Sota-ES - Sotatercept in Patients With Congenital Heart Disease and Eisenmenger´s Syndrome

Inclusion Criteria: * Age ≥18 years * Congenital heart disease with Eisenmenger syndrome (known unrepaired atrial septal defect, and/or ventricular septal defect, and/or patent ductus arteriosus; patients with anomalous pulmonary venous drainage will not be considered) * Eisenmenger syndrome defined as right-to-left or bi-directional shunt with a mPAP \>25 mmHg, PAWP \< 15 mmHg, and PVR \>5 WU * In patients with pre-tricuspid shunt, the consideration of Eisenmenger syndrome requires one of the following: Systemic arterial O2 saturation (SaO2) at rest \<88% and more than 70%, and/or SaO2 \<80% during 6MWT, and secondary erythrocytosis (Hb \> 15.0 g/dl for females and 16.0 g/dl for males) * On stable doses of background PAH therapy\* and diuretics (i.e., patient-individual dose goal for each therapy achieved) for ≥30 days * 6-minute walking distance \>100 m * WHO-FC II or III * Written informed consent Exclusion Criteria: * Age \<18 years * Diagnosis of pulmonary hypertension groups 2, 3, 4, or 5 * Hospitalization or change in PAH background therapies within 30 days prior to screening (changes in dose of diuretics or parenteral prostanoids \[\<10% change in infusion rate over the preceding 3 months\] are allowed) * Uncontrolled systemic hypertension as evidenced by sitting systolic blood pressure \>160 mmHg or sitting diastolic blood pressure \>100 mmHg during screening visit after a period of rest * Baseline systolic blood pressure \<90 mmHg at screening * Left v…