Safety and Efficacy of IV Treprostinil for Pulmonary Arterial Hypertension

Tapson, Victor F.; Gomberg‐Maitland, Mardi; McLaughlin, Vallerie V.; Benza, Raymond L.; Widlitz, Allison; Krichman, Abigail; Barst, Robyn J.

doi:10.1378/chest.129.3.683

Cited by 272 publications

(166 citation statements)

References 16 publications

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“…Treatment by either route improves the 6-minute walk distance in patients who are in WHO functional class III or IV. 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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“…78 Evidence on the intravenous administration of treprostinil in PAH-CHD is very limited. 79 Nonetheless, long-term use of treprostinil is felt to have similar survival benefits as intravenous epoprostenol. 80 Selexipag is one of the latest pulmonary therapies to be tested in PAH.…”

Section: Prostacyclin Analogs and Prostacyclin Receptor Agonistsmentioning

confidence: 99%

Eisenmenger syndrome: current perspectives

Nashat¹,

Kempny²,

McCabe³

et al. 2017

RRCC

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Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). It results from a cardiac defect allowing significant systemic-to-pulmonary (left-to-right) shunting, which triggers the development of pulmonary vascular disease (PVD) if the defect is not repaired in a timely fashion. Once severe PVD has developed, the defect cannot be repaired. With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. ES is a multisystem disorder causing chronic hypoxemia and reduced cardiac output resulting in significant morbidity and mortality. While lung (plus defect repair) or combined heart and lung transplantation is thought be the definitive treatment for ES, transplant organs are a limited resource and long-term results are still suboptimal. PAH pharmacotherapy was, until quite recently, largely directed at symptomatic relief and had no impact on morbidity and mortality. Targeted PAH therapies have recently been proven to be beneficial in various forms of PAH in terms of functional status, progression of disease, and prognosis. Data on the effect of PAH therapies in the ES cohort remain limited, but available studies demonstrate evidence of improvement in symptoms, exercise capacity, and some evidence of survival benefit. ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. However, local physicians working in cardiology, respiratory medicine, primary care, and emergency services are likely to encounter ES patients and need to be aware of the main issues and pitfalls in their care. The authors present an overview of the management of ES, focusing on the most common issues and complications.

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“…151 Data are not available to compare the efficacy of intravenous epoprostenol to treprostinil. However, Ivy and colleagues reported the successful transition of 13 children from intravenous epoprostenol to treprostinil without changes in exercise capacity or intolerable adverse effects.…”

Section: Nitric Oxide-cgmp Cascade: Inhaled Nitric Oxidementioning

confidence: 99%

Advances in the Management of Pediatric Pulmonary Hypertension

2011

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Pulmonary hypertension is a rare disease in neonates, infants, and children, and is associated with substantial morbidity and mortality. An adequate understanding of the controlling pathophysiologic mechanisms is lacking. Moreover, a minority of research is focused specifically on neonatal and pediatric populations. Although therapeutic options have increased over the past several decades, they remain limited. In advanced pulmonary hypertension, progressive pulmonary vascular functional and structural changes ultimately cause increased pulmonary vascular impedance, rightventricular failure, and death. Management includes the prevention and/or treatment of active pulmonary vasoconstriction, the support of right-ventricle function, treatment of the underlying disease (if possible), and the promotion of regressive remodeling of structural pulmonary vascular changes. Most currently available therapies augment or inhibit factors, or mediators of their downstream signaling cascades, that originate in the pulmonary vascular endothelium. These pathways include nitric-oxide/cyclic guanosine monophosphate (cGMP), prostacyclin, and endothelin-1. The ability to reverse advanced structural changes remains an as yet unattained goal. This paper reviews the epidemiology, pathophysiology, current treatments, and emerging therapies related to neonatal and pediatric pulmonary hypertension.

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Safety and Efficacy of IV Treprostinil for Pulmonary Arterial Hypertension

Cited by 272 publications

References 16 publications

Pulmonary Hypertension: Diagnosis and Management

Pulmonary Hypertension: Diagnosis and Management

Eisenmenger syndrome: current perspectives

Advances in the Management of Pediatric Pulmonary Hypertension

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