PMID- 34250282
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20231107
IS  - 2434-0790 (Electronic)
IS  - 2434-0790 (Linking)
VI  - 3
IP  - 7
DP  - 2021 Jul 9
TI  - Results of Macitentan in Japanese Patients With Chronic Thromboembolic Pulmonary 
      Hypertension　- A Prospective, Multicenter, Open-Label, Single-Arm, Phase 3 Study.
PG  - 405-413
LID - 10.1253/circrep.CR-21-0034 [doi]
AB  - Background: Macitentan, an endothelin-receptor antagonist, is approved in Japan 
      for the treatment of pulmonary arterial hypertension (PAH). This study evaluated 
      the use of macitentan for chronic thromboembolic pulmonary hypertension (CTEPH) 
      in Japanese patients. Methods and Results: This open-label single-arm Phase 3 
      study evaluated the efficacy and safety of oral macitentan 10 mg (once daily) in 
      Japanese CTEPH patients. The study was prematurely discontinued due to the 
      sponsor's decision to not develop macitentan 10 mg further for the indication of 
      CTEPH (unrelated to safety concerns). Of the 9 patients enrolled in the study, 4 
      completed 24 weeks of treatment. The mean (+/-SD) ratio of pulmonary vascular 
      resistance (PVR) at Week 16 to baseline was 71.9+/-34.3%. The mean (+/-SD) decreases 
      in PVR and the PVR index (PVRI) from baseline to Week 16 were 181.4+/-243.9 
      dyn.s/cm(5) and 280.6+/-366.0 dyn.s.m(2)/cm(5), respectively. The mean (+/-SD) 
      increase in the 6-min walk distance from baseline to Week 24 was 44.3+/-46.8 m. All 
      treatment-emergent adverse events (TEAEs) were mild or moderate in severity, 
      except for 1 serious TEAE of angioplasty reported in 1/9 patients that was severe 
      in intensity. Conclusions: Definite conclusions regarding the efficacy of 
      macitentan 10 mg in Japanese patients with CTEPH cannot be drawn because of 
      premature study discontinuation. No safety concerns were observed, and the safety 
      profile was consistent with previously reported studies in CTEPH and PAH 
      patients.
CI  - Copyright (c) 2021, THE JAPANESE CIRCULATION SOCIETY.
FAU - Sakurai, Satomi
AU  - Sakurai S
AD  - Neuroscience, Pulmonary Hypertension, Cardiovascular & Metabolism Clinical 
      Development Department, Clinical Science Division, Janssen Pharmaceutical K.K. 
      Tokyo Japan.
FAU - Ukyo, Yoshifumi
AU  - Ukyo Y
AD  - Statistics and Decision Sciences Japan, Janssen Pharmaceutical K.K. Tokyo Japan.
LA  - eng
PT  - Journal Article
DEP - 20210609
PL  - Japan
TA  - Circ Rep
JT  - Circulation reports
JID - 101746642
PMC - PMC8258181
OTO - NOTNLM
OT  - Chronic thromboembolic pulmonary hypertension
OT  - Endothelin receptor antagonists
OT  - Japan
OT  - Macitentan
OT  - Pulmonary arterial hypertension
COIS- S.S. and Y.U. are employees of Janssen Pharmaceutical K.K. (Tokyo, Japan).
EDAT- 2021/07/13 06:00
MHDA- 2021/07/13 06:01
PMCR- 2021/06/09
CRDT- 2021/07/12 06:00
PHST- 2021/04/01 00:00 [received]
PHST- 2021/04/27 00:00 [revised]
PHST- 2021/05/10 00:00 [accepted]
PHST- 2021/07/12 06:00 [entrez]
PHST- 2021/07/13 06:00 [pubmed]
PHST- 2021/07/13 06:01 [medline]
PHST- 2021/06/09 00:00 [pmc-release]
AID - 10.1253/circrep.CR-21-0034 [doi]
PST - epublish
SO  - Circ Rep. 2021 Jun 9;3(7):405-413. doi: 10.1253/circrep.CR-21-0034. eCollection 
      2021 Jul 9.