Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction

insights from the randomised controlled GRIPHON study

Maurice Beghetti, Richard N. Channick, Kelly M Chin, Lilla Di Scala, Sean Gaine, Hossein Ardeschir Ghofrani, Marius M. Hoeper, Irene M. Lang, Vallerie V. McLaughlin, Ralph Preiss, Lewis J. Rubin, Gérald Simonneau, Olivier Sitbon, Victor F. Tapson, Nazzareno Galiè

Research output: Contribution to journalArticle

1 Citation (Scopus)

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 languageEnglish (US)
JournalEuropean Journal of Heart Failure
DOIs
StateAccepted/In press - Jan 1 2019

Fingerprint

Pulmonary Hypertension
Heart Diseases
Therapeutics
selexipag
Confidence Intervals
Ductus Arteriosus
Atrial Heart Septal Defects
Ventricular Heart Septal Defects
Proportional Hazards Models
Disease Progression
Randomized Controlled Trials
Placebos
Morbidity
Mortality

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 journalArticle

Beghetti, M, Channick, RN, Chin, KM, Di Scala, L, Gaine, S, Ghofrani, HA, Hoeper, MM, Lang, IM, McLaughlin, VV, Preiss, R, Rubin, LJ, Simonneau, G, Sitbon, O, Tapson, VF & Galiè, N 2019, 'Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study', European Journal of Heart Failure. https://doi.org/10.1002/ejhf.1375
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. / Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction : insights from the randomised controlled GRIPHON study. In: European Journal of Heart Failure. 2019.
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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.",
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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

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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

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