Efficacy and Safety of a Novel Endothelin Receptor Antagonist, Macitentan, in Japanese Patients With Pulmonary Arterial Hypertension

Tahara, Nobuhiro; Dobashi, Hiroaki; Fukuda, Keiichi; Funauchi, Masanori; Hatano, Masaru; Ikeda, Satoshi; Joho, Shuji; Kihara, Yasuki; Kimura, Takeshi; Kondo, Takahisa; Matsushita, Masakazu; Minamino, Tohru; Nakanishi, Norifumi; Saji, Tsutomu; Sakai, Satoshi; Tanabe, Nobuhiro; Watanabe, Hiroshi; Yamada, Hisakata; Yoshioka, Koichiro; Sasayama, Shígetake

doi:10.1253/circj.cj-15-1305

Cited by 10 publications

(9 citation statements)

References 29 publications

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“…19,36 In this study, more than half of the patients had to stop macitentan due to oedema. 19 Growing experience with the use of macitentan will hopefully improve understanding of this associated side effect. Whereas paediatric data on side effects with macitentan are not available yet, the commonly used ERAs were associated with lower rates of side effects compared to adults.…”

Section: Discussionmentioning

confidence: 83%

“…The incidence of peripheral edema according to the SERAPHIN trial was 18,2% in the 10mg macitentan treatment group. Apart from that, macitentan has been associated with peripheral edema in other smaller studies with one including complex older patients 35,36 . In this study more than half of the patients had to stop macitentan due to edema 36 .…”

Section: Side Effects/ Safetymentioning

confidence: 94%

“…Apart from that, macitentan has been associated with peripheral edema in other smaller studies with one including complex older patients 35,36 . In this study more than half of the patients had to stop macitentan due to edema 36 . Growing experience with the use of macitentan will hopefully improve understanding of this associated side effect.…”

Section: Side Effects/ Safetymentioning

confidence: 94%

“…Classification of pulmonary hypertension (PH) was based on the consensus paper reported by the paeditatric taskforce of the Pulmonary vascular Research Institute (Panama Paper) and the updated classification proposed by the 6th World Symposium on Pulmonary Hypertension. 19 Neonates (<1 month of age) and adults as well as patients with postcapillary PH were excluded from the study.…”

Section: Methodsmentioning

confidence: 99%

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Macitentan in infants and children with pulmonary hypertensive vascular disease. Feasibility, tolerability and practical issues – a single‐centre experience

et al. 2021

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Macitentan is a safe and effective substance for treatment of adults with pulmonary arterial hypertension. Data on its use in paediatric patients is limited. In this single centre prospective study we report on our experience with macitentan in children focusing on applicability and practical aspects. Between 12/2014 and 07/2018 macitentan was introduced to paediatric patients according to a dosing protocol adjusted to body weight. Blood pressure, heart rate, saturation and clinical symptoms were recorded daily during introduction. Liver function parameters and haemoglobin levels were measured at baseline, 4 weeks and 3 months after initiation and after one year of treatment. 24 patients (14 male, 10 female) were enrolled for treatment with macitentan. The mean age was 10.7 +/- 7.6 years (range 0.1 y â 23 y). 15/24 pts were WHO FC II, 7 pts in FC III and 2 pts in FC IV. 20/24 patients (83%) received additional advanced therapy with sildenafil and /or prostacyclines. We had two early discontinuations because of clinical relevant edema. In the remaining 22 patients macitentan was well tolerated. Liver function parameters and blood count levels remained stable during the observational time. The introduction of macitentan was feasible and mostly well tolerated in paediatric patients. Special attention should be paid to edema during introduction of the drug. To the best of our knowledge, this is the first prospective observational study to report on its applicability in infants and children. However larger prospective trials are warranted to verify these preliminary findings.

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Section: Discussionmentioning

confidence: 83%

Section: Side Effects/ Safetymentioning

confidence: 94%

Section: Side Effects/ Safetymentioning

confidence: 94%

Section: Methodsmentioning

confidence: 99%

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Macitentan in infants and children with pulmonary hypertensive vascular disease. Feasibility, tolerability and practical issues – a single‐centre experience

et al. 2021

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“…Эффективность и безопасность терапии как бозентаном, так и мацитентаном у больных ЛАГ исследовалась в ряде пилотных работ и крупных рандомизированных клинических исследованиях [1,4,[11][12][13][14]. Однако в настоящее время имеется очевидный дефицит работ по прямому сравнению эффективности бозентана и мацитентана у больных ЛАГ, и, следовательно, имеется потребность в проведении подобного рода исследований.…”

Section: Discussionunclassified

Dynamics of the clinical functional and hemodynamic profile of patients with pulmonary arterial hypertension with initial monotherapy with endothelin receptor antagonists: bosentan vs. macitentan

Martynyuk

Aleevskaya

2020

Kardiologiia

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Aim To compare results of 24-h treatments with bosentan and macitentan by the clinical functional status and indexes of pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH).Materials and methods Based on the Russian National Registry (NCT03707561), 44 patients older than 18 years with PAH (34 patients with idiopathic pulmonary hypertension (IPH) and 10 patients with Eisenmenger syndrome) were retrospectively included into this study. Based on the statistical method of pairwise comparison, two groups were formed and matched by age, gender, WHO functional class (FC), and 6-min walk distance (6MWD). 22 patients of group 1 (17 with IPH and 5 with Eisenmenger syndrome) were treated with macitentan 10 mg/day, and 22 patients of group 2 (17 with IPH and 5 with Eisenmenger syndrome) were treated with bosentan 250 mg/day. Clinical instrumental data (6MWD, Borg dyspnea score, chest X-ray, transthoracic echocardiography (EchoCG), and right heart catheterization (RHC)) were evaluated at baseline and after 24 weeks of therapy.Results By week 24 of the treatment, FC and 6MWD improved in both groups. The macitentan treatment was associated with a significant decrease in Borg score. Significant intergroup differences in EchoCG data were not observed. The bosentan treatment was associated with a decrease in right ventricular (RV) dimension and a tendency towards a decrease in calculated pulmonary artery systolic pressure (PASP). By week 24, the macitentan treatment as compared to the bosentan treatment, was associated with a decrease in cardiothoracic ratio (CTR). In both groups, RHC showed decreases in PASP, mean pulmonary artery pressure and pulmonary vascular resistance, and improvements in cardiac output (CO), cardiac index, and stroke volume (SV) values. By week 24, the increase in SV was greater in the macitentan treatment group than in the bosentan treatment group (р=0.05).Conclusion The 24-week treatment with bosentan or macitentan provided significant and comparable improvement of the functional profile in PAH patients with FC II (WHO) at baseline. The decrease in CTR was significantly more pronounced in the macitental treatment group compared to the bosentan treatment group. The 24-week bosentan treatment resulted in a decrease in RV anterior-posterior dimension, a tendency towards a decrease in PASP according to EchoCG data. Macitentan provided more pronounced dynamics of dyspnea than bosentan according to the results of 6MWD test and the increase in SV according to RHC data.

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Left ventricular end-diastolic dimension and septal e′ are predictors of cardiac index at rest, while tricuspid annular plane systolic excursion is a predictor of peak oxygen uptake in patients with pulmonary hypertension

et al. 2017

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Little is known regarding a correlation of hemodynamics at rest or exercise capacity with echocardiographic parameters in patients with pulmonary hypertension (PH). To clarify these potential correlations, we performed transthoracic echocardiography, right heart catheterization, and cardiopulmonary exercise testing in 53 patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Left ventricular end-diastolic dimension (LVDd), early diastolic velocity of the septal mitral annulus (septal e′), tricuspid regurgitation peak gradient (TRPG), and tricuspid annular plane systolic excursion (TAPSE) were significantly correlated with cardiac index (LVDd; r = 0.477, P < 0.001, septal e′; r = 0.463, P = 0.001, TRPG; r = − 0.455, P = 0.001 and TAPSE; r = 0.406, P = 0.003, respectively). Multiple regression analysis revealed that LVDd and septal e′ were significantly associated with cardiac index (CI) and stroke volume index at rest. Among the exercise capacity markers evaluated, TAPSE, TRPG, and LVDd were significantly correlated with peak oxygen uptake (TAPSE; r = 0.534, P < 0.001, TRPG; r = − 0.466, P = 0.001 and LVDd; r = 0.411, P = 0.002, respectively). Multiple regression analysis showed that TAPSE was significantly associated with peak oxygen uptake (VO2). In PAH and CTEPH patients, LVDd and septal e′ were significantly associated with CI at rest, whereas TAPSE was significantly associated with peak VO2. Echocardiographic parameters may predict the prognostic factors of PAH and CTEPH patients.Electronic supplementary materialThe online version of this article (10.1007/s00380-017-1086-0) contains supplementary material, which is available to authorized users.

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Efficacy and Safety of a Novel Endothelin Receptor Antagonist, Macitentan, in Japanese Patients With Pulmonary Arterial Hypertension

Cited by 10 publications

References 29 publications

Macitentan in infants and children with pulmonary hypertensive vascular disease. Feasibility, tolerability and practical issues – a single‐centre experience

Macitentan in infants and children with pulmonary hypertensive vascular disease. Feasibility, tolerability and practical issues – a single‐centre experience

Dynamics of the clinical functional and hemodynamic profile of patients with pulmonary arterial hypertension with initial monotherapy with endothelin receptor antagonists: bosentan vs. macitentan

Left ventricular end-diastolic dimension and septal e′ are predictors of cardiac index at rest, while tricuspid annular plane systolic excursion is a predictor of peak oxygen uptake in patients with pulmonary hypertension

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