosis, histiocytosis X and lymphangiomatosis
Reference
1. J Am Coll Cardiol 2004; 43: Suppl S: 5S-12S
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