A Comparison of Continuous Intravenous Epoprostenol (Prostacyclin) with Conventional Therapy for Primary Pulmonary Hypertension

Barst, Robyn J.; Rubin, Lewis J.; Long, Walker; McGoon, Michael D.; Rich, Stuart; Badesch, David B.; Groves, Bertron Μ.; Tapson, Victor F.; Bourge, Robert C.; Brundage, Bruce H.; Koerner, Spencer K.; Langleben, David; Keller, Cesar A.; Murali, Srinivas; Uretsky, Barry F.; Clayton, Linda M.; Jöbsis, Maria M.; Blackburn, Shelmer D.; Shortino, Denise; Crow, James W.

doi:10.1056/nejm199602013340504

Cited by 2,457 publications

(1,649 citation statements)

References 29 publications

Supporting

Mentioning

1,522

Contrasting

Unclassified

Order By: Relevance

“…Second prospective randomised open trial reported improvement in 6MWT by 32 m, in the epoprostenol group after 12 weeks of therapy. 40 Survival advantage was also demonstrated in this trial in the group of patients on epoprostenol. Following these studies, intravenous epoprostenol was approved in USA and France for treatment of iPAH for those in functional class III or IV.…”

Section: Epoprostenolsupporting

confidence: 59%

“…To date it is the only medication that has a mortality benefit. 40 Intravenous epoprostenol is now considered as a first line therapy in the western world. In a survey collected from 19 centres in the US, it was shown that more than two-thirds of patients with iPAH who were treated with intravenous epoprostenol could be taken off the list for lung transplantation.…”

Section: Epoprostenolmentioning

confidence: 99%

“…The incidence of catheter related sepsis has been reported to be 0.1-0.4 per patient-year. 40 Severe catheter related sepsis has resulted in death in some cases. Delivery system malfunction can be the other serious complication resulting in sudden, sometimes fatal decompensation due to interruption of drug supply.…”

Section: Epoprostenolmentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary hypertension—“state of the art” management in 2012

Saxena

2012

Indian Heart Journal

View full text Add to dashboard Cite

A B S T R A C TPulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not change the natural history of the disease. However, over the years, improvement in understanding the pathogenesis has resulted in the development of targeted approaches to the treatment of PAH. Survival advantage has also been shown with some of the pharmacologic agents. This review article discusses the current management strategy for PAH with special emphasis on an idiopathic variety, in an Indian context.

show abstract

Section: Epoprostenolsupporting

confidence: 59%

Section: Epoprostenolmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary hypertension—“state of the art” management in 2012

Saxena

2012

Indian Heart Journal

View full text Add to dashboard Cite

show abstract

“…[1][2][3][4][5][6] However, epoprostenol has several inherent drawbacks. Epoprostenol is administered through continuous intravenous infusion, and its dosing and regulation is complex.…”

Section: Introductionmentioning

confidence: 99%

Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan

Ivy

Doran

Claussen

et al. 2004

The American Journal of Cardiology

View full text Add to dashboard Cite

In 7 of 8 children with idiopathic pulmonary arterial hypertension treated with intravenous epoprostenol for > 1 year, concomitant use of bosentan allowed a reduction of epoprostenol and decreased its associated side effects without deterioration of clinical and hemodynamic parameters. In 3 children with normal or near-normal pulmonary artery pressure on epoprostenol, the addition of bosentan allowed discontinuation of epoprostenol and stabilization of hemodynamics for up to 1 year.Long-term therapy with epoprostenol, a potent prostacyclin and short-acting vasodilator, has been shown to improve hemodynamics, exercise capacity, and survival in adults and children with pulmonary hypertension. [1][2][3][4][5][6] However, epoprostenol has several inherent drawbacks. Epoprostenol is administered through continuous intravenous infusion, and its dosing and regulation is complex. Endothelin-1, a potent vasoconstrictor peptide, is increased in the pulmonary arteries of patients with pulmonary hypertension 7 ; endothelin levels are also increased and correlated with the severity of disease in adults with idiopathic primary pulmonary arterial hypertension (IPAH). 8 Bosentan, a dual endothelin receptor antagonist, lowers pulmonary artery pressure and resistance and improves exercise tolerance in adults with pulmonary arterial hypertension. 9 In children with pulmonary arterial hypertension related to congenital heart disease or IPAH, bosentan lowered pulmonary pressure and resistance and was well tolerated. 10 A recent case series has suggested that epoprostenol may be withdrawn from a select group of adult patients with normal pulmonary pressure. 11 Our study was undertaken to determine whether the addition of bosentan concomitant with stepwise epoprostenol dose reductions could decrease the severity of side effects experienced with epoprostenol while maintaining hemodynamic stability in children with IPAH.After institutional review board approval, informed consent, and assent where appropriate, patients were enrolled in a longitudinal follow-up study. Pediatric patients with IPAH in World Health Organization (WHO) functional class II or III, who received continuous epoprostenol therapy for > 1 year at the Pediatric Pulmonary Hypertension Program of the Children's Hospital in Denver, Colorado, were recruited (Table 1). All known causes of pulmonary hypertension were excluded, including congenital heart disease and lung disease. All patients had severe, life-threatening pulmonary hypertension before initiation of epoprostenol (Table 2). NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author Manuscript dose for children 10 to 20 kg was 31.25 mg twice daily, for children 20 to 40 kg, the dose was 62.5 mg twice daily, and for children >40 kg, the dose was 125 mg twice daily. Epoprostenol doses were decreased 1 to 2 ng/kg/min weekly, depending on stable or decreasing right ventricular peak systolic pressure as determined by echocardiography and/ or cardiac catheterization. Patients remained on concomitant medi...

show abstract

“…Prostacyclin has vasodilatory, anti-proliferative, anti-inflammatory and antithrombotic properties and is therefore an important target substance in PAH-specific therapy [1] . Epoprostenol was the first specific therapy approved for the treatment of PAH, after showing a positive effect on survival [2] . Epoprostenol is chemically unstable with a short biological half-life of 3-5 min, and must be continuously administered by intravenous (IV) infusion using an external pump and indwelling central venous catheter.…”

Section: Introductionmentioning

confidence: 99%

Safety, tolerability and clinical effects of a rapid dose titration of subcutaneous treprostinil therapy in pulmonary arterial hypertension: a prospective multi-centre trial.

et al. 2016

View full text Add to dashboard Cite

arterial hypertension on stable treatment with oral pulmonary arterial hypertension-approved drugs (90% on dual combination therapy) were included. Patients achieved a median treprostinil dosage of 35.7 ng/kg/min after 16 weeks. A good overall safety profile was demonstrated with 3 patients (8%) withdrawing due to infusion site pain, which occurred in 97% of patients. After 16 weeks, median 6-min walking distance, cardiac index, pulmonary vascular resistance, and tricuspid annular plane systolic excursion improved. Conclusions: Rapid up-titration of subcutaneous treprostinil was well tolerated, achieving a clinically effective dose associated with improvement of exercise capacity and haemodynamics after 16 weeks. A rapid dose titration regimen and proactive infusion site pain management may improve the handling of this therapy and contribute to better treatment outcome. © 2016 S. Karger AG, Basel Key Words Treprostinil · Pulmonary arterial hypertension · Subcutaneous infusionAbstract Background: Subcutaneous treprostinil has dose-dependent beneficial effects in patients with severe pulmonary arterial hypertension, but adverse effects like infusion site pain can lead to treatment discontinuation. Objectives: The objective of this study was to evaluate safety, tolerability and clinical effects of a rapid up-titration dosing regimen of subcutaneous treprostinil using proactive infusion site pain management. Methods: Effects of rapid up-titration dosing regimen on tolerability and clinical parameters were evaluated in this 16-week, open-label multi-centre study. Results: Thirty-nine patients with idiopathic or heritable pulmonary

show abstract

A Comparison of Continuous Intravenous Epoprostenol (Prostacyclin) with Conventional Therapy for Primary Pulmonary Hypertension

Abstract: As compared with conventional therapy, the continuous intravenous infusion of epoprostenol produced symptomatic and hemodynamic improvement, as well as improved survival in patients with severe primary pulmonary hypertension.

Cited by 2,457 publications

References 29 publications

Pulmonary hypertension—“state of the art” management in 2012

Pulmonary hypertension—“state of the art” management in 2012

Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan

Safety, tolerability and clinical effects of a rapid dose titration of subcutaneous treprostinil therapy in pulmonary arterial hypertension: a prospective multi-centre trial.

Contact Info

Product

Resources

About