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Clinical Trial
. 2019 Mar;21(3):352-359.
doi: 10.1002/ejhf.1375. Epub 2019 Jan 11.

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

Maurice Beghetti  1 , Richard N Channick  2 , Kelly M Chin  3 , Lilla Di Scala  4 , Sean Gaine  5 , Hossein-Ardeschir Ghofrani  6 , Marius M Hoeper  7 , Irene M Lang  8 , Vallerie V McLaughlin  9 , Ralph Preiss  4 , Lewis J Rubin  10 , Gérald Simonneau  11 , Olivier Sitbon  11 , Victor F Tapson  12 , Nazzareno Galiè  13
Affiliations
Clinical Trial

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

Maurice Beghetti et al. Eur J Heart Fail. 2019 Mar.

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.

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Figures

Figure 1
Figure 1
Effect of selexipag on the primary composite endpoint of morbidity/mortality in patients with pulmonary arterial hypertension associated with congenital heart disease after defect correction.
See this image and copyright information in PMC

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