Conclusion
It is hoped that further improvement of our understanding of the pathophysiological mechanisms involved in the development of PAH will be accompanied by the development of more effective treatments and that the role of combination therapy will be defined. However, PAH remains a progressive lethal disease that is frequently not diagnosed because initial symptoms are non-specific. Improved awareness is therefore necessary to ensure that patients with PAH receive earlier diagnosis and are referred to an appropriate specialist centre to have the condition characterised and to receive appropriate therapeutic intervention as soon as possible. It is widely held that patients with PAH should be managed in a PH centre where there are specialist clinicians and nurses trained in the assessment and management of these challenging patients. Often this model occurs in a combination or shared cared arrangement with one or more satellite hospitals who have agreed shared protocols, joint MDT meetings and clinics. This offers convenience for the patients and should they develop problems locally they can attend their local hospital and have appropriate advice and treatment given. If necessary, support can be sought from the primary specialist centre. Patients who are pregnant require careful MDT assessment and regular antenatal and perinatal input from the PH team. Likewise patients with PH who are referred for surgical intervention require close communication between anaesthetists, surgeons and the PH team.