Table 1—

Diagnostic classification of pulmonary hypertension(Venice 2003)

Pulmonary arterial hypertension
 Associated with:
  Connective tissue disease
  Congenital systemic to pulmonary shunts
  Portal hypertension
  HIV infection
  Drugs and toxins
  Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary haemorrhagic telangiectasia, haemoglobinopathies, myeloproliferative disorders or splenectomy)
 Associated with significant venous or capillary involvement
  Pulmonary veno-occlusive disease
  Pulmonary capillary haemangiomatosis
 Persistent pulmonary hypertension of the newborn
Pulmonary hypertension associated with left-sided heart disease
Left-sided atrial or ventricular heart disease
 Left-sided valvular heart disease
Pulmonary hypertension associated with lung respiratory diseases or hypoxia
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
Thromboembolic obstruction of proximal pulmonary arteries
 Thromboembolic obstruction of distal pulmonary arteries
 Nonthrombotic pulmonary embolism (tumour, parasites or foreign material)
 Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumour or fibrosing mediastinitis)