Effect of bosentan therapy on ventricular and atrial function in adults with Eisenmenger syndrome. A prospective, multicenter study using conventional and Speckle tracking echocardiography

Rahman, Mohamed Y. Abd El; Rentzsch, Axel; Scherber, Philipp Robert; Mebus, Siegrun; Miera, Oliver; Balling, Gunter; Böttler, Petra; Dubowy, Karl-Otto; Farahwaschy, Birgit; Hager, Alfred; Kreuder, Joachim; Peters, Brigitte; Berger, Felix; Schulze‐Neick, Ingram; Abdul‐Khaliq, Hashim

doi:10.1007/s00392-014-0703-5

Cited by 12 publications

(7 citation statements)

References 29 publications

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“…4 Bosentan is an orally active, selective and competitive non-peptide dual endothelin receptor (both ET A and ET B ) antagonist and usually used in cure of PAH. 5 Bosentan is also being considered for treatment of other conditions such as Eisenmenger syndrome, 6 persistent pulmonary hypertension of the newborn, 7 digital ulcer prevention in patients with systemic sclerosis, 8 adolescent and adult patients who have undergone Fontan operation, 9 vascular remodeling and dysfunctional angiogenesis in diabetes 10 and possibly even depression. 11 For treatment of PAH, bosetan is currently administered at the daily dose of 125-250 mg. 12 The maximum plasma peak is seen within 3-5 h after oral intake and the half-life of drug is 5.4 h. 13 Moreover, the bioavailability of bosentan after oral administration is approximately low (50%) 14 and variability is seen in bosentan absorption which may get back to its poor water solubility.…”

Section: Introductionmentioning

confidence: 99%

D-optimal Design for Preparation and Optimization of Fast Dissolving Bosentan Nanosuspension

Ghasemian¹,

Motaghian²,

Vatanara³

2016

Adv Pharm Bull

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Section: Introductionmentioning

confidence: 99%

D-optimal Design for Preparation and Optimization of Fast Dissolving Bosentan Nanosuspension

Ghasemian¹,

Motaghian²,

Vatanara³

2016

Adv Pharm Bull

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“…Pretreatment with advanced therapies [17,18,19] for a su cient period to assess the hemodynamic and symptomatic response is strongly recommended before closure [13,14] .Supomo et al [20] described a ASD-ES female with highly symptomatic PAH(NYHA class III, mPAP 77 mmHg, PVR 4 Wood U) underwent occlusion successfully after oral beraprost for two years. After surgery her mPAP decrease to 38 mmHg with PVR of 2.52 Wood U. Hu et al [21] reported a ventricular septal defect (VSD)-ES patient with initial PVR of 18.84 Wood U underwent a successful operation after oral bosentan for 12 weeks, as a result of which her PVR decreased to 9.63 Wood U.…”

Section: Discussionmentioning

confidence: 99%

Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

Wang

Shen

et al. 2020

Preprint

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Background Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidence of benefit from targeted drug therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether the PDA in patients with ES can be treated with transcatheter closure (TCC).Methods Between August 2014 and July 2016, four out of fifteen PDA-ES patients whose Qp/Qs improved significantly and Qp/Qs > 1.5 after acute vasodilator testing with 100% oxygen were selected to receive TCC by diagnostic treatment and repair strategy. PAH-targeted drugs were prescribed before and after occlusion for all patients. Trial occlusion was performed before permanent closure.Results The first TCC failed after the initiation of PAH-targeted drugs for 6 months in four patients. After the medication was adjusted and extended to 12 months, TCC was performed for all without hemodynamic intolerances during perioperative period. Pulmonary artery systolic pressure (PASP) was significantly decreased (≥ 40%) immediately after TCC. During the follow-up period, there was a further decrease of PASP in two patients, the other two showed improved WHO functional class and six-minute walking distance although a worsening PASP.Conclusion Some selected PDA-ES patients with PVR < 15Wood U and Qp/Qs > 1.5 at baseline might benefit from TCC by diagnostic treatment and repair strategy and uninterrupted combination of PAH-targeted drugs pre- and post-occlusion play a crucial role.

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“…Treatment with advanced therapies [17,18,19] for a su cient period to assess the hemodynamic and symptomatic response is strongly recommended before closure [13,14] .Supomo et al [20] described a atrial septal defect(ASD)-ES female with highly symptomatic PAH(NYHA class III, mean PAP 77 mmHg, PVR 4 Wood U) underwent occlusion successfully after oral beraprost for two years. After surgery her mean PAP decrease to 38 mmHg with PVR of 2.52 Wood U. Hu et al [21] reported a ventricular septal defect (VSD)-ES patient with initial PVR of 18.84 Wood U underwent a successful operation after oral bosentan for 12 weeks, as a result of which her PVR decreased to 9.63 Wood U.…”

Section: Discussionmentioning

confidence: 99%

Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

Wang

Shen

et al. 2020

Preprint

View full text Add to dashboard Cite

Background: Patent ductus arteriosus ( PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidence of benefit from targeted therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether the PDA in patients with ES can be treated with transcatheter closure (TCC).Methods: Between August 2014 and July 2016, four out of fifteen PDA-ES patients whose Qp/Qs improved significantly and Qp/Qs>1.5 after acute vasodilator testing with 100% oxygen were selected to receive TCC and pulmonary vasodilator therapy. PAH-targeted drugs were prescribed before and after occlusion for all patients. Trial occlusion was performed before permanent closure.Results: The first TCC failed after the initiation of PAH-targeted drugs for 6 months in four patients. After the medication was adjusted and extended to 12 months, TCC was performed for all without hemodynamic intolerances during perioperative period. Pulmonary artery systolic pressure (PASP) was significantly decreased (≥ 40%) immediately after TCC. During a mean 52-month follow-up, there was a further decrease of PASP in two patients, the other two showed improved pulmonary vascular resistance (PVR), WHO functional class and six-minute walking distance although PASP was deteriorated again.Conclusion: Some selected PDA-ES patients with PVR<15Wood U and Qp/Qs>1.5 at baseline might benefit from TCC and PAH-targeted drugs pre- and post-occlusion play a crucial role.

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Effect of bosentan therapy on ventricular and atrial function in adults with Eisenmenger syndrome. A prospective, multicenter study using conventional and Speckle tracking echocardiography

Abstract: In adult patients with Eisenmenger syndrome, bosentan therapy improves ventricular and atrial functions resulting in enhancement of physical exercise and reduction in the NT-proBNP level, while the pulmonary vascular resistance does not change substantially.

Cited by 12 publications

References 29 publications

D-optimal Design for Preparation and Optimization of Fast Dissolving Bosentan Nanosuspension

D-optimal Design for Preparation and Optimization of Fast Dissolving Bosentan Nanosuspension

Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

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