The changing landscape of pulmonary arterial hypertension and implications for patient care

Abstract

Registries have provided a wealth of information on the clinical and disease characteristics of patients living with pulmonary arterial hypertension (PAH) since the 1980s. Certain PAH demographics, such as the prevalence of various PAH subgroups and preponderance of female patients, appear to have remained stable over time. Contemporary registry data indicate that the average age of patients diagnosed with PAH has increased, at least in the Western world. Older patients with PAH are more likely to be diagnosed with a more advanced stage of the disease, have lower exercise capacity and present with multiple comorbidities. They also have worse survival compared with younger patients. Within the PAH population, there is also a subset of patients with a lower diffusing capacity of the lung for carbon monoxide who are generally older and display more severe disease characteristics. This review discusses the implications that the increased age of the PAH population at diagnosis has on the treatment and management of the disease, as well as the need for earlier and improved diagnosis in these patients.

Figure 1.

Survival rates in older (>65 years) compared with younger (18–65 years) patients with idiopathic pulmonary arterial hypertension. a) Expected (- - - -) and observed (––––) survival rates and b) age-/sex-matched survival rates. Reproduced from [13] with permission from the publisher.