Pulmonary Arterial Hypertension: A Palliative Medicine Review of the Disease, Its Therapies, and Drug Interactions

David Christiansen; Sandra Porter; Lindsay Hurlburt; Andrea Weiss; John Granton; Kirsten Wentlandt

doi:10.1016/j.jpainsymman.2019.11.023

Pulmonary Arterial Hypertension: A Palliative Medicine Review of the Disease, Its Therapies, and Drug Interactions

J Pain Symptom Manage. 2020 Apr;59(4):932-943. doi: 10.1016/j.jpainsymman.2019.11.023. Epub 2019 Dec 2.

Authors

David Christiansen¹, Sandra Porter², Lindsay Hurlburt³, Andrea Weiss⁴, John Granton⁵, Kirsten Wentlandt⁶

Affiliations

¹ Department of Internal Medicine, Section of Respiratory Medicine, University of Manitoba, Winnipeg, Manitoba, Canada.
² Department of Pharmacy, University Health Network, Toronto, Ontario, Canada.
³ Division of Palliative Care, Department of Supportive Care, University Health Network, Toronto, Ontario, Canada; Department of Anesthesia, University of Toronto, Toronto, Ontario, Canada.
⁴ Division of Palliative Care, Department of Supportive Care, University Health Network, Toronto, Ontario, Canada; Division of Palliative Care, Department of Family and Community Medicine, University of Toronto, Toronto, Ontario, Canada.
⁵ Division of Respirology, Department of Medicine, University Health Network, Sinai Health System, University of Toronto, Toronto, Ontario, Canada.
⁶ Division of Palliative Care, Department of Supportive Care, University Health Network, Toronto, Ontario, Canada; Division of Palliative Care, Department of Family and Community Medicine, University of Toronto, Toronto, Ontario, Canada. Electronic address: kirsten.wentlandt@uhn.ca.

PMID: 31805363
DOI: 10.1016/j.jpainsymman.2019.11.023

Abstract

Pulmonary arterial hypertension (PAH) is often a progressive and ultimately fatal disease. It is characterized by an elevated mean pulmonary arterial pressure because of disease of the small pulmonary arterioles. PAH leads to a constellation of symptoms, including dyspnea, fatigue, syncope, chest discomfort, and peripheral edema. Disease-targeted therapies for PAH produce symptomatic and functional improvement, but long-term survival remains uncommon without lung transplantation. Palliative care is appropriate to support patients with advanced PAH who typically have a high symptom burden. However, palliative care has historically focused on supporting patients with malignant disease, rather than progressive chronic disease such as PAH. Our aim is to provide palliative care clinicians with a background in the classification, pathophysiology, and modern treatment of PAH. This review describes disease-targeted therapies and their effects on symptoms, physical functioning, and health-related quality of life. We also review the unique physiology of PAH and its implication for palliative interventions. Pharmacological interactions with, and precautions related to commonly used palliative care medications, are discussed.

Keywords: Pulmonary arterial hypertension; drug interactions; function; health-related quality of life; palliative care; symptom.

Publication types

Review

MeSH terms

Drug Interactions
Humans
Hypertension, Pulmonary* / drug therapy
Palliative Medicine*
Pulmonary Arterial Hypertension*
Quality of Life