Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil

Barst, Robyn J.; Galiè, Nazzareno; Naeije, Robert; Simonneau, Gérald; Jeffs, Roger; Arneson, Carl; Rubin, Lewis J.

doi:10.1183/09031936.06.00044406

Cited by 299 publications

(246 citation statements)

References 29 publications

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“…62,63 Three-year survival of patients with IPAH treated with subcutaneous treprostinil monotherapy has been reported at 71%. 64 Efficacy of treprostinil administered by the intravenous route may be similar to that of epoprostenol; however, comparative survival data are lacking.…”

Section: Prostacyclin Analoguesmentioning

confidence: 99%

Pulmonary Hypertension: Diagnosis and Management

McGoon

Kane

2009

Mayo Clinic Proceedings

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Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. Effective management requires timely recognition and accurate diagnosis of the disorder and appropriate selection among therapeutic alternatives. Despite progress in treatment, obstacles remain that impede the achievement of optimal outcomes. The current article provides an overview of the pathobiologic mechanisms of pulmonary arterial hypertension, including genetic substrates and molecular and cellular mechanisms, and describes the clinical manifestations and classification of pulmonary arterial hypertension. The article also reviews established approaches to evaluation and treatment, with emphasis on the appropriate application of calcium channel blockers, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase 5 inhibitors. In addition, the authors discuss unresolved issues that may complicate patient management, such as the clinical importance of mild or exercise-related pulmonary arterial hypertension, and they identify avenues by which treatment may advance in the future through the use of combination treatment, outcomes assessment, and exploration of alternative pharmacologic strategies. Mayo Clin Proc. 2009;84(2):191-2075-HTT = 5-hydroxytryptamine transporter; BMPR2 = bone morphogenetic protein receptor, type II; FDA = Food and Drug Administration; IPAH = idiopathic pulmonary arterial hypertension; mPAP = mean pulmonary arterial pressure; PAH = pulmonary arterial hypertension; PASP = pulmonary arterial systolic pressure; PCWP = pulmonary capillary wedge pressure; PDE5 = phosphodiesterase 5; PH = pulmonary hypertension; TGF-β β β β β = transforming growth factor β β β β β; WHO = World Health Organization T he diagnosis, management, and pathobiologic mechanisms of pulmonary arterial hypertension (PAH) have been of intense interest during the past decade, in large part because of the development of effective treatments that have enhanced the outcome for patients. In the absence of effective treatments, patients with PAH had a median life expectancy of only 2.8 years. Current approaches to the evaluation and management of PAH and to the understanding of the underlying pathophysiologic mechanisms of this condition have been well outlined in recent reviews.

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Section: Prostacyclin Analoguesmentioning

confidence: 99%

Pulmonary Hypertension: Diagnosis and Management

McGoon

Kane

2009

Mayo Clinic Proceedings

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“…Adult data have shown the effectiveness of SCTre. 5,8 Transition from IV epoprostenol to SCTre has been reported without clinical deterioration in adults and children. 9,10 Adverse effects of the SC infusion include site pain in 89% to 92% of adults, and site reactions in 81% to 100%.…”

Section: Discussionmentioning

confidence: 99%

“…9,10 Adverse effects of the SC infusion include site pain in 89% to 92% of adults, and site reactions in 81% to 100%. 5,8 In adults, 23% discontinued SCTre owing to adverse effects, mainly site pain. 5 Typically this is managed with the use of a preferred site, frequent site changes, oral and topical analgesics, and rapid increases in dosage to goal.…”

Section: Discussionmentioning

confidence: 99%

“…5,8 In adults, 23% discontinued SCTre owing to adverse effects, mainly site pain. 5 Typically this is managed with the use of a preferred site, frequent site changes, oral and topical analgesics, and rapid increases in dosage to goal. 11 Levy et al reported on 8 children (ages 1.5 to 10 years, median 4 years) who were started on SCTre after failure of oral medications.…”

Section: Discussionmentioning

confidence: 99%

“…In the past, pediatric PAH specialists were reluctant to use subcutaneous treprostinil (SCTre) in young children because of the significant infusion site pain described in older patients. 5 This is the first reported use of SCTre in select infants who have PAH-CLD.…”

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Subcutaneous Treprostinil for Pulmonary Hypertension in Chronic Lung Disease of Infancy

et al. 2014

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Pulmonary arterial hypertension (PAH) associated with chronic lung disease of infancy can be a life-threatening disease affecting an increasing number of former premature infants. There is a need for improved delivery of targeted PAH therapies for this subgroup of patients who have severe and persistent PAH despite standard respiratory care for chronic lung disease. Currently infants who have severe PAH despite oral or inhaled therapy receive continuous intravenous prostanoid therapy (mostly epoprostenol), which is complicated because of the need for central venous access and associated catheter-related complications. We present a series of 5 infants who were successfully treated with a continuous infusion of subcutaneous treprostinil, which is a longeracting prostanoid with similar hemodynamic effects. There were improvements in echocardiographic assessment of right ventricular function and estimated pulmonary hypertension, and in respiratory support required within weeks of therapy. Unlike commonly in adults, these 5 infants had no instances of severe site erythema, bleeding, bruising, or infection. In our experience with 5 former extremely preterm infants who had PAH associated with chronic lung disease, subcutaneous treprostinil was safe, efficacious, and well tolerated. We believe that subcutaneous treprostinil can be beneficial in a select group of former premature infants who have chronic lung disease and severe pulmonary arterial hypertension who have not responded adequately to conservative therapies. Pediatrics 2014;134:e274-e278 AUTHORS:

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Advances in diagnosis and treatment in patients with pulmonary arterial hypertension

Park

2008

Cathet Cardio Intervent

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Pulmonary arterial hypertension (PAH) is a progressive disease marked by vasoconstriction and vascular remodeling within pulmonary arteries leading to right heart failure and death. Significant advances in understanding the pathobiology of the disease have identified three key pathways involved in progression of this disease, which are the endothelin pathway, the prostacyclin pathway, and the nitric oxide/cyclic guanosine monophosphate pathway. Echocardiogram is the best screening tool to obtain an estimation of the pulmonary artery systolic pressure but right heart catheterization remains the standard by which the diagnosis is made. There are currently six FDA approved therapies for PAH. The mechanistic rationale, evidence behind their use and side effect considerations in utilizing these therapies in PAH patients will be the focus of this review.

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Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil

Cited by 299 publications

References 29 publications

Pulmonary Hypertension: Diagnosis and Management

Pulmonary Hypertension: Diagnosis and Management

Subcutaneous Treprostinil for Pulmonary Hypertension in Chronic Lung Disease of Infancy

Advances in diagnosis and treatment in patients with pulmonary arterial hypertension

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