Conclusion
PAH is a progressive and lethal disease whose initial symptoms are non-specific. Improved awareness is required to enable patients with PAH to be diagnosed earlier and to have their disease carefully characterised and receive appropriate therapeutic intervention as soon as possible. Patients with PAH should be managed in a centre with specialist clinicians and nurses who are trained in the assessment and management of these challenging patients (often in conjunction with a local hospital) and who are available to offer advice and support when complications arise, should patients become pregnant or should surgical intervention be necessary.
It is hoped that an improved understanding of the pathophysiological mechanisms involved in PAH will lead to the development of more effective treatments.