Living with pulmonary hypertension: quality not just quantity

Pulmonary arterial hypertension (PAH) is a rare disease characterised by progressive right heart failure and premature death with a median survival prior to the availability of effective treatments of <3 yrs [1, 2]. There has been significant progress in its management over the last two decades with the introduction of three classes of targeted therapies: prostanoids, endothelin-1 receptor antagonists (ERAs) and phosphodiesterase-5 inhibitors [3]. A number of randomised controlled trials (RCTs) have demonstrated the efficacy of these drugs in terms of exercise capacity, functional class and pulmonary haemodynamics [4–11]. Observational studies have suggested improved survival when compared with historical series [12–14] and a recent meta-analysis of RCTs has confirmed a significant survival benefit associated with targeted therapies [15]. These therapies, however, have potentially significant side-effects related to continuous intravenous or subcutaneous infusion, frequent nebulisation or the requirement for close biochemical monitoring. In this context, the demonstration of therapy-related improvements in health-related quality of life (HRQoL), and not just in relatively modest improvements in exercise capacity or pulmonary haemodynamics, is of great importance to patients, prescribers and providers alike. The majority of RCTs in PAH have assessed HRQoL as a secondary outcome using either generic tools, …