Effectiveness of Transition from Intravenous Epoprostenol to Oral/Inhaled Targeted Pulmonary Arterial Hypertension Therapy in Pediatric Idiopathic and Familial Pulmonary Arterial Hypertension

Melnick, Laura; Barst, Robyn J.; Rowan, Cherise A.; Kerstein, Diane; Rosenzweig, Erika B.

doi:10.1016/j.amjcard.2009.12.075

Cited by 38 publications

(35 citation statements)

References 20 publications

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“…This subset of children may eventually be transitioned from intravenous to oral therapy with close monitoring. 315,316 However, this should be considered only in a pediatric PH center with significant experience in treating children with PAH owing to the potential for significant adverse response in some children who may not be candidates for weaning from intravenous epoprostenol to oral/inhaled dugs. Inhaled epoprostenol has been used in the critical care setting.…”

Section: Pgi 2 Analogsmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

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949

405

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Abstract-Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension. Key Words: AHA Scientific Statements ◼ bronchopulmonary dysplasia ◼ congenital diaphragmatic hernia ◼ congenital heart disease ◼ genetics ◼ persistent pulmonary hypertension of the newborn ◼ sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.This document was approved by the American Heart Association Science Advisory and Coordinating Committee on May 12, 2015, the American Heart Association Executive Committee on July 22, 2015, and the American Thoracic Society on July 24, 2015.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIR.0000000000000329/-/DC1. The American Heart Association requests that this document be cited as follows: Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Cardiovascular Surgery and Anesthesia, and the American Thoracic Society. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132:2037-2099 Copies: This document is available on the World Wide Web site of the American Heart Associat...

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Section: Pgi 2 Analogsmentioning

confidence: 99%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

Self Cite

949

405

View full text Add to dashboard Cite

show abstract

“…Although uncommon, children with a dramatic sustained response to epoprostenol may be weaned to oral or inhaled therapy if hemodynamics return to near normal values. (33, 34) A room temperature formulation is FDA approved for use in adults, but has not been studied in children.…”

Section: Prostacyclinsmentioning

confidence: 99%

Advances in pediatric pulmonary arterial hypertension

Ivy

2012

Current Opinion in Cardiology

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Purpose of Review Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality in children. Approved medications for the treatment of adult PAH have been used to treat children but evidence based treatment algorithms for children are lacking. Recent Findings Pediatric PAH registries have begun to define the incidence and prevalence of idiopathic PAH and PAH associated with congenital heart disease. A pediatric specific classification of pulmonary hypertensive vascular disease has been proposed. Furthermore, the first randomized placebo-controlled trial of type-5 phosphodiesterase therapy in treatment naïve children with PAH has been completed and reported. This trial highlights the importance of the difficulties of performing clinical trials children with targeted PAH therapy as well as the importance of long-term follow-up of adverse events. Summary Classification, clinical trials, and therapy for children with PAH must take into account the unique aspects of PAH in children.

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“…[122] Patients treated with intravenous epoprostenol that showed marked improvement in hemodynamics have also been successfully transitioned to oral or inhaled targeted PAH therapy without deterioration of clinical and hemodynamic parameters. [126]…”

Section: Targeted Pharmacological Therapiesmentioning

confidence: 99%

“…[171–174] In adult patients, combination therapy has also allowed reduction of epoprostenol dosing and subsequent decrease in prostacyclin side effects with stabilization of hemodynamics. [126] Its use children is increasing as most patients in the REVEAL registry received combination therapy for pediatric PAH. [7] In the limited number of studies that have included pediatric patients, combination therapy has proven to be safe and effective.…”

Section: Combination Therapymentioning

confidence: 99%

Drug Treatment of Pulmonary Hypertension in Children

Vorhies

Ivy

2013

Pediatr Drugs

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Pulmonary arterial hypertension (PAH) is a rare disease in infants and children that is associated with significant morbidity and mortality. The disease is characterized by progressive pulmonary vascular functional and structural changes resulting in increased pulmonary vascular resistance and eventual right heart failure and death. In the majority of pediatric patients, PAH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Although treatment of the underlying disease and reversal of advanced structural changes has not yet been achieved with current therapy, quality of life and survival have been improved significantly. Targeted pulmonary vasodilator therapies, including endothelin receptor antagonists, prostacyclin analogues and phosphodiesterase type 5 inhibitors, have demonstrated hemodynamic and functional improvement in children. The management of pediatric PAH remains challenging as treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts. This article reviews the current drug therapies and their use in the management of PAH in children.

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Effectiveness of Transition from Intravenous Epoprostenol to Oral/Inhaled Targeted Pulmonary Arterial Hypertension Therapy in Pediatric Idiopathic and Familial Pulmonary Arterial Hypertension

Cited by 38 publications

References 20 publications

Pediatric Pulmonary Hypertension

Pediatric Pulmonary Hypertension

Advances in pediatric pulmonary arterial hypertension

Drug Treatment of Pulmonary Hypertension in Children

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