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.
Acetamides
/ therapeutic use
Aged
Antihypertensive Agents
/ therapeutic use
Chronic Disease
Cohort Studies
Drug Therapy, Combination
Echocardiography
Endothelin Receptor Antagonists
/ therapeutic use
Enzyme Activators
/ therapeutic use
Exercise Test
Female
Humans
Male
Middle Aged
Natriuretic Peptide, Brain
/ blood
Oxygen Consumption
Peptide Fragments
/ blood
Phosphodiesterase 5 Inhibitors
/ therapeutic use
Pulmonary Arterial Hypertension
/ blood
Pulmonary Embolism
/ blood
Pyrazines
/ therapeutic use
Quality of Life
Retrospective Studies
Soluble Guanylyl Cyclase
Tricuspid Valve
Walk Test
Chronic thromboembolic pulmonary hypertension
Endothelin receptor antagonist
Phosphodiesterase inhibitor
Pulmonary arterial hypertension
Pulmonary hypertension
Selexipag
Vasodilator therapy
Journal
Lung
ISSN: 1432-1750
Titre abrégé: Lung
Pays: United States
ID NLM: 7701875
Informations de publication
Date de publication:
06 2019
06 2019
Historique:
received:
25
09
2018
accepted:
25
03
2019
pubmed:
10
4
2019
medline:
7
3
2020
entrez:
10
4
2019
Statut:
ppublish
Résumé
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. 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). 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. 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.
Sections du résumé
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.
Identifiants
pubmed: 30963265
doi: 10.1007/s00408-019-00222-7
pii: 10.1007/s00408-019-00222-7
doi:
Substances chimiques
Acetamides
0
Antihypertensive Agents
0
Endothelin Receptor Antagonists
0
Enzyme Activators
0
Peptide Fragments
0
Phosphodiesterase 5 Inhibitors
0
Pyrazines
0
pro-brain natriuretic peptide (1-76)
0
Natriuretic Peptide, Brain
114471-18-0
selexipag
5EXC0E384L
Soluble Guanylyl Cyclase
EC 4.6.1.2
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
353-360Références
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