Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study.

Details

Ressource 1Download: Beghetti_et_al-2019-European_Journal_of_Heart_Failure.pdf (275.75 [Ko])
State: Public
Version: Final published version
License: CC BY-NC-ND 4.0
Serval ID
serval:BIB_E88556185F8F
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study.
Journal
European journal of heart failure
Author(s)
Beghetti M., Channick R.N., Chin K.M., Di Scala L., Gaine S., Ghofrani H.A., Hoeper M.M., Lang I.M., McLaughlin V.V., Preiss R., Rubin L.J., Simonneau G., Sitbon O., Tapson V.F., Galiè N.
ISSN
1879-0844 (Electronic)
ISSN-L
1388-9842
Publication state
Published
Issued date
03/2019
Peer-reviewed
Oui
Volume
21
Number
3
Pages
352-359
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Abstract
Patients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) after defect correction have a poor prognosis compared with other CHD-PAH patients. Therefore, it is important that these patients are treated as early and effectively as possible. Evidence supporting the use of PAH therapies in patients with corrected CHD-PAH from randomised controlled trials is limited. The purpose of these analyses was to characterise the corrected CHD-PAH patients from the GRIPHON study and examine the response to selexipag.
Out of the 110 patients diagnosed with corrected CHD-PAH, 55 had atrial septal defects, 38 had ventricular septal defects, 14 had persistent ducti arteriosus, and 3 had defects not further specified. Hazard ratios (HR) and 95% confidence intervals (CI) for the primary composite endpoint were calculated using Cox proportional hazard models. Compared with the non-CHD patients from GRIPHON, patients with corrected CHD-PAH were slightly younger, with a greater proportion being treatment-naive and in World Health Organization functional class I/II. The rate of the primary composite endpoint of morbidity/mortality was lower in patients with corrected CHD-PAH who were treated with selexipag compared with those treated with placebo (HR 0.58; 95% CI 0.25, 1.37). The most common adverse events were those known to be related to selexipag.
These post-hoc analyses of GRIPHON provide valuable information about a large population of patients with corrected CHD-PAH, and suggest that selexipag may delay disease progression and was well-tolerated in patients with corrected CHD-PAH.
Keywords
Congenital heart disease, Disease progression, Efficacy, Pulmonary arterial hypertension, Randomised controlled trial, Selexipag
Pubmed
Web of science
Open Access
Yes
Create date
14/01/2019 9:33
Last modification date
20/08/2019 16:11
Usage data