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.
| Original language | English (US) |
|---|---|
| Journal | European Journal of Heart Failure |
| DOIs | |
| State | Accepted/In press - Jan 1 2019 |
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Keywords
- Congenital heart disease
- Disease progression
- Efficacy
- Pulmonary arterial hypertension
- Randomised controlled trial
- Selexipag
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
Cite this
Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction : insights from the randomised controlled GRIPHON study. / Beghetti, Maurice; Channick, Richard N.; Chin, Kelly M; Di Scala, Lilla; Gaine, Sean; Ghofrani, Hossein Ardeschir; Hoeper, Marius M.; Lang, Irene M.; McLaughlin, Vallerie V.; Preiss, Ralph; Rubin, Lewis J.; Simonneau, Gérald; Sitbon, Olivier; Tapson, Victor F.; Galiè, Nazzareno.
In: European Journal of Heart Failure, 01.01.2019.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction
T2 - insights from the randomised controlled GRIPHON study
AU - Beghetti, Maurice
AU - Channick, Richard N.
AU - Chin, Kelly M
AU - Di Scala, Lilla
AU - Gaine, Sean
AU - Ghofrani, Hossein Ardeschir
AU - Hoeper, Marius M.
AU - Lang, Irene M.
AU - McLaughlin, Vallerie V.
AU - Preiss, Ralph
AU - Rubin, Lewis J.
AU - Simonneau, Gérald
AU - Sitbon, Olivier
AU - Tapson, Victor F.
AU - Galiè, Nazzareno
PY - 2019/1/1
Y1 - 2019/1/1
N2 - 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.
AB - 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.
KW - Congenital heart disease
KW - Disease progression
KW - Efficacy
KW - Pulmonary arterial hypertension
KW - Randomised controlled trial
KW - Selexipag
UR - http://www.scopus.com/inward/record.url?scp=85059889300&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85059889300&partnerID=8YFLogxK
U2 - 10.1002/ejhf.1375
DO - 10.1002/ejhf.1375
M3 - Article
C2 - 30632656
AN - SCOPUS:85059889300
JO - European Journal of Heart Failure
JF - European Journal of Heart Failure
SN - 1388-9842
ER -