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New Classification for Pulmonary Arterial Hypertension

The 3rd World Symposium on Pulmonary Hypertension in Venice, June 23rd-25th, 2003 has provided the opportunity to evaluate the impact and usefulness of the Evian classification, and to propose modifications. Almost 90 % of the experts consider that the Evian classification is now well accepted and widely used in clinical practice, especially in centers with the most experience. In contrast, non-expert physicians apparently still use the old classification (primary vs. secondary).

The main suggestions proposed to improve the Evian classification were:

1. Pulmonary arterial hypertension (PAH) subgroup

• To abandon the term 'primary pulmonary hypertension' and to use the term 'idiopathic pulmonary arterial hypertension'.
• To combine pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) in the same subgroup, since they share the same characteristics, and to include them in a subgroup of PAH, since their clinical presentation is very similar (except in terms of their response to vasodilator therapy) and they share some identical risk factors (scleroderma, HIV; anorexigens, BMPRII mutation), and probably represent variants of the same disease.
• To improve the definition of the Eisenmenger subcategory in specifying large or small shunts, and repaired or not repaired shunts.
• To include other diseases possibly associated with PAH, such as type I glycogen storage disease, Gaucher's disease, haemoglobinopathies, thyroid disorders and hereditary haemorrhagic telangiectasia
• With regard to genetic classification, it is considered that it is probably too early to propose a classification based on genetic defects
  
  

2. Pulmonary venous hypertension subgroup

• To restrict this subgroup to left-sided heart disease only (atrial, ventricular or valvular), which represents a relatively homogeneous group.
• PVOD has been moved to group 1 And extrinsic compression of central pulmonary veins to group 5.

3. Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxaemia

and

4. Pulmonary hypertension due to chronic thrombolitic and/or embolic disease

• These two groups should were unchanged, or modified only slightly
  
  

5. Miscellaneous causes of pulmonary hypertension

• Fibrosing mediastinitis, tumours, sarcoidosis, histiocytosis
• PCH was moved to the PAH group, and schistosomiasis to the thromboembolic group.