Selexipag as Add-on Therapy for Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: A Single-Center Retrospective Study

Purpose: This study examined the efficacy and safety of selexipag in treating pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Materials and Methods: We conducted a retrospective study of patients with CHD-associated PAH, treated with selexipag since December 2017. Thirteen adult patients (mean age, 45.4 years; women, 77%) were treated with selexipag as add-on therapy. Baseline characteristics, World Health Organization functional class, 6-minute walking distance (6MWD) test results, N-terminal pro-B-type natriuretic peptide levels, echocardiographic data, and incidence of side effects were assessed. Results: The majority of patients (12/13, 92.3%) experienced more than one treatment-associated complication; one patient dropped out of the study due to intolerable myalgia. The results of 6MWD test (from 299.2 ± 56.2 m to 363.8 ± 86.5 m, p = 0.039) and tricuspid regurgitation (TR) pressure gradient (from 84.7 ± 20.5 mmHg to 61.6 ± 24.0 mmHg, p = 0.018) improved and remained improved after selexipag treatment in 12 patients. Based on the results of a non-invasive risk assessment, 8 (66.7%) patients showed improvement, 3 (25.0%) showed no interval change, and the status of one patient (8.3%) deteriorated. Moreover, compared to patients treated with a low dosage, patients treated with a medium-to-high dosage showed a greater increase in 6MWD results (88.3 ± 26.4 m vs. 55.3 ± 27.6 m, p = 0.043) and a greater reduction in the TR pressure gradient (−33.7 ± 10.9 mmHg vs. −12.5 ± 12.0 mmHg, p = 0.015). Conclusion: Selexipag is an efficient pulmonary vasodilator as add-on therapy in treating CHD-associated PAH.