Pulmonary Arterial Hypertension in Octogenarians: The Pulmonary Hypertension Association Registry

Pulmonary arterial hypertension (PAH) was historically viewed as a disease of young adults; however, emerging evidence suggests rising prevalence in the elderly, which is an insufficiently characterized population. We evaluated clinical and sociodemographic features of PAH patients over 80 years and compared their outcomes with younger patients. We performed a prospective cohort study utilizing the Pulmonary Hypertension Association Registry to compare clinical characteristics, functional capacity, and quality of life measures between octogenarians (≥ 80 years) and non-octogenarians (< 80 years) at enrollment and follow-up visits. Associations between age subgroups and outcomes were assessed using linear, binomial, and Cox regression. The cohort comprised 113 octogenarians and 3,094 non-octogenarians with PAH. Smoking history was more common in octogenarians, who also more frequently had idiopathic or connective tissue disease-related PAH. Octogenarians had lower right atrial pressure and mean pulmonary artery pressure (p < 0.001) than younger patients. At baseline, octogenarians had a lower absolute 6-min walk distance and higher World Health Organization functional class (p < 0.001). Octogenarians were more often initially treated with monotherapy and less likely to receive a prostacyclin analog. Octogenarians had a 57% higher risk of all-cause hospitalization and twice the mortality of younger adults after covariate adjustment (IRR 1.57, 95%CI 1.06-2.33, p < 0.05 and HR 2.38, 95%CI 1.62-3.52, p = 0.001, respectively). The oldest PAH patients exhibit distinct clinical profiles, therapies, and outcomes compared to younger adults, though it remains uncertain whether these differences reflect a unique phenotype, reduced therapy response, or the cumulative effects of aging and comorbid conditions.