2003 Third world symposium on pulmonary arterial hypertension classification
Dr Yuranga Weerakkody and Dr Frank Gaillard et al.
There are numerous causes of pulmonary arterial hypertension, and thus not surprisingly there have been many classification systems.
In 2003 the Third World Symposium on PAH met in Venice and produced an updated classification system.
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pulmonary arterial hypertension (PAH)
- idiopathic (IPAH)
- familial (FPAH)
- associated with (APAH):
- connective tissue disease
- congenital systemic to pulmonary shunts
- portal hypertension
- HIV infection
- 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
- persistent pulmonary hypertension of the newborn (PPHN)
- pulmonary hypertension associated with left heart diseases
- left-sided atrial or ventricular heart disease
- left-sided valvular heart disease
- pulmonary hypertension associated with lung respiratory diseases and/or hypoxia
- chronic obstructive pulmonary disease (COPD)
- interstitial lung disease
- sleep disordered breathing
- alveolar hypoventilation disorders
- chronic exposure to high altitude
- developmental abnormalities
- pulmonary hypertension due to chronic thrombotic and/or embolic disease
- thromboembolic obstruction of proximal pulmonary arteries
- thromboembolic obstruction of distal pulmonary arteries
- non-thrombotic pulmonary embolism (tumour, parasites, foreign material)
- miscellaneous
- sarcoidosis
- Langerhans cell histiocytosis (LCH / histiocytosis X)
- lymphangiomyomatosis (LAM)
- compression of pulmonary vessels
- adenopathy
- tumour
- fibrosing mediastinitis
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