Pulmonary arterial hypertension (PAH) is a devastating disease that has a considerable effect on quality of life and eventually leads to right heart failure. The past ten years have seen improvements in drug therapy and patient care, and the prospects for patients with PAH are now considerably better. Although new drugs have played a major role in this improvement, the focus is on improving the patient care aspect of PAH management. Specialist and general nurses are expected to play a key role in patient care, and this article summarises how changes in the management of PAH can be implemented, with special emphasis on the role of the multidisciplinary team. Lessons learned from this specialty may be effectively transferred to similar rare chronic disease areas.