Prostacyclin's, Pulmonary Hypertension, and End of Life Care

Abstract

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a disease characterized by high pressures within the pulmonary arteries leading to right sided heart failure and often premature death [1]. Here, we present a case of PAH in which we examine the role of advanced pharmacological management, specifically intravenous prostacyclin [PC] therapy, during the transition of a patient's goals of care.CASE PRESENTATION: 64-year-old male with recently diagnosed Sjogren's disease presented with acute, decompensated right ventricular failure. He was effectively diuresed and right heart catheterization showed pulmonary arterial pressure of 71/26 mmHg (mean 42 mmHg) and pulmonary capillary wedge pressure 5 mmHg. By Fick calculations, cardiac index was 1.7 and pulmonary vascular resistance of 2.88 Wood units. He was diagnosed with Group 1 PAH related to underlying Sjogren's disease and given his low cardiac index and severely elevated mean pulmonary artery pressure, intravenous treprostinil was initiated.Unfortunately, the patient's disease progression was refractory to advanced therapy. Despite reaching a dose of 28 ng/kg/m of intravenous treprostinil, as well as Tadalafil 40mg and Ambrisentan 10mg daily, the patient's diseased right ventricle was unable to be salvaged. In the subsequent 4 months, he spent only a total of 16 days outside of the hospital. During his last hospitalization, the patient began suffering from severe malnutrition and acute metabolic encephalopathy. Unable to tolerate parenteral feeding, the family decided to transition to palliation. At the time of transition, the patient's vitals were: BP 95/70 (on norepinephrine at 12 mcg/min and dobutamine 2.5 mcg/kg/m), HR 105, afebrile, and saturating 99% on 2L NC. He was started on Dilaudid and Ativan infusion, up titrated to optimize comfort. The norepinephrine and dobutamine were stopped, and the Treprostinil remained at a dose of 28 ng/kg/m. The patient passed comfortably 10 hours after transitioning to palliative measures. DISCUSSION: At our institution, there was no protocol for titration of PC therapy during transitions to comfort measures. PC medications, unlike vasopressors, cannot be rapidly titrated as this could potentially result in severe cardiopulmonary compromise, i.e. sudden cardiac death, and thus would not be in line with a palliative care philosophy. As a result of this case, our institution developed a novel protocol for prostacyclin dosing adjustments during end-of-life care with the underlying philosophy that active monitoring of a patient's richmond agitation sedation scale can provide a valuable roadmap on prostacyclin titration. CONCLUSIONS:There is minimal evidence regarding the use of PC therapy during palliation for patients with advanced PAH. Following the implementation of a Prostacyclin End of Life Titration Protocol, we hope to provide optimal comfort and pain control for end of life PAH patients.