Advanced Pulmonary Hypertension: Considerations for Care Planning at End of Life (427)

Abstract

Objectives 1. Explain the pathophysiology of and symptoms associated with advanced pulmonary hypertension including the mechanisms of action of medications commonly used for advanced pulmonary hypertension and their impact on symptoms. 2. Discuss the current literature on managing patients with advanced pulmonary hypertension at the end-of-life. 3. Propose a guideline for providing end-of-life care for patients with advanced pulmonary hypertension. The survival of patientswithWorldHealthOrganization functional class IV pulmonary hypertension is less than one year if untreated. Although initiation of prostacyclin analogues improves functional status and survival, class IV pulmonary hypertension remains a terminal illness. Unfortunately, there is little evidence on how best to manage these patients at the end-oflife. This session will use case studies of patients admitted to our MICU on continous IV prostacyclin analogues to discuss the challenges and considerations inherent in providing care to patients dying with advanced pulmonary hypertension. Briefly, we will describe the pathophysiology of advanced pulmonary hypertension including a review of associated symptoms. Next, we will outline the pharmacokinetics of intravenous prostacyclin analogues (epoprostenol and trepostinil) used for patients with class IV pulmonary hypertension, including the impact of these medications on patient symptoms. Given the dearth of literature on end-oflife care for patients with class IV pulmonary hypertension, we will propose general guidelines for medical management by extrapolating from studies examining discontinuation or transitions off of other medications with similar mechanisms (eg, nitric oxide, sildenafil, and prostacyclin) as well as from our own experiences. Finally, we will use these cases to examine various psychosocial challenges associated with patients dying from pulmonary hypertension, including considerations for place of death (eg, hospital setting, home hospice, inpatient hospice). A detailed handout will be provided that outlines our proposed medical management guidelines for care of patients dying from advanced pulmonary hypertension.