Management of Critically Ill Patients With Pulmonary Arterial Hypertension in Transport: A Narrative Review

Abstract

Pulmonary arterial hypertension (PAH) is a unique disease process with a highly complex physiology. Patients with PAH are often on specialized medications that exert specific hemodynamic effects. Furthermore, when they become critically ill, the management strategy can be counterintuitive. Commonly accepted, evidence-based management for the general population, such as fluid boluses in the setting of sepsis, can be harmful to this patient cohort. Often, these patients require highly specialized care at tertiary and quaternary centers, which necessitates critical care transport. Therefore, it is important that critical care transport crews understand the distinctive pathophysiology and management of critically ill patients with PAH.