PMID- 30963265
OWN - NLM
STAT- MEDLINE
DCOM- 20200305
LR  - 20200309
IS  - 1432-1750 (Electronic)
IS  - 0341-2040 (Linking)
VI  - 197
IP  - 3
DP  - 2019 Jun
TI  - Real-Life Experience with Selexipag as an Add-On Therapy to Oral Combination 
      Therapy in Patients with Pulmonary Arterial or Distal Chronic Thromboembolic 
      Pulmonary Hypertension: A Retrospective Analysis.
PG  - 353-360
LID - 10.1007/s00408-019-00222-7 [doi]
AB  - BACKGROUND: Patients with pulmonary arterial hypertension (PAH) and distal 
      chronic thromboembolic pulmonary hypertension (CTEPH) who still reveal risk 
      factors of worse prognosis on double combination therapy may benefit from add-on 
      therapy with the novel oral selective prostacyclin receptor agonist selexipag. 
      METHODS: We reviewed all patients with PAH/distal CTEPH in the Zurich cohort who 
      received selexipag as add-on to oral combination therapy and retrieved New York 
      Heart Association (NYHA) functional class, 6-min walk distance (6MWD), 
      NT-pro-BNP, quality of life questionnaires (CAMPHOR and EuroQoL), tricuspid 
      pressure gradient (TPG) by echocardiography and cardiopulmonary exercise test 
      parameters (power output and oxygen uptake). RESULTS: Twenty-three patients with 
      PAH/CTEPH (20/3), 14 females, median (quartiles) age 56 (46; 66) years received 
      an oral triple therapy containing selexipag at a median dose of 2000 (1600; 3100) 
      mcg during 221 (113; 359) days. The following parameters were stabilized from 
      baseline to last FU: 6MWD (440 (420; 490) to 464 (420; 526) m), NYHA class (three 
      to two), NT-pro-BNP (326 (167; 1725) to 568 (135; 1856)  ng/l), TPG, power 
      output, and oxygen uptake. Quality of life reflected by the CAMPHOR and EuroQoL 
      improved. CONCLUSIONS: Early initiation of triple oral combination therapy 
      including selexipag in PAH/CTEPH with intermediate risk factor profile may help 
      to stabilize functional class, exercise performance, and pulmonary hemodynamics 
      in a real-life setting and potentially improves quality of life. Whether these 
      beneficial effects can be truly attributed to the addition of selexipag should be 
      addressed in future randomized controlled trials.
FAU - Berlier, Charlotte
AU  - Berlier C
AD  - Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland.
FAU - Schwarz, Esther I
AU  - Schwarz EI
AD  - Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland.
FAU - Saxer, Stephanie
AU  - Saxer S
AD  - Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland.
FAU - Lichtblau, Mona
AU  - Lichtblau M
AD  - Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland.
FAU - Ulrich, Silvia
AU  - Ulrich S
AD  - Clinic of Pulmonology, University Hospital of Zurich, Zurich, Switzerland. 
      silvia.ulrich@usz.ch.
AD  - Center for Integrative Human Physiology, University of Zurich, Zurich, 
      Switzerland. silvia.ulrich@usz.ch.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20190408
PL  - United States
TA  - Lung
JT  - Lung
JID - 7701875
RN  - 0 (Acetamides)
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Endothelin Receptor Antagonists)
RN  - 0 (Enzyme Activators)
RN  - 0 (Peptide Fragments)
RN  - 0 (Phosphodiesterase 5 Inhibitors)
RN  - 0 (Pyrazines)
RN  - 0 (pro-brain natriuretic peptide (1-76))
RN  - 114471-18-0 (Natriuretic Peptide, Brain)
RN  - 5EXC0E384L (selexipag)
RN  - EC 4.6.1.2 (Soluble Guanylyl Cyclase)
SB  - IM
MH  - Acetamides/*therapeutic use
MH  - Aged
MH  - Antihypertensive Agents/*therapeutic use
MH  - Chronic Disease
MH  - Cohort Studies
MH  - Drug Therapy, Combination
MH  - Echocardiography
MH  - Endothelin Receptor Antagonists/*therapeutic use
MH  - Enzyme Activators/*therapeutic use
MH  - Exercise Test
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Natriuretic Peptide, Brain/blood
MH  - Oxygen Consumption
MH  - Peptide Fragments/blood
MH  - Phosphodiesterase 5 Inhibitors/*therapeutic use
MH  - Pulmonary Arterial Hypertension/blood/*drug therapy/etiology/physiopathology
MH  - Pulmonary Embolism/blood/complications/*drug therapy/physiopathology
MH  - Pyrazines/*therapeutic use
MH  - Quality of Life
MH  - Retrospective Studies
MH  - Soluble Guanylyl Cyclase
MH  - Tricuspid Valve
MH  - Walk Test
OTO - NOTNLM
OT  - Chronic thromboembolic pulmonary hypertension
OT  - Endothelin receptor antagonist
OT  - Phosphodiesterase inhibitor
OT  - Pulmonary arterial hypertension
OT  - Pulmonary hypertension
OT  - Selexipag
OT  - Vasodilator therapy
EDAT- 2019/04/10 06:00
MHDA- 2020/03/07 06:00
CRDT- 2019/04/10 06:00
PHST- 2018/09/25 00:00 [received]
PHST- 2019/03/25 00:00 [accepted]
PHST- 2019/04/10 06:00 [pubmed]
PHST- 2020/03/07 06:00 [medline]
PHST- 2019/04/10 06:00 [entrez]
AID - 10.1007/s00408-019-00222-7 [pii]
AID - 10.1007/s00408-019-00222-7 [doi]
PST - ppublish
SO  - Lung. 2019 Jun;197(3):353-360. doi: 10.1007/s00408-019-00222-7. Epub 2019 Apr 8.