Journal article

Publication year: 2019

Pages: 352-359

Journal: European Journal of Heart Failure

Volume number: 21

Issue number: 3

ISSN: 1388-9842

eISSN: 1879-0844

Open access status: Hybrid

DOI Link: https://doi.org/10.1002/ejhf.1375

Publisher: Wiley

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.

Harvard Citation style: Beghetti, M., Channick, R., Chin, K., Di Scala, L., Gaine, S., Ghofrani, H., et al. (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, 21(3), pp. 352-359. https://doi.org/10.1002/ejhf.1375

APA Citation style: Beghetti, M., Channick, R., Chin, K., Di Scala, L., Gaine, S., Ghofrani, H., Hoeper, M., Lang, I., McLaughlin, V., Preiss, R., Rubin, L., Simonneau, G., Sitbon, O., Tapson, V., & Galie, 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. 21(3), 352-359. https://doi.org/10.1002/ejhf.1375