Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results

Aypar, Ebru; Alehan, Dursun; Karagöz, Tevfik; Aykan, H. Hakan; Ertuğrul, İlker

doi:10.1017/s1047951120000773

Cited by 9 publications

(3 citation statements)

References 22 publications

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“…A single center prospective study in a cohort of 13 patients aged 12 to 35 years over 24-week showed a beneficial effect of a switch from bosentan to macitentan with respect to exercise capacity (61). After 24 weeks, there was no further increment in exercise capacity in this cohort of patients with different etiologies of PAH (62). Eighteen patients younger than 12 years were prospectively assessed after either switching from sildenafil and/or bosentan to macitentan or who were treatment-naïve (20).…”

Section: Endothelin Receptor Antagonistsmentioning

confidence: 77%

Treatment of pulmonary arterial hypertension in children

Gorenflo

Ziesenitz

2021

Cardiovasc Diagn Ther

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Pulmonary arterial hypertension (PAH) is a devastating illness causing already significant morbidity in childhood. Currently approved treatment options for children comprise the endothelin receptor antagonist bosentan, as well as the phosphodiesterase-5 inhibitor sildenafil. But PAH treatment has advanced significantly over the past decade, and new classes of targeted drug therapies, such as stimulators of the soluble guanylate cyclase (riociguat) or prostacyclin receptor agonists (selexipag), are currently evaluated regarding their efficacy and safety in children, in order to limit off-label use. Due to the different etiologies in children, such as PAH-CHD, there is no evidence that initial combination therapy in children is superior to a mono-therapy with respect to survival. Special attention should also be paid to the pharmacology of PAH drugs in children, which might be impacted by ontogeny or drug-drug-interactions. Therapeutic drug monitoring may be useful in pediatric patients. There is a clear need for more controlled studies of PAH medications, alone or in combination therapy in the pediatric age group. Data from clinical trials as well as from patient registries should be pooled to optimize drug development and evaluation, trial design, and evidence-based pharmacotherapy in pediatric patients with PAH. In this review, the current treatment options of pediatric PAH are summarized, and an overview of new treatment concepts, which are already evaluated in adults, is presented.

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Section: Endothelin Receptor Antagonistsmentioning

confidence: 77%

Treatment of pulmonary arterial hypertension in children

Gorenflo

Ziesenitz

2021

Cardiovasc Diagn Ther

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“…Exercise capacity was assessed with 6MWD changes in 6 studies ( 15 , 19 – 23 ). Biased studies were excluded, and a fixed-effects model was applied, as shown in Figure 2 , which showed an increase in the 6MWD of 20.71 m (95% CI: 10.35–31.07, P < 0.00001, I 2 = 0%).…”

Section: Resultsmentioning

confidence: 99%

Efficacy and safety of switching from bosentan or ambrisentan to macitentan in pulmonary arterial hypertension: A systematic review and meta-analysis

Yang

Wang

et al. 2022

Front. Cardiovasc. Med.

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BackgroundThere is little evidence of the effectiveness of switching from the endothelin receptor antagonists (ERAs) bosentan and ambrisentan to a novel ERA, macitentan, in patients with pulmonary arterial hypertension (PAH). Therefore, a systematic review and meta-analysis was performed to evaluate the efficacy and safety of patients with PAH switching from other ERAs to macitentan.MethodsWe retrieved the relevant literature published before January 2022 for the meta-analysis from the PubMed, EMBASE, and Cochrane Library databases. Efficacy included changes in the 6-min walk distance (6MWD), World Health Organization functional class (WHO-FC), N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, hemodynamics, echocardiography and survival.ResultsNine studies, consisting of 408 PAH patients, that met the inclusion criteria were included. The switch from bosentan or ambrisentan to macitentan effectively increased the 6MWD by 20.71 m (95% CI: 10.35-31.07, P < 0.00001, I2 = 0%). Six months after conversion, the tricuspid annular plane systolic excursion was found to improve from 19.0 ± 4.0 to 21.0 ± 5.0 mm in adults and from 16.00 ± 5.0 to 18.25 ± 4.8 mm in children. Ordinal logistic regression showed that the WHO-FC significantly improved by 0.412 (95% CI: 0.187-0.908, P = 0.028). The switch did not show significant improvement in NT-proBNP levels. In addition, the switch was well tolerated.ConclusionThe switch from bosentan or ambrisentan to macitentan significantly increased the 6MWD in PAH patients, improved the WHO-FC, and exerted safety benefits. The effects of the switch on NT-proBNP levels, hemodynamics, and echocardiography still need to be further confirmed.Systematic review registration[https://www.crd.york.ac.uk/prospero/], identifier [CRD42021292554].

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“…There have been few studies on the transition between ERAs, and most of them were from bosentan or ambrisentan to macitentan. [8][9][10] However, the effect of transition from bosentan to ambrisentan needs to be investigated, and the present study aimed to address this question.…”

Section: Introductionmentioning

confidence: 99%

Transition from Bosentan to Ambrisentan in Pulmonary Arterial Hypertension: A Single-Center Prospective Study

Chen

Luo

Yang

et al. 2021

IJGM

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Background: Pulmonary hypertension patients experienced a high financial burden due to the high cost of drug therapy, high incidence of comorbidities and hospitalizations. Endothelin receptor antagonists (ERAs) in PAH treatment showed a high cost. While ambrisentan has been covered by medical insurance of a local government of China, there has been a drug transition from bosentan to ambrisentan in treating PAH patients. We evaluated the safety, efficacy and tolerability of ambrisentan after drug transition. Methods: Liver and renal functions were inspected at baseline, month 1, 3 and 6. NT-proBNP, echocardiographic variables, WHO functional class (WHO-FC), 6-minute walking distance (6MWD) were measured in the baseline and month 6 to evaluate the safety and efficacy. Quality of life (QOL) scale was used in the baseline and month 6 to investigate the tolerability and quality of life of PAH patients. Results: Among 224 PAH patients, 49 stable PAH patients meet the inclusion criteria were enrolled, among which three patients discontinued during the study. Our results showed no difference in 6-minute walking distance (6MWD) of PAH patients from baseline and month 6. The liver and renal function, N-terminal pro-brain natriuretic peptide (NT-proBNP), WHO functional class (WHO-FC) showed no difference either. For echocardiography parameters, the left ventricular end-diastolic dimension (LVEDD) of month 6 decreased. Other parameters were no significant difference from the baseline. There was no difference in the QOL scale between baseline and month 6. Conclusion:Our results suggested that it is safe and tolerable for stable PAH patients to transition from bosentan to ambrisentan without influencing hematologic parameters or heart function.

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Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results

Cited by 9 publications

References 22 publications

Treatment of pulmonary arterial hypertension in children

Treatment of pulmonary arterial hypertension in children

Efficacy and safety of switching from bosentan or ambrisentan to macitentan in pulmonary arterial hypertension: A systematic review and meta-analysis

Transition from Bosentan to Ambrisentan in Pulmonary Arterial Hypertension: A Single-Center Prospective Study

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