Transition from intravenous or subcutaneous prostacyclin therapy to inhaled treprostinil in patients with pulmonary arterial hypertension: a retrospective case series

2014 ◽  
Vol 39 (5) ◽  
pp. 496-500 ◽  
Author(s):  
C. Y. Enderby ◽  
M. Soukup ◽  
M. Al Omari ◽  
T. Zeiger ◽  
C. Burger
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Case Series ◽  
Retrospective Case ◽  
Retrospective Case Series ◽  
Pulmonary Arterial

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

2022 ◽  
Vol 27 ◽  
pp. 107424842110690
Author(s):  
Heather Torbic ◽  
Benjamin Hohlfelder ◽  
Sudhir Krishnan ◽  
Adriano R. Tonelli
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Extracorporeal Membrane Oxygenation ◽  
Arterial Hypertension ◽  
Case Series ◽  
Retrospective Case ◽  
Bridge To Transplant ◽  
Membrane Oxygenation ◽  
Drug Concentrations ◽  
Pulmonary Arterial ◽  
Va Ecmo

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.

scholarly journals Transition from Intravenous Epoprostenol to Oral or Subcutaneous Therapy in Pulmonary Arterial Hypertension: A Retrospective Case Series and Systematic Literature Review

2011 ◽  
Vol 18 (3) ◽  
pp. 157-162 ◽  
Author(s):  
Kirily Park ◽  
David Ostrow ◽  
Robert D Levy ◽  
John Swiston
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Case Series ◽  
Cochrane Collaboration ◽  
Retrospective Case ◽  
Period Range ◽  
Ovid Medline ◽  
Pulmonary Arterial ◽  
The Cochrane Collaboration

BACKGROUND: Intravenous epoprostenol, a prostaglandin analogue, has been a mainstay of therapy for patients with advanced pulmonary arterial hypertension (PAH) since the early 1990s. This medication has multiple side effects, and sudden discontinuation is potentially associated with severe sequelae. Several recent case series have described the transition from intravenous to newer oral or subcutaneous therapies. A case series detailing the authors’ experience with such transitions, and a systematic lierature review is presented.METHODS: All consecutive PAH patients seen at the Vancouver Pulmonary Hypertension Clinic (Vancouver, British Columbia) between June 1995 and July 2009 were reviewed for cases in which weaning or transition from intravenous epoprostenol was attempted. The Cochrane Collaboration, Cochrane Register of Controlled Trials, Journals@Ovid, MEDLINE, EMBASE and Papers First were searched using predefined key words for publications describing transition of PAH patients from parenteral prostanoids to oral or subcutaneous agents.RESULTS: Of the six patients who attempted, all transitioned successfully to oral or subcutaneous agents, having been on intravenous epoprostenol for a mean of 3.8 years (range 1.8 to 9.75 years). Five are living, surviving a mean of 5.5 years after transition. The literature search yielded nine studies and, of 127 patients described, 82 transitioned successfully. The length of pretransition prostanoid treatment (range 1.7 to 7.6 years) and the post-transition follow-up period (range two months to 70 months) were shorter than for patients described in the present study.CONCLUSIONS: Given the rarity of PAH, the absolute numbers of patients transitioned from intravenous epoprostenol are still low. With the advent of new therapies, these numbers will hopefully increase; continued study is necessary to identify factors that are predictive of success.

Transition From Subcutaneous or Inhaled Treprostinil to Oral Treprostinil at Home in Patients With Pulmonary Arterial Hypertension: A Retrospective Case Series

2017 ◽  
Vol 31 (2) ◽  
pp. 163-166 ◽  
Author(s):  
Kimberly A. Ackerbauer ◽  
Rajive Tandon
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Hospital Setting ◽  
Case Series ◽  
Common Adverse Event ◽  
Functional Class ◽  
World Health ◽  
Retrospective Case ◽  
Pulmonary Arterial ◽  
At Home

Pulmonary arterial hypertension (PAH) is a progressive condition that can lead to right ventricular failure and death. Treprostinil is a prostacyclin analogue that has proven clinical efficacy in patients with PAH. Difficulties in the administration of inhaled and parenteral prostacyclins led to the development of extended-release treprostinil diolamine for oral use. Limited data exist on the transition to oral treprostinil. The purpose of this case series is to describe the transition from subcutaneous or inhaled treprostinil to oral treprostinil in the outpatient setting. With the current availability of oral prostacyclins and prostacyclins analogues, most transitions to oral therapy are done in the hospital setting resulting in increased cost and risk of hospital-acquired infections. Four patients on background phosphodiesterase type 5 therapy with baseline World Health Organization functional class (WHO FC) II PAH were transitioned at home. Three of the 4 patients were safely transitioned as outpatients and maintained WHO FC II status at 10 and 12 months. The fourth patient had worsening right heart failure and was admitted within 2 months of the transition and started back on parenteral prostacyclin therapy. The 6-minute walk distance (6MWD) increased in patients transitioning from inhaled therapy and decreased in the patient transitioning from subcutaneous therapy. The most common adverse event was nausea.

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

scholarly journals EXPRESS: The transition from Parenteral Prostacyclins to Selexipag: Safety and Feasibility in Select Patients

2021 ◽  
pp. 204589402110366
Author(s):  
Nael Aldweib ◽  
Nate Verlinden ◽  
Hayah Kassis-george ◽  
Amresh Raina
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Case Series ◽  
Outpatient Setting ◽  
Limited Data ◽  
Standardized Protocol ◽  
Pulmonary Arterial

There is limited data regarding the feasibility of transitioning from intravenous prostacyclins to selexipag in pulmonary arterial hypertension (PAH) patients. We present a case series of successful transitions from intravenous prostacyclins to selexipag in the majority of carefully selected five stable PAH patients using a standardized protocol in the outpatient setting.

scholarly journals Replacing a phosphodiesterase-5 inhibitor with riociguat in patients with connective tissue disease-associated pulmonary arterial hypertension: a case series

2017 ◽  
Vol 7 (3) ◽  
pp. 741-746 ◽  
Author(s):  
Amresh Raina ◽  
Raymond L. Benza ◽  
Harrison W. Farber
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Clinical Response ◽  
Connective Tissue Disease ◽  
Case Series ◽  
Response To Treatment ◽  
Tolerability Profile ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial

Patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-PAH-CTD) such as systemic sclerosis (SSc) have a poorer response to treatment and increased mortality compared with patients with idiopathic PAH. Current treatment options for PAH-CTD include prostanoids, phosphodiesterase type-5 inhibitors (PDE-5i), endothelin receptor antagonists, and the soluble guanylate cyclase stimulator riociguat. In this case series, we describe three patients with PAH-CTD related to limited scleroderma who were switched from a PDE-5i to riociguat due to insufficient clinical response. The switch to riociguat was associated with an improvement in respiratory and hemodynamic parameters and a favorable tolerability profile. These cases demonstrate that switching to riociguat is a therapeutic option in patients with PAH-CTD who have not achieved a satisfactory clinical response to a PDE-5i.

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