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

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.