Epoprostenol discontinuation in patients with pulmonary arterial hypertension: a complex medical and social problem

Chebib, Nader; Cottin, Vincent; Taharo-Ag-Ralissoum, Martine; Chuzeville, Michel; Mornex, Jean‐François

doi:10.1177/2045893217753352

Cited by 6 publications

(5 citation statements)

References 14 publications

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“…These include the PGI2 analogs and PGI2 IP receptor agonist, selexipag [5]. The rapid discontinuation of this medication has catastrophic consequences, including rapid right heart failure and cardiogenic shock [2][3][4]. The potency of prostacyclin medications is the reason for the aggressive withdrawal symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…Despite being such a detrimental disease, pharmaceuticals have improved patient symptoms, quality of life, and survival. The abrupt disruption of these medications can result in rebound pulmonary hypertension, right heart failure, and cardiogenic shock [2][3][4]. Although PAH literature has increased over the years, there is little data about rebound PAH after the brief discontinuation of medications and how to restart them.…”

Section: Introductionmentioning

confidence: 99%

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Pause at Your Own Peril: A Case Series on Rebound Pulmonary Hypertension

Murali¹,

Khanal²,

Banerjee³

et al. 2022

Cureus

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Severe pulmonary arterial hypertension (PAH) is associated with high morbidity and mortality. Therapeutic approaches for intermediate-and high-risk pulmonary arterial hypertension have now shifted toward initial combination management, often including parenteral epoprostenol and iloprost and early assessment for a lung transplant. After the initiation of therapy, usually various combinations of different classes of medication, it is important to consider the potential interruption in therapy causing rebound PAH. We present two patients recently admitted to our hospital with rebound symptoms after interruption of their pulmonary vasodilator therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pause at Your Own Peril: A Case Series on Rebound Pulmonary Hypertension

Murali¹,

Khanal²,

Banerjee³

et al. 2022

Cureus

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“…Best practice guidance recommends avoiding abrupt discontinuation of PAH medications due to risk for rebound PH and even death. 20 -24 However, PAH medication management is particularly challenging in critically ill patients due to alterations in medication pharmacokinetics due to end-organ damage, changes in volume of distribution, and patient factors impacting medication absorption, distribution, metabolism, and excretion. Many of the medications approved for use in PAH patients are oral therapies which may present a challenge in critically ill patients who do not always have oral access or adequate enteral absorption due to high vasopressor requirements or impairments impacting gastrointestinal function.…”

Section: Treatment Of Pah In the Icumentioning

confidence: 99%

“…Patients who are admitted to the ICU on intravenous (IV) prostacyclins should have additional IV access in place to reduce the risk of abrupt discontinuation of therapy in the event of line malfunction, which can be life threatening. 24,40 For patients receiving subcutaneous (SC) treprostinil, consideration should be made to transition the patient to IV administration due to concerns for impaired SC absorption in critically ill patients, especially those in shock with high vasopressor requirements. Patients should be closely monitored as they are transitioned from SC to IV treprostinil as lower doses may be needed.…”

Section: Treatment Of Pah In the Icumentioning

confidence: 99%

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

Torbic

Hohlfelder

Krishnan

et al. 2022

J Cardiovasc Pharmacol Ther

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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.

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“…24 Given that parenteral PGI 2 analogues are the most potent medications for PAH, abrupt discontinuations can have catastrophic consequences, including the rapid development of acute right ventricular failure. [68][69][70] Drug inserts recommend avoiding abrupt withdrawal or sudden large reductions in dosing. No specific recommendations are provided in case of sudden cessation of the parenteral medications.…”

Section: Prostacyclin Analogues and Prostacyclin Ip Receptor Agonistmentioning

confidence: 99%

Treatment Discontinuation or Interruption in Pulmonary Arterial Hypertension

Narechania

Torbic

Tonelli

2019

J Cardiovasc Pharmacol Ther

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Pulmonary arterial hypertension (PAH) is a progressive disease, which can be potentially fatal. The management of a complex disease like PAH requires a multidisciplinary approach from a team consisting of physicians, nurses, social workers, and pharmacists. Adherence to PAH-specific therapy is one of the key factors in the management of this disease. Poor adherence to treatment is a common problem in PAH as it is in many chronic diseases. Management of medication interruptions is a challenge in patients with PAH that can lead to negative consequences. However, for most PAH-specific drugs, there are no clear guidelines on how to manage temporary or abrupt medication discontinuations. In this review, we summarized the available literature and provide suggestions on how to manage interruptions of PAH-specific therapies.

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Epoprostenol discontinuation in patients with pulmonary arterial hypertension: a complex medical and social problem

Cited by 6 publications

References 14 publications

Pause at Your Own Peril: A Case Series on Rebound Pulmonary Hypertension

Pause at Your Own Peril: A Case Series on Rebound Pulmonary Hypertension

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

Treatment Discontinuation or Interruption in Pulmonary Arterial Hypertension

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