Abstract
Pulmonary arterial hypertension is a rare and progressive disease with significant morbidity and mortality risk. Several medications targeting three major disease pathways are approved for treatment. However, the management of pulmonary arterial hypertension pharmacotherapies in a patient admitted to an intensive care unit poses unique challenges. Factors such as intubation and altered mental status may prevent the continuation of home oral and/or inhaled therapy, and the progression of the disease may require escalation of therapy. This review will focus on practical management strategies for the continuation of home pulmonary arterial hypertension pharmacotherapy and escalation of therapy.