Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial

Iversen, Kasper; Jensen, Annette Schophuus; Jensen, Tim; Vejlstrup, Niels; Søndergaard, Lars

doi:10.1093/eurheartj/ehq011

Cited by 171 publications

(106 citation statements)

References 34 publications

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“…All three PDE-5is approved for the treatment of erectile dysfunction-sildenafil, tadalafil and vardenafil-cause significant pulmonary vasodilation, with maximum effects observed after 60, 75-90 and 40-45 minutes, respectively [204]. [205][206][207][208]. An RCT addressing the effects of adding sildenafil to epoprostenol showed improvements after 12 weeks in 6MWD and time to clinical worsening.…”

Section: Phosphodiesterase Type 5 Inhibitors and Guanylate Cyclase Stmentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

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Section: Phosphodiesterase Type 5 Inhibitors and Guanylate Cyclase Stmentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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“…While patients with CHD-PAH and closed defects can be treated analogously to iPAH patients [53], the use of combination therapy is less well founded on evidence in the ES population. A prospective, randomized double-blind crossover study in 21 ES patients, failed to show a significant improvement in exercise capacity after addition of sildenafil to bosentan monotherapy [53,65]. However, these were stable ES patients and, therefore, the study does not adequately reflect routine clinical practice of initiating combination therapy in patients deteriorating on oral monotherapy.…”

Section: Eras and Pdesmentioning

confidence: 99%

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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“…There are very few randomized data on specific pulmonary therapies in ES, with further support from a number of prospective, open-label, and retrospective studies. [51][52][53][54] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. In a systematic review of literature and reanalysis of data in treatment-naïve patients with ES, Diller et al found that after adjusting for immortal time bias, the 10-year mortality in 1,131 patients from 12 studies was 30%-40% and survival prospects have not considerably improved over the last decade in untreated patients.…”

Section: Disease-specific Targeted Pah Therapymentioning

confidence: 99%

“…When combined with sildenafil, there was no further improvement in walk distance; however, there was an increase in oxygen saturations at rest. 54 In a prospective open-label study, D'Alto et al also studied the combined effect of bosentan and sildenafil in ES patients and reported an improvement in functional class. 85 Interestingly, Diller et al showed no survival benefit on direct comparison of patients with combination therapy versus monotherapy.…”

Section: Combination Therapymentioning

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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Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial

Abstract: In Eisenmenger syndrome, treatment with bosentan significantly improved walking distance, pulmonary blood flow, and PVR. Adding sildenafil to bosentan did not significantly improve walking distance but did increase saturation at rest. http://www.ClinicalTrial.gov: NCT00303004.

Cited by 171 publications

References 34 publications

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

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

Eisenmenger syndrome: current perspectives

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