Breathe-5: Bosentan Improves Hemodynamics and Exercise Capacity in the First Randomized Placebo-Controlled Trial in Eisenmenger Physiology

Galiè, Nazzareno; Beghetti, Maurice; Granton, John; Berger, Rolf M. F.; Lauer, Andrea; Chiossi, Eleonora; Landzberg, Michael J.

doi:10.1378/chest.128.4_meetingabstracts.496s

Cited by 20 publications

(12 citation statements)

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“…346 Bosentan has been studied in adult patients with ES in a placebo-controlled trial, showing few adverse events and improved exercise capacity and hemodynamics. 288 Other studies have further demonstrated beneficial effects of bosentan in patients with ES. 351,352 A specific water-soluble, cloverleaf formulation has been recently approved for use in children with PAH in Europe.…”

Section: Et Receptor Antagonistsmentioning

confidence: 96%

“…388,376,432 In a randomized, placebo-controlled trial of ES patients in WHO functional class III, bosentan therapy reduced PVR and mPAP and increased 6MWD by 53.1 m without worsening gas exchange. 288 Children but not adults with ES developed a progressive decline in exercise capacity during prolonged bosentan therapy. 349 Sildenafil therapy improves functional class and hemodynamic parameters in patients with ES.…”

Section: Eisenmenger Syndromementioning

confidence: 96%

See 1 more Smart Citation

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

931

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: Et Receptor Antagonistsmentioning

confidence: 96%

Section: Eisenmenger Syndromementioning

confidence: 96%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

931

405

View full text Add to dashboard Cite

show abstract

“…This was accompanied by an improvement in WHO functional class in patients on bosentan. Furthermore, a reduction of indexed PVR could be demonstrated [55]. This study was followed by a 6-month open-label extension study, demonstrating a significant improvement in 6MWD in former placebo patients switched to open-label bosentan [56].…”

Section: Eras and Pdesmentioning

confidence: 82%

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Radke

Diller

Baumgartner

2013

Expert Review of Cardiovascular Therapy

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“…24 Unlike idiopathic PAH, the cause of PAH in CHD congenital cardiac surgery, with crises occurring in 0.75%. 7 The mortality in those suffering a crisis, nonetheless, remains high at 20%, and pulmonary vascular disease is identified as a major contributor to length of stay in hospital, and the need for prolonged mechanical ventilation (see a recent review by Adatia and Beghetti for further detail, as this is not the scope of this review.…”

Section: Non-operable Preeisenmengermentioning

confidence: 99%