Palliative Care and Pulmonary Arterial Hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a chronic and progressive disease associated with impaired health-related quality of life and survival. Palliative care (PC) is patient- and family-centered care provided by an interdisciplinary team with an overarching goal of alleviating suffering and improving quality of life for patients with advanced illness. PC in other chronic diseases is associated with improved quality of life, symptom management, illness understanding, and reduced caregiver burden, but there is limited data regarding PC in PAH. Despite limited evidence, there is strong rationale for involving PC specialists in the longitudinal management of PAH. There are currently no guidelines to help clinicians determine the most appropriate timing for referral of PAH patients to PC specialty teams. Consequently, referrals are limited and often delayed. Adoption of a standardized approach to PC referrals based on clinical or patient triggers could facilitate earlier involvement of PC as an adjunct to ongoing PAH disease-directed care. Incorporation of PC in the longitudinal management of PAH may be beneficial to address the multidimensional aspects of living with a chronic and life-limiting illness.