Abstract
Pulmonary arterial hypertension (PAH) is commonly treated with pulmonary arteriolar vasodilator therapy. When a patient on PAH medication is admitted to intensive care, determining how to manage their medication during the critical illness is often complicated. There may be considerations related to the inability to take medication by mouth, related to acute renal failure or acute liver injury, related to altered mental status or delirium, or related to hypotension and bacteremia. Decisions of how to manage these medications can have a major impact on the patient’s clinical course. Presently, provider experience is the major tool in navigating the decisions regarding these medications. In this review, we offer our recommendations of how to manage PAH patients with critical illness who are on PAH medications. These recommendations include how to deliver medications via feeding tubes, how to dose medications in the setting of acute renal failure or acute liver failure, and how to manage medications during hypotension or when a tunneled catheter needs to be removed.
Highlights
Pulmonary arterial hypertension (PAH) is a progressive disorder of the pulmonary circulation, which leads to right ventricular failure and death
Many patients with PAH die in an ICU setting [1,2], and the reported prevalence of PAH may have increased from the 1980s, when a National Institutes of Health registry enrolled less than 200 patients [3], to the present, with more than 3,500 patients enrolled in the US-based Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) [4]
We offer our single-center experience with PAH therapies in the ICU setting, as well as our recommended approach to their use during ICU admission
Summary
Pulmonary arterial hypertension (PAH) is a progressive disorder of the pulmonary circulation, which leads to right ventricular failure and death. Hold or reduce dose if patient presents with significant acute liver injury and hypotension Probably safe for use in patients with liver dysfunction; consider dosage reduction if patient is hypotensive and is experiencing increased side effects (flushing, headache, jaw pain); do not abruptly discontinue therapy. Exposure is increased in patients with hepatic insufficiency; if patient presents with acute liver injury and signs of increased drug exposure (hypotension, headache, flushing, jaw pain, and so forth), increase dosing interval and/or decrease inhalations per treatment until symptoms subside. Patients with PAH are prone to developing congestive hepatopathy due to the likelihood of right ventricular failure in this population Medications such as bosentan have been implicated in drug-induced hepatic injury, including cases of severe hepatotoxicity [13,47].
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