Management of Pulmonary Arterial Hypertension in the ICU

Patients with pulmonary arterial hypertension (PAH) who are admitted to the intensive care unit (ICU) pose a challenge to the multidisciplinary health-care team due to the complexity of the pathophysiology of their disease state and the medication considerations that must be made to appropriately manage them. PAH is a progressive disease with the majority of patients ultimately dying as a result of right ventricular (RV) failure. During an acute decompensation, patients must be appropriately managed to optimize volume status, RV function, cardiac output, and systemic perfusion, while treating the underlying cause of the exacerbation. During times of critical illness, the ability to administer medications approved for use in PAH can be impacted by end-organ damage, hemodynamic instability, new drug interactions, or available dosage forms. Balancing the multimodal treatment approach needed to manage an acute exacerbation and the pharmacokinetic and administration concerns impacting baseline PAH therapy as a result of critical illness requires an expert multiprofessional PAH team. The purpose of this review is to evaluate specific management considerations for critically ill patients with PAH in the ICU.