Prostacyclin receptor agonist Selexipag in patients with pulmonary arterial hypertension and congenital heart disease

INTERVENTION: Trade Name: Selexipag Product Name: Selexipag Pharmaceutical Form: Film‐coated tablet CONDITION: Pulmonary arterial hypertension related to congenital heart disease. Therapeutic area: Diseases [C] ‐ Cardiovascular Diseases [C14] PRIMARY OUTCOME: Main Objective: To determine whether the use of selexipag on top of current PAH medication improves exercise capacity in adult patients with pulmonary arterial hypertension related to congenital heart disease Primary end point(s): percentage change in exercise capacity Secondary Objective: 1. Time‐to‐first‐(PAH‐related) morbidity event or death; 2. Onset of (supra)ventricular arrhythmias; 3. Worsening of WHO functional class; 4. Increase right ventricular function parameters with echocardiography; 5. Decrease in serum biomarkers (NT‐pro‐BNP); 6. Increase in Quality‐of‐Life scores; 7. Number of adverse and serious adverse events; Timepoint(s) of evaluation of this end point: At 6 months of treatment with selexipag and at 12 months of study SECONDARY OUTCOME: Secondary end point(s): 1. Time‐to‐first‐(PAH‐related) morbidity event or death; 2. Onset of (supra)ventricular arrhythmias; 3. Worsening of WHO functional class; 4. Increase right ventricular function parameters with echocardiography; 5. Decrease in serum biomarkers (NT‐pro‐BNP); 6. Increase in Quality‐of‐Life scores; 7. Number of adverse events and serious adverse events; Timepoint(s) of evaluation of this end point: At 6 months of treatment with selexipag and at 12 months of study INCLUSION CRITERIA: ‐ Male and female patients = 18 years old ‐ Symptomatic PAH related to congenital heart disease of all complexities ‐ WHO functional class II‐III ‐ Documented hemodynamic diagnosis of PAH by right heart catheterization or echocardiography, performed at time prior to screening ‐ Patients not receiving treatment for PAH or those who are receiving endothelin‐receptor antagonist, a phosphodiesterase type 5 inhibitor or both at a dose that has been stable for 3 months ‐ Signed informed consent Are the trial subjects under 18? no Number of subjects for this age range: F.1.2 Adults (18‐64 years) yes F.1.2.1 Number of subjects for this age range 85 F.1.3 Elderly (>=65 years) yes F.1.3.1 Number of subjects for this age range 5