Inhaled nitric oxide (INO) is a selective pulmonary vasodilator that has the ability to produce vasodilation in the pulmonary vascular bed without causing it in the systemic circulation. This property of INO has made it a useful therapy in the management of both adult and paediatric patients with a variety of conditions associated with pulmonary hypertension (PH), with or without hypoxia. Toxicity, cost and negative-outcome studies have prompted a search for alternative agents. These include inhaled prostacyclin and alternative prostaglandin preparations such as inhaled iloprost, treprostinol and beraprost. The phospodiesterase inhibitors show real potential in the management of both acute and chronic forms of PH, and antagonists of endogenous pulmonary vasoconstrictors, such as endothelin and thromboxane, are being evaluated for the long-term treatment of conditions such as primary pulmonary hypertension.