Human responses to pulmonary arterial hypertension: review of the literature.

Pulmonary arterial hypertension (PAH) is a devastating disease characterized by elevation in pulmonary artery pressures causing progressive symptoms that lead to functional decline and poor quality of life. There are multiple causes of PAH including familial disease, connective tissue disease, and HIV. The estimated life expectancy is 4 years after onset of symptoms and approximately 6 to 7 years with PAH treatment. Much of the current research has focused on pharmacological treatments to improve functional status and decrease mortality. A comprehensive literature review was conducted using the CINAHL, PubMed, and MEDLINE to identify and synthesize current studies on human responses to PAH organized by emotional responses and physical functioning. Eight studies fulfilled the search criteria. Patients with PAH were learning to cope and live with uncertainty and treatment. Pulmonary arterial hypertension produced the emotional responses of anxiety, depression, and panic attacks along with impairments in cognition and memory as well as reductions in physical functioning.