The initial 18 months of the first multi-disciplinary regional Pulmonary Arterial Hypertension Clinic in Australia

Objective: To report the initial 18 months experience of the first multi-disciplinary regional Pulmonary Arterial Hypertension (PAH) Clinic in Australia. Design: Prospective cohort study. Setting: Community setting on the mid-north coast of New South Wales. Participants: A total of 47 patients (mean age ± standard error of the mean: 71.8 ± 1.8 years; male/female ratio 13/34). Main outcome measures: Diagnosis of PAH, exclusion of other causes of pulmonary hypertension, commencement of PAH-specific pharmacotherapy. Results: Twenty-three (49%) patients were discharged back to their GP with pulmonary hypertension from a combination of ischaemic heart and/or lung disease. Three (6%) patients died from connective tissue disease (CTD)-related PAH with one death (2%) from ischaemic heart disease. Five (11%) patients remain on treatment (n = 2, Bosentan for congenital heart disease-related PAH; n = 1 Bosentan for CTD-related PAH; and n = 1 Bosentan and n = 1 Sildenafil for primary PAH). Fifteen (32%) patients have ongoing review for PAH related to CTD (n = 11), carcinoid (n = 1) and uncertain cause (n = 3). Conclusion: Patients with CTD-related PAH have a poor prognosis. PAH should be considered in anyone with dyspnoea without obvious features of cardiac or pulmonary disease, especially in the setting of a CTD. Regional population centres are under-resourced with PAH specialty medical services. We have sought to address this by establishment of the first regional multi-disciplinary PAH Clinic in Australia.