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Clinical Classification according to Venice 2003

1. Pulmonary arterial hypertension (PAH)
1.1. Idiopathic
1.2. Familial
1.3. Related to:
    1.3.1. Collagen vascular disease (BMPRII-)
    1.3.2. Congenital systemic to pulmonary shunts
    1.3.3. Portal hypertension
    1.3.4. HIV infection (BMPRII - )
    1.3.5. Drugs and toxins (rare BMPRII + in anorexigens PAH)
    1.3.6. Other: thyroid disorders, type I glycogen storage disease, Gaucher's
               disease, hereditary haemorrhagic telangiectasia, hemoglobinopathies,
               myeloproliferative disorders,
1.4. Associated with significant venous and/or capillary involvement
    1.4.1. Pulmonary veno-occlusive disease (PVOD)
    1.4.2. Pulmonary capillary hemangiomatosis (PCH)
1.5. Persistent pulmonary hypertension of the newborn

2. Pulmonary hypertension associated with left heart disease
2.1. Left-sided atrial or ventricular heart disease
2.2. Left-sided valvular heart disease

3. Pulmonary hypertension associated with lung diseases and/or hypoxaemia
3.1. Chronic obstructive pulmonary disease
3.2. Interstitiallung disease
3.3. Sleep-disordered breathing
3.4. Alveolar hypoventilation disorders
3.5. Chronic exposure to high altitude
3.6. Developmental abnormalities

4. Pulmonary hypertension 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. Pulmonary embolism (tumour, parasites, foreign material)

5. Miscellaneous
Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of the pulmonary vessels (adenopathy, tumour, fibrosing mediastinitis)