A Review of Pulmonary Arterial Hypertension Treatment in Extracorporeal Membrane Oxygenation: A Case Series of Adult Patients

Abstract

Background: Little data is published describing the use of medications prescribed for pulmonary arterial hypertension (PAH) in patients receiving extracorporeal membrane oxygenation (ECMO). Even though many patients with PAH may require ECMO as a bridge to transplant or recovery, little is reported regarding the use of PAH medications in this setting. Methods: This retrospective case series summarizes the clinical experience of 8 patients with PAH receiving ECMO and reviews medication management in the setting of ECMO. Results: Eight PAH patients, 5 of whom were female, ranging in age from 21 to 61 years old, were initiated on ECMO. Veno-arterial (VA) ECMO was used in 4 patients, veno-venous (VV) ECMO and hybrid ECMO configurations in 2 patients respectively. Common indications for ECMO included cardiogenic shock, bridge to transplant, and cardiac arrest. All patients were on intravenous (IV) prostacyclin therapy at baseline. Refractory hypotension was noted in 7 patients of whom 5 patients required downtitration or discontinuation of baseline PAH therapies. Three patients had continuous inhaled epoprostenol added during their time on ECMO. In patients who were decannulated from ECMO, PAH therapies were typically resumed or titrated back to baseline dosages. One patient required no adjustment in PAH therapy while on ECMO. Two patients were not able to be decannulated from ECMO. Conclusion: The treatment of critically ill PAH patients is challenging given a variety of factors that could affect PAH drug concentrations. In particular, PAH patients on prostacyclin analogues placed on VA ECMO appear to have pronounced systemic vasodilation requiring vasopressors which is alleviated by temporarily reducing the intravenous prostacyclin dose. Patients should be closely monitored for potential need for rapid titrations in prostacyclin therapy to maintain hemodynamic stability.

Hybrid extracorporeal membrane oxynegation in pediatric intensive care patients: A single center experience: More is better?

“…The incidence of neurologic complications in the studies of Vogel et al (12 adults) and Werner et al (8 pediatric patients) was 24.9% and 29%, respectively. 14 In the adult study of Vogel et al, there were no data related to the cannulation site. In the study of Werner et al, 9 there was no carotid cannulation in the group with neurologic complications.…”

“…Even though cumulative oxygen saturation does not improve with transforming V-AV ECMO, by using a reinfusion cannula in the internal jugular vein (IJV), hemodynamic stability and oxygenized blood distribution to an upper extremity can be secured. 14 Cannulation was performed over the carotid artery in three patients, and seizures were observed in only one. Brain death did not develop in any patients who underwent hybrid ECMO.…”

Extracorporeal Life Support in Pulmonary Hypertension: Practical Aspects

“…No general recommendations can be made as these decisions require a careful assessment of the individual hemodynamic situation. 11 The goal is to avoid systemic hypotension while maintaining pulmonary perfusion.…”

“…There are some reports about the use of venovenous (v-v) ECMO in patients with PH, where both inflow and outflow are directed into the venous system. 11 12 13 14 V-v ECMO is widely used to treat patients with hypoxemic and/or hypercapnic lung failure. When v-v ECMO is considered in patients with PH and right heart failure, it has to be noted that this ECMO configuration does not unload the RV and does not support the systemic circulation.…”

ECMO in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension as a bridge to therapy

“…This may be an option in critically ill patients with PAH or CTEPH. However, evidence in this setting is scarce [6]. A multidisciplinary approach to determine a specific strategy in each case is crucial [7].…”