Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience

Sitbon, Olivier; Vonk‐Noordegraaf, Anton

doi:10.1183/16000617.0055-2016

Cited by 86 publications

(70 citation statements)

References 85 publications

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“…Of note, a synthetic PGI 2 (epoprostenol) was the first PAH therapy to be approved and is still considered to be the gold-standard treatment for PAH [24]. For patients in WHO FC IV, epoprostenol is the only therapy with class I recommendation and evidence level of A (the highest available according to the ESC/ERS guidelines) [9,10].…”

Section: Combination Therapy For Optimal Pah Managementmentioning

confidence: 99%

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Gaine

McLaughlin

2017

Eur Respir Rev

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Recent advances in the treatment of pulmonary arterial hypertension (PAH) have led to improved patient outcomes. Multiple PAH therapies are now available and optimising the use of these drugs in clinical practice is vital. In this review, we discuss the management of PAH patients in the context of current treatment guidelines and supporting clinical evidence. In clinical practice, considerable emphasis is placed on the importance of making treatment decisions guided by each patient's risk status, which should be assessed using multiple prognostic parameters. As PAH is a progressive disease, regular assessments are essential to ensure that any change in risk is detected in a timely manner and treatment is adjusted accordingly. With the availability of therapies that target three different pathogenic pathways, combination therapy is now the standard of care. For most patients, this involves dual combination therapy with agents targeting the endothelin and nitric oxide pathways. Therapies targeting the prostacyclin pathway should be added for patients receiving dual combination therapy who do not achieve a low-risk status. There is also a need for a holistic approach to treatment beyond pharmacological therapies. Implementation of all these approaches will ensure that PAH patients receive maximal benefit from currently available therapies.

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Section: Combination Therapy For Optimal Pah Managementmentioning

confidence: 99%

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Gaine

McLaughlin

2017

Eur Respir Rev

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“…S8). Ptgis is also a target gene for Epoprostenol--a drug used for treating pulmonary hypertension (39). The idiopathic pulmonary fibrosis-associated gene, Thbs1 (40)(41)(42), was highly expressed and more responsive to IH in myofibroblasts compared to other cell types.…”

Section: Pulmonary Disease-regulated Genes Provide Clinical Implicatimentioning

confidence: 99%

Short-term exposure to intermittent hypoxia leads to changes in gene expression seen in chronic pulmonary disease

Lee

Gulla

et al. 2020

Preprint

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Obstructive sleep apnea (OSA) results from intermittent episodes of airway collapse and hypoxia and is associated with a host of health complications including dementia, diabetes, heart failure, and stroke. Cellular mechanisms causing disease progression across multiple systems in OSA are unknown. Although it is known that pulmonary diseases share general mechanisms, such as systemic inflammation and oxidative stress, there is an incomplete understanding of the early-stage changes to the lung from OSA. Using intermittent hypoxia (IH) as a mouse model of OSA, we showed profound cell-type specific changes in genome-wide expression in the lung. With single-cell RNA analysis, we identified substantial similarities between lungs of mice exposed to IH and human lung tissue from patients with pulmonary disease--most notably pulmonary hypertension, COPD, and asthma. Many IH-responsive genes encode targets of drugs currently available to treat pulmonary disease. Present data provide insights into the initiation of specific cellular responses which drive disease progression in a model of OSA. This information can help direct therapies to the most relevant cells and molecular pathways.

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“…It has a short half-life of approximately 3 to 6 minutes in human blood, which necessitates administration via continuous IV infusion. 87 Epoprostenol needs to be administered via a permanent tunnel catheter through a portable infusion pump. Treatment with continuous infusion of epoprostenol has to be initiated by experienced teams.…”

Section: Epoprostenolmentioning

confidence: 99%

“…Treatment with continuous infusion of epoprostenol has to be initiated by experienced teams. 87 Freshly prepared solutions of epoprostenol must be administered within 8 to 12 hours at room temperature or within 24 hours if maintained at 2 to 8°C using ice packs. A temperature stable formulation of epoprostenol is now available allowing a 24-hour infusion and it does not require cooling with ice packs.…”

Section: Epoprostenolmentioning

confidence: 99%

“…94 The most common adverse events observed with epoprostenol are jaw pain, headache, diarrhea, pain in extremities, nausea, and vomiting. 87 Most serious complications include catheter-related infection, cellulitis, pneumothorax, and thrombosis. 87 In observational studies, sepsis was reported at a rate of 0.3 infections per patient per year.…”

Section: Epoprostenolmentioning

confidence: 99%

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Medical Treatment of Pulmonary Arterial Hypertension

Sahay

Humbert

Sitbon

2017

Semin Respir Crit Care Med

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Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease leading to right ventricular failure and death if left untreated. Over the past two decades, progress in the understanding of pathophysiological mechanisms of the disease has led to the development of medications targeting the three major pathways of endothelial dysfunction: prostanoids, endothelin-receptor antagonists, and phosphodiesterase type-5 inhibitors. Efficacy of PAH-targeted medications has been demonstrated in monotherapy through randomized clinical trials leading to their regulatory approval. However, despite the growing numbers of available PAH-targeted medications, many patients with PAH continue to deteriorate and the disease ultimately remains fatal. The availability of multiple classes of drugs targeting different pathophysiological pathways provides strong biological rationale for the use of combination therapy in PAH. Evidence to support this strategy is growing, and many studies have demonstrated that combination therapy, administered as either a sequential or an initial regimen, can improve long-term outcomes in PAH. Treatment strategy for PAH has thereby changed significantly over the past decade, combination therapy becoming progressively the gold standard of care in patients with PAH. This is underscored by the current European Society of Cardiology/European Respiratory Society guidelines, in which combination therapy now plays a central part in the treatment algorithm.

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Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience

Cited by 86 publications

References 85 publications

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Short-term exposure to intermittent hypoxia leads to changes in gene expression seen in chronic pulmonary disease

Medical Treatment of Pulmonary Arterial Hypertension

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