Oedema/fluid retention has been associated with the use of ERAs and is also a clinical manifestation of right heart failure and underlying PAH disease. In a long-term double-blind study in patients with PAH, the incidence of oedema AEs in the macitentan 10 mg and placebo treatment groups was 11.0 events / 100 patient-years on macitentan 10 mg compared to 12.5 events / 100 patient-years on placebo.