The Journal of Heart and Lung Transplantation
International Society for Heart and Lung Transplantation.

Characterisation of Pulmonary Arterial Hypertension (PAH) Patients Initiating a New PAH Specific Therapy in the Context of Age: Insights from EXPOSURE


      In PAH, the proportion of older patients is increasing. Real-world data from the EXPOSURE study were used to describe clinical characteristics and treatment patterns of patients, depending on age, in current clinical practice.


      EXPOSURE (EUPAS19085) is an ongoing, multicenter, prospective, observational study conducted in Europe and Canada of PAH patients initiating a PAH-specific therapy. Patients were grouped according to age at treatment initiation (baseline): ≥65 and <65 years.


      From September 2017 to June 2020, 396 patients ≥65 years and 484 patients <65 years had follow-up data. At baseline, median (range) age was 73 (65, 88) and 52 (18, 64) years in the ≥65 and <65 years subgroups respectively. Patients were predominantly incident PAH in both subgroups (Table). Compared with younger patients, older patients were more likely to have more severe disease characteristics, more comorbidities, and present with an intermediate/high risk of 1-year mortality (COMPERA method). Mean right atrial pressure and diffusing capacity of lung for carbon monoxide were more compromised in older vs younger patients (Table). Patients ≥65 years were mainly initiated on monotherapy. During the median (range) observation period of 8.9 (0.2, 30.2) months, older patients had more PAH-related hospitalizations (incidence rate per 100 person-years [95% CI]: 24.8 [19.3-31.3]), and a higher mortality rate (18.7 [14.3-24.1] per 100 person-years [95% CI]) vs younger patients.


      Despite more severe disease characteristics, older patients with PAH were more likely to initiate PAH-specific monotherapy over combination therapy compared with those <65 years. These data suggest physicians are less likely to follow current treatment recommendations in older patients. Greater disease severity, more comorbidities, and more monotherapy use in older patients might contribute to the worse clinical outcomes observed in these patients.