Research in pulmonary hypertension has led to exciting advances over the last decade, both in terms of understanding aetiology and expanding treatment options. This review highlights the current approach to classification and disease management. In particular, we review the significant data and recommendations on the use of prostacyclin analogues, endothelin receptor antagonists, phosphodiesterase inhibitors and drug combinations. (C) 2004 Elsevier Ireland Ltd. All rights reserved.