hypertension in the pulmonary arteries Pulmonary arterial hypertension due to congenital heart disease
Conditions
Interventions
Planned treatment: Subjects will receive 2dd individualized maximal tolerated
dose of selexipag for 6 consecutive months.
Congenital heart disease
Pulmonary arterial hypertension
Sponsors
Academisch Medisch Centrum
Eligibility
Age
18 Years to 64 Years
Inclusion criteria
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
Exclusion criteria
Exclusion criteria: - WHO functional class I of IV - Patients who have received prostacyclin (analogs) treatment within 1 month before baseline visit or are scheduled to receive any of these drugs during the trial - Patients with moderate or severe hepatic impairment (Child-Pugh B and C) - Females who are lactating or pregnant or plan to become pregnant during the study (a pregnancy test is offered to every female patient within the fertile age) - Known hypersensitivity to any of the excipients of the drug formulations - Contraindication for trial medication - Contraindication for cardiopulmonary exercise test - Incapable of giving informed consent
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Percentage change in exercise capacity (either V*O2peak in cardiopulmonary exercise test or 6-minute walk distance) between baseline and 6 months of treatment with selexipag | — |
Secondary
| Measure | Time frame |
|---|---|
| * Time-to-first- PAH-related morbidity event or death by any cause. Morbidity events defined as any of the following events: disease progression or worsening of pulmonary arterial hypertension that resulted in hospitalization, initiation of parenteral prostanoid therapy or long-term oxygen therapy, or the need for lung transplantation or balloon atrial septostomy. Disease progression was defined as a decrease in 6-minute walk distance from baseline (>=15%, confirmed by a 2nd test on a different day) combined with worsening of WHO FC for patients belonging to WHO FC II/III at baseline, or combined with the need for additional PAH-specific therapy for patients belonging to WHO FC III/IV at baseline. * Onset of (supra)ventricular arrhythmias; supraventricular arrhythmia defined as an episode of supraventricular bradyarrhythmia or tachyarrhythmia (reported on (24-hour ambulatory) ECG or requiring change of antiarrhythmic medication, electrical cardioversion, ablation or pacemaker implantation); ventricular arrhythmia, defined as nonsustained or sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator shock therapy or aborted ventricular fibrillation. * Difference of WHO functional class * Difference right ventricular function parameters with echocardiography * Difference in serum biomarkers * Difference in Quality-of-Life scores * Safety endpoint: number of (serious) adverse events | — |
Countries
The Netherlands
Outcome results
None listed