@article{ca409ee0e984446ba761472dd4b6d95d,
title = "Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study",
abstract = "Aims: 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. Methods and results: 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. Conclusions: 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",
author = "Maurice Beghetti and Channick, {Richard N.} and Chin, {Kelly M.} and {Di Scala}, Lilla and Sean Gaine and Ghofrani, {Hossein Ardeschir} and Hoeper, {Marius M.} and Lang, {Irene M.} and McLaughlin, {Vallerie V.} and Ralph Preiss and Rubin, {Lewis J.} and G{\'e}rald Simonneau and Olivier Sitbon and Tapson, {Victor F.} and Nazzareno Gali{\`e}",
note = "Funding Information: The authors thank Dr. Ruth Lloyd from nspm ltd for medical writing assistance, funded by Actelion Pharmaceuticals Ltd. Funding Information: This work was supported by Actelion Pharmaceuticals Ltd, Allschwil, Switzerland. Conflict of interest: M.B. reports non-financial support, grants and personal fees from Actelion Pharmaceuticals Ltd; grants and personal fees from Bayer HealthCare; and consultancy fees from Eli Lilly, Pfizer and GSK. R.N.C. consults for Actelion Pharmaceuticals Ltd and Bayer HealthCare and has received a research grant from Bayer HealthCare. K.M.C. reports personal fees, grants and non-financial support from Actelion Pharmaceuticals Ltd; grants and personal fees from Bayer HealthCare and Gilead Sciences Inc; and grants from GeNO and the NIH. L.D.S. is an employee of Actelion Pharmaceuticals Ltd and holds stock in the parent company Johnson&Johnson. S.G. reports personal fees and non-financial support from Actelion Pharmaceuticals Ltd, Bayer HealthCare, GSK, Novartis and Daiichi-Sankyo, and personal fees from United Therapeutics and Pfizer. H.A.G. reports personal fees from Actelion Pharmaceuticals Ltd, Bayer HealthCare, Ergonex, GSK, Novartis, Pfizer, AbbVie, Bellerophon Pulse Technologies, Gilead, Medscape, MSD, OMT, Web MD Global, Deutschlandfunk and the PVRI. M.M.H. reports personal fees and non-financial support from Actelion Pharmaceuticals Ltd, and personal fees from Pfizer, Bayer HealthCare, GSK, MSD and Gilead. I.M.L. reports grants, personal fees and non-financial support from Actelion Pharmaceuticals Ltd; grants and personal fees from United Therapeutics, Bayer HealthCare and GSK; and personal fees from Novartis. V.V.McL. reports grants, personal fees and non-financial support from Actelion Pharmaceuticals Ltd, Bayer HealthCare, Funding Information: Gilead, and Ikaria; grants from Novartis; and personal fees from SteadyMed Therapeutics and St. Jude Medical. R.P. is an employee of Actelion Pharmaceuticals Ltd and holds stock in the parent company Johnson&Johnson. L.J.R. has consulted for Acte-lion Pharmaceuticals Ltd, United Therapeutics, Lung LLC, GeNO, and Gilead. G.S. consults for and has received grants from Acte-lion Pharmaceuticals Ltd, Bayer HealthCare, GSK, and Pfizer. O.S. has consulted for Actelion Pharmaceuticals Ltd, GSK, Pfizer, Eli Lilly, United Therapeutics and Bayer HealthCare, and has received grants from Actelion Pharmaceuticals Ltd, GSK, Pfizer, Eli Lilly and Bayer HealthCare. V.F.T. reports grants, personal fees and non-financial support from Actelion Pharmaceuticals Ltd; grants and personal fees from Bayer HealthCare, United Therapeutics, Janssen, EKOS/BTG, Arena and Reata; and personal fees from Gilead Sciences and Daiichi-Sankyo. N.G. reports grants, personal fees and non-financial support from Actelion Pharmaceuticals Ltd, and grants and personal fees from Bayer HealthCare, GSK, and Pfizer.",
year = "2019",
month = mar,
doi = "10.1002/ejhf.1375",
language = "English (US)",
volume = "21",
pages = "352--359",
journal = "European Journal of Heart Failure",
issn = "1388-9842",
publisher = "Oxford University Press",
number = "3",
}