Abstract
The development of effective orally administered medical therapy for pulmonary arterial hypertension (PAH) has made a significant impact on outcome in patients with PAH. Identification of patient groups likely to derive optimal benefit is important, given cost and potential side effects; the clinical effectiveness of these therapies in older patients with PAH is unclear as the presence of co-morbidity may limit benefits of therapy.
We evaluated the epidemiology of PAH in a contemporary cohort to assess the influence of age on long-term outcome using PAH-specific therapies.
A total of 119 patients (88% female; mean age 65±12 years) were reviewed, comprising 52% with underlying connective tissue disease. Bosentan was the PAH specific agent most frequently used. The baseline 6MWT distance in the entire cohort was 304m with age associated with a significant decline in 6MWT.
In a large cohort of patients treated with PAH-specific therapies, patients less than 55 years of age showed improvement in 6MWT with older patients demonstrating stabilisation or decline.