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.

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Background: A high prevalence of Pulmonary Hypertension (PH) in sickle cell disease (SCD) has been reported in several studies. However, few studies that describe the hemodynamics have actually measured pulmonary artery occlusive pressure (PAOP). Furthermore, even PAOP has been shown to be unreliable in discriminating pulmonary artery hypertension from pulmonary venous hypertension. We prospectively examined the accuracy of PAOP using simultaneous left ventricular end diastolic pressure (LVEDP) measurement as the gold standard. Hypothesis: In patients with SCD, PAOP may not reflect LVEDP leading to over-diagnosis of PAH. Methods: We prospectively examined hemodynamic data on 26 patients with SCD, at a large academic center, from 2009 through 2011. These patients underwent simultaneous PAOP and LVEDP measurements. Results: We tested 106 adult SCD patients with 2-D Echocardiography for evaluation of PH. Of the 106 patients, 43 (41%) were found to have a tricuspid regurgitant jet velocity ≥ 2.5 m/sec. Of these 43, 26 patients underwent right heart catheterization (RHC) and simultaneous measurement of LVEDP. Twelve patients among the 106 (11.1%) patients were found to have PH. Eight of these (7.5 %) had PAH by PAOP criteria but only 4/106 (3/7%) had PAH by LVEDP criteria. PAOP significantly underestimated the LVEDP in both the PH group and group with normal hemodynamics (p=0.00004). BNP, and creatinine levels significantly increased in PAH group (p< 0.02, 0.01, 0.03). PAOP misclassified 50% of patients in this sickle cell disease cohort. In conclusion, PAOP may underestimate LVEDP in sickle cell patients with pulmonary hypertension and can lead to misclassification.