Table 1

 Revised diagnostic classification of pulmonary hypertension (2003)

• Pulmonary artery hypertension (PAH)
    Idiopathic PAH
    Familial PAH
    Related to:Collagen vascular disease
Congenital systemic to pulmonary shunts
Portal hypertension
HIV infection
Drugs and toxins
    Associated with significant venous or capillary involvement
        Pulmonary veno-occlusive disease
        Pulmonary capillary haemangiomatosis
• Pulmonary hypertension with left heart disease
• Pulmonary hypertension associated with lung disease and/or hypoxaemia
• Pulmonary hypertension caused by chronic thrombotic or embolic disease
• Miscellaneous