The Prophet Trial -Pilot Study to Assess Safety and Efficacy of a Novel Atrial Flow Regulator (AFR) in Patients With Pulmonary Hypertension

Inclusion Criteria:

1. Age is ≥ 18 / ≥6 years (phase 1 / phase 2).
2. Patient consents to participation
3. The patient or his/her legal representative should have the ability to fluently speakand under-stand the language in which the study is being conducted. If the patientspeaks a different language, then a sentence-to-sentence translation for unequivocalunderstanding must be provided.
4. Written, informed consent by the patient or her/his legally-authorized representativefor participation in the study.
5. Patient agrees to comply with the follow-up schedule.
6. Patient has had a successful BAS procedure and is in a stable hemodynamic state, asassessed by the investigator.
7. Conventional treatment options for the patient are exhausted according to ESC and AHAguidelines.
8. SpO2> 86 % pulsoxymetric measurement) This document is confidential and property ofOcclutech. It´s only for clinical personnel, Ethical committees and third person indirect contact with the clinical responsible. It is not allowed to distributeinformation contained in this document without the permission of Occlutech unless theinformation is already published.
9. "Syncope"(Group A-PH) 9.1. Syncope due to acute PH episodes (as defined by exclusionof other causes) 9.2. Other causes of syncope must have been actively excluded 9.3.Syncope (Black-out) or pre-syncope (episodic dizziness) >2 last 3 months 9.4. PH (defined as mean pulmonary artery pressure > 25 mmHg, or pulmonary vascular resistanceof > 3 Wood Units) must exist, RV-failure is however not a prerequisite
10. "RV-Failure"(Group B-PH) 10.1. Right heart failure, chronic and clinically severe 10.1.1.NYHA class III or worse 10.1.2. 6 min walk < 320 m 10.1.3. Signs of venouscongestion (distended veins, edema, ascites, etc) 10.1.4.Symptomatic disease resultingin 1 or more PH-related hospitalization over the last 12 months. Elective hospitaladmissions solely for the purpose of performing diagnostic procedures do not count forthis.

10.2. Severe pulmonary hypertension as evident by echocardiography Echocardiographic: 10.2.1. RV larger than LV; 10.2.2. RA larger than LA; 10.2.3. atrial septum bulging intoleft atrium 10.2.4.ventricular septum bulging into the left ventricle 10.2.5.Reduced (belowage-related normal mean value) TAPSE 10.3. Severe pulmonary hypertension as evident by CATHCATH-data: 10.3.1.Mean RA pressure (RAP) of > 10 and ≤ 20 mmHg; 10.3.2.Mean LA pressure (LAP) < 15 mmHg 10.3.3.Mean RAP > mean LAP; 10.3.4.Mean pulmonary arterial pressure >25 mmHg 10.3.5. Echocardiographically demonstrated continuous right to left shunt followingballoon aterial septostomy (BAS) and before AFR device implantation. Exclusion Criteria Processes which interfere medically with invasive device implantation

1. Local or generalized sepsis or other acute infection(s)
2. Thrombophilic coagulation disorder
3. Allergy to nickel and/or titanium and/or nickel/titanium-based materials
4. Allergy to anti-platelet, -coagulant, or -thrombotic therapy
5. Intolerance to contrast agents
6. Participation in other medical trials shorter than 30 days before the intended AFRimplantation procedure
7. Pregnancy - (assessed in patients with child bearing potential by urine dip stick)
8. Any intracardiac intervention within the last 30 days
9. Thickness of atrial septum > 12mm OR Processes which would technically disturb thesafe intervention as planned
10. Occluded inferior vena cava access 2. Previous ASD/PFO closure device in place 3.Intracardiac thrombus OR any other circumstance that, in the opinion of the Investigator, might interfere with the implantation, might affect the patient's well-being thereafter ormight interfere with the conduct and follow up within the Study is general.

Exclusion Criteria:

• Local or generalized sepsis or other acute infection(s)
• Thrombophilic coagulation disorder
• Allergy to nickel and/or titanium and/or nickel/titanium-based materials
• Allergy to anti-platelet, -coagulant, or -thrombotic therapy
• Intolerance to contrast agents
• Participation in other medical trials shorter than 30 days before the intended AFRimplantation procedure
• Pregnancy
• Any intracardiac intervention within the last 30 days
• Occluded inferior vena cava access
• Previous ASD/PFO closure device in place
• Intracardiac thrombus