PMID- 32421542
OWN - NLM
STAT- MEDLINE
DCOM- 20201020
LR  - 20201020
IS  - 1532-3064 (Electronic)
IS  - 0954-6111 (Linking)
VI  - 167
DP  - 2020 Jun
TI  - Long-term real world clinical outcomes of macitentan therapy in chronic 
      thromboembolic pulmonary hypertension.
PG  - 105966
LID - S0954-6111(20)30106-2 [pii]
LID - 10.1016/j.rmed.2020.105966 [doi]
AB  - BACKGROUND: Macitentan treatment for chronic thromboembolic pulmonary 
      hypertension (CTEPH) in the routine clinical setting is increasing. However, 
      'real world' macitentan experience is scarce and is needed to differentiate from 
      controlled clinical trial settings. OBJECTIVE: We describe our outcomes and 
      clinical 'real world' experience of macitentan mono- and combination therapy with 
      riociguat or sildenafil in CTEPH. METHODS: We included all consecutive CTEPH 
      patients, either non-operated or with residual PH after pulmonary endarterectomy 
      (PEA), treated with macitentan in the St. Antonius hospital in Nieuwegein, the 
      Netherlands, between 01-2014 and 11-2019. We describe clinical outcomes and 
      adverse events (AEs) until 2 years after macitentan initiation. RESULTS: In total 
      73 CTEPH patients on macitentan were included, of which 18 patients were 
      clinically inoperable (n = 7 declined PEA, n = 11 nonacceptable risk-benefit) and 
      55 had technically inoperable CTEPH (n = 48)/residual PH (n = 7). Clinically 
      inoperable patients (mean age 72.4 +/- 10.2 years, 61% female, 28% macitentan 
      monotherapy, observation period 2.0 (1.9-2.0) years) had a survival of 100% and 
      clinical worsening (CW)-free survival of 88% at 2-year follow-up respectively, 
      with a significant increased 6-min walking distance (6MWD). Technically 
      inoperable/residual PH patients (mean age 62.1 +/- 14.1 years, 60% female, 27% 
      macitentan monotherapy, observation period 2.0 (1.0-2.0) years) had a 2-year 
      survival and CW-free survival of 86% and 68% respectively, with significant 
      improved 6MWD and NT-proBNP. Nonsevere AEs were reported in 30% of all patients. 
      CONCLUSION: Macitentan mono- and combination therapy in non-operated CTEPH and 
      residual PH is safe and improves clinical outcomes till 2-year follow-up.
CI  - Copyright (c) 2020 Elsevier Ltd. All rights reserved.
FAU - van Thor, M C J
AU  - van Thor MCJ
AD  - Dept of Cardiology, St. Antonius Hospital, Nieuwegein, the Netherlands; Dept of 
      Pulmonary Medicine, St. Antonius Hospital, Nieuwegein, the Netherlands.
FAU - Ten Klooster, L
AU  - Ten Klooster L
AD  - Dept of Pulmonary Medicine, St. Antonius Hospital, Nieuwegein, the Netherlands.
FAU - Snijder, R J
AU  - Snijder RJ
AD  - Dept of Pulmonary Medicine, St. Antonius Hospital, Nieuwegein, the Netherlands.
FAU - Mager, J J
AU  - Mager JJ
AD  - Dept of Pulmonary Medicine, St. Antonius Hospital, Nieuwegein, the Netherlands.
FAU - Post, M C
AU  - Post MC
AD  - Dept of Cardiology, St. Antonius Hospital, Nieuwegein, the Netherlands. 
      Electronic address: m.post@antoniusziekenhuis.nl.
LA  - eng
PT  - Journal Article
DEP - 20200409
PL  - England
TA  - Respir Med
JT  - Respiratory medicine
JID - 8908438
RN  - 0 (Pyrimidines)
RN  - 0 (Sulfonamides)
RN  - Z9K9Y9WMVL (macitentan)
SB  - IM
MH  - Aged
MH  - Aged, 80 and over
MH  - Chronic Disease
MH  - Disease-Free Survival
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Hypertension, Pulmonary/*drug therapy/mortality/physiopathology
MH  - Male
MH  - Middle Aged
MH  - Netherlands
MH  - Pulmonary Embolism/*drug therapy/mortality/physiopathology
MH  - Pyrimidines/*therapeutic use
MH  - Sulfonamides/*therapeutic use
MH  - Time Factors
MH  - Treatment Outcome
MH  - Walk Test
MH  - Walking
OTO - NOTNLM
OT  - Chronic thromboembolic pulmonary hypertension
OT  - Clinical worsening
OT  - Combination therapy
OT  - Macitentan
OT  - Survival
COIS- Declaration of competing interest M. van Thor, J. Mager and M. Post report grants 
      from Actelion Pharmaceuticals. L ten Klooster has nothing to disclose. R. Snijder 
      reports grants from Pfizer and Actelion Pharmaceuticals.
EDAT- 2020/05/19 06:00
MHDA- 2020/10/21 06:00
CRDT- 2020/05/19 06:00
PHST- 2020/02/01 00:00 [received]
PHST- 2020/03/24 00:00 [revised]
PHST- 2020/04/05 00:00 [accepted]
PHST- 2020/05/19 06:00 [entrez]
PHST- 2020/05/19 06:00 [pubmed]
PHST- 2020/10/21 06:00 [medline]
AID - S0954-6111(20)30106-2 [pii]
AID - 10.1016/j.rmed.2020.105966 [doi]
PST - ppublish
SO  - Respir Med. 2020 Jun;167:105966. doi: 10.1016/j.rmed.2020.105966. Epub 2020 Apr 
      9.