[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"eisenmenger-syndrome\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:eisenmenger-syndrome":28},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,3,0,[8,46,77],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":15,"eligibilityCriteria":16,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":23,"briefSummary":25,"conditions":26,"keywords":30,"overallStatus":34,"whyStopped":4,"lastUpdateSubmitDate":35,"lastUpdatePostDateStruct":36,"startDateStruct":39,"completionDateStruct":41,"leadSponsor":43,"locationsCount":4},"100631294","phase-2-sota-es---sotatercept-in-patients-with-congenital-heart-disease-and-eisenmengers-syndrome-100631294",false,"NCT07498803","Sota-ES - Sotatercept in Patients With Congenital Heart Disease and Eisenmenger´s Syndrome","Sota-ES - A Prospective, Non-randomized, Open-label, Multi-center Study of the Activin Signaling Inhibitor Sotatercept in Patients With Congenital Heart Disease and Eisenmenger´s Syndrome","CHASE","Inclusion Criteria:\n\n* Age ≥18 years\n* Congenital heart disease with Eisenmenger syndrome (known unrepaired atrial septal defect, and\u002For ventricular septal defect, and\u002For patent ductus arteriosus; patients with anomalous pulmonary venous drainage will not be considered)\n* Eisenmenger syndrome defined as right-to-left or bi-directional shunt with a mPAP \\>25 mmHg, PAWP \\\u003C 15 mmHg, and PVR \\>5 WU\n* In patients with pre-tricuspid shunt, the consideration of Eisenmenger syndrome requires one of the following: Systemic arterial O2 saturation (SaO2) at rest \\\u003C88% and more than 70%, and\u002For SaO2 \\\u003C80% during 6MWT, and secondary erythrocytosis (Hb \\> 15.0 g\u002Fdl for females and 16.0 g\u002Fdl for males)\n* On stable doses of background PAH therapy\\* and diuretics (i.e., patient-individual dose goal for each therapy achieved) for ≥30 days\n* 6-minute walking distance \\>100 m\n* WHO-FC II or III\n* Written informed consent\n\nExclusion Criteria:\n\n* Age \\\u003C18 years\n\n  * Diagnosis of pulmonary hypertension groups 2, 3, 4, or 5\n  * Hospitalization or change in PAH background therapies within 30 days prior to screening (changes in dose of diuretics or parenteral prostanoids \\[\\\u003C10% change in infusion rate over the preceding 3 months\\] are allowed)\n  * 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\n  * Baseline systolic blood pressure \\\u003C90 mmHg at screening\n  * Left ventricular systolic dysfunction (LVEF 40%)\n  * Restrictive lung disease with a TLC \\\u003C 60% AND demonstration of more than mild fibrosis on chest CT prior to enrolment (note that patients with congenital heart disease may have thoracic cage deformities \\[e.g. pectus\\] that may lead to thoracic cage restriction in the absence of parenchymal lung disease).\n  * Obstructive lung disease (FEV1 \\\u003C 60% pred. and FEV1\u002FFVC \\\u003C60%)\n  * Chronic thromboembolic disease (intermediate or high probability) on V\u002FQ scan and evidence of distal thromboembolism on CT angiography (note that proximal in-situ thrombosis is a known complication of Eisenmenger's and will not be considered an exclusion criterion - such patients may be reviewed centrally for inclusion)\n  * Significant liver disease (Child II or III)\n  * Any of the following clinical laboratory values at the screening visit:\n\n    * Estimated glomerular filtration rate (eGFR) \\\u003C30 mL\u002Fmin\u002Fm2 (as defined by the Modification of Diet in Renal Disease \\[MDRD\\] equation)\n    * Serum alanine aminotransferase, aspartate aminotransferase, or total bilirubin levels \\>3 × ULN (bilirubin criterion waived if there is a documented history of Gilbert's syndrome)\n  * Baseline platelet count \\\u003C50,000\u002Fµl (\\\u003C50.0 x 109\u002FL) at screening\n  * Documented episodes of previous repetitive hyperviscosity syndrome\n  * History of haemoptysis within 12 months prior to screening, and\u002For repeated severe epistaxis (≥ 1 episode per month)\n  * Uncorrected iron deficiency (iron stores must be normal defined as a Ferritin \\> 100 and transferrin saturation \\[TSAT\\] ≥ 20%)\n  * Recent (\\\u003C 3 months) enrolment into a rehabilitation program\n  * Untreated sleep disordered breathing with an AHI \\> 10. Patients with sleep apnea will need to have been on CPAP or BiPAP for 3 months prior to enrolment.\n  * Prior or current exposure to sotatercept or luspatercept\n  * Treatment by phlebotomy within 1 month prior to screening\n  * Participation in another interventional study\n  * Pregnant (serum B-HCG) or lactating women","ALL","18 Years",{"count":20,"type":21},40,"ESTIMATED","INTERVENTIONAL",[24],"PHASE2","The present study seeks to provide pilot data on the safety and efficacy of medical therapy with sotatercept in patients with an established diagnosis of congenital heart disease and Eisenmenger syndrome.\n\nCHASE is an interventional, single-arm, open-label study, that will enroll 40 patients with an established diagnosis of CHD and Eisenmenger syndrome. PAH background therapy may be present at the discretion of the investigators at the time of enrolment. CHASE will be performed only in countries where standard PAH therapies are available and reimbursed. At the end of the 24-week patient period, PAH treatment is left to the investigator's discretion.",[27,28,29],"Congenital Heart Disease (CHD)","Eisenmenger Syndrome","PAH",[31,32,33],"Congenital heart disease with Eisenmenger syndrome","WHO-FC II or III","PAH therapy","NOT_YET_RECRUITING","2026-03-23",{"date":37,"type":38},"2026-03-27","ACTUAL",{"date":40,"type":21},"2026-09-01",{"date":42,"type":21},"2028-12-31",{"name":44,"class":45},"Philipps University Marburg","OTHER",{"id":47,"slug":48,"hasResults":11,"nctId":49,"briefTitle":50,"officialTitle":51,"acronym":52,"eligibilityCriteria":53,"healthyVolunteers":11,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":54,"targetDuration":4,"studyType":22,"phases":56,"briefSummary":58,"conditions":59,"keywords":61,"overallStatus":66,"whyStopped":4,"lastUpdateSubmitDate":67,"lastUpdatePostDateStruct":68,"startDateStruct":70,"completionDateStruct":72,"leadSponsor":74,"locationsCount":76},"100620373","phase-4-a-study-of-sotatercept-for-patients-with-eisenmenger-syndrome-or-unrepaired-shunt-associated-pulmonary-arterial-hypertension-resistant-to-vasodilator-therapy-100620373","NCT07356778","A Study of Sotatercept for Patients With Eisenmenger Syndrome or Unrepaired Shunt-Associated Pulmonary Arterial Hypertension Resistant to Vasodilator Therapy","An Open-label, Randomized, Controlled Trial to Evaluate the Efficacy of Sotatercept Add-on Therapy Compared to Standard PAH Therapy With Pulmonary Vasodilators for Pulmonary Arterial Hypertension Associated With Pulmonary Vasodilator-resistant, Unrepaired Congenital Shunts (ASD, VSD, PDA) Including Eisenmenger Syndrome：SuMILE Trial","SuMILE","Inclusion Criteria:\n\n* adults (≥18 years)\n* unrepaired ASD, VSD or PDA\n* ≥90 days of pulmonary vasodilator therapy; and either (i) pulmonary vascular resistance (PVR) ≥5 Wood units and mean pulmonary arterial pressure (mPAP) \\> 20 mm Hg on right heart catheterization within 180 days, or (ii) echocardiographic tricuspid regurgitation velocity \\>3.4 m\u002Fs with right-to-left\u002Fbidirectional shunt plus resting SpO₂ ≤92% consistent with cyanosis\n* baseline 6MWD ≥100 m\n* ability to complete questionnaires\n\nExclusion Criteria:\n\n* WHO functional class IV; other unrepaired intracardiac shunts\n* severe renal\u002Fhepatic\u002Fparenchymal lung disease or LVEF \\\u003C40%\n* prior sotatercept use\n* contraindication to sotatercept per label\n* investigator-judged unsuitability",{"count":55,"type":21},36,[57],"PHASE4","What is this study about? This study will test whether adding sotatercept to usual medicines for pulmonary arterial hypertension (PAH) can help adults who have PAH due to unrepaired congenital heart defects (atrial or ventricular septal defect, or patent ductus arteriosus), including Eisenmenger syndrome. These conditions often cause long-standing changes in the lung blood vessels and low oxygen levels.\n\nWho can join? About 36 adults (age ≥18 years) in Japan whose PAH has not improved enough with pulmonary vasodilators may join. People with very severe symptoms (WHO class IV) or other serious illnesses will not be enrolled.\n\nWhat will happen if I join?\n\nParticipants will be randomly assigned (like a coin flip, in a 2:1 ratio) to:\n\nSotatercept + vasodilator-based PAH care, or\n\nvasodilator-based PAH care alone. The study lasts 24 weeks. Those who receive sotatercept will have injections every 3 weeks. All participants will have clinic visits and tests at the start, week 12, and week 24, including a 6-minute walk test (how far you can walk in 6 minutes), blood tests, questionnaires, and other heart-lung assessments used in routine PAH care.\n\nWhat are the possible benefits? Sotatercept improved exercise capacity and heart-lung measures in other PAH studies, but people with unrepaired heart defects were not included. This study may or may not help you directly, but it may help doctors learn how to use sotatercept safely in this group.\n\nWhat are the possible risks? Side effects seen with sotatercept include increase in haemoglobin, low platelets, nosebleeds, telangiectasia (small dilated blood vessels), bleeding, and blood clots. People with Eisenmenger syndrome can have both bleeding (for example, haemoptysis) and clotting risks. The study will check complete blood counts (CBC) regularly and adjust or pause dosing using label-based rules. Other risks are those of standard PAH care and blood tests.\n\nTime and location The study is conducted at multiple hospitals in Japan. Study participation lasts about 6 months.\n\nCosts and payments The study drug and study-specific tests will be provided at no cost. Usual medical care not required by the study will follow each hospital's standard billing. There is no required payment to join. Any travel reimbursement or stipends will follow site policy.\n\nPrivacy Your information will be kept confidential. Results will be shared in journals and at meetings without using your name.\n\nWho to contact If you are interested or have questions, please contact the study team at the participating hospital.",[28,60],"Pulmonary Arterial Hypertension of Congenital Heart Disease",[62,63,64,65],"Eisenmenger syndrome","pulmonary arterial hypertension","Congential heart disease","sotatercept","RECRUITING","2026-01-20",{"date":69,"type":38},"2026-01-21",{"date":71,"type":38},"2025-10-07",{"date":73,"type":21},"2028-04-30",{"name":75,"class":45},"Kazuya Hosokawa",10,{"id":78,"slug":79,"hasResults":11,"nctId":80,"briefTitle":81,"officialTitle":82,"acronym":4,"eligibilityCriteria":83,"healthyVolunteers":11,"sex":17,"minAge":84,"maxAge":85,"enrollmentInfo":86,"targetDuration":4,"studyType":22,"phases":88,"briefSummary":90,"conditions":91,"keywords":92,"overallStatus":66,"whyStopped":4,"lastUpdateSubmitDate":99,"lastUpdatePostDateStruct":100,"startDateStruct":102,"completionDateStruct":104,"leadSponsor":106,"locationsCount":108},"100486222","pentoxifylline-as-an-adjunct-therapy-for-patients-with-eisenmenger-syndrome-100486222","NCT05611268","Pentoxifylline as an Adjunct Therapy for Patients With Eisenmenger Syndrome","Pentoxifylline as an Adjunct Therapy for Patients With Eisenmenger Syndrome: a Randomized Study","Inclusion Criteria:\n\n1. Eisenmenger syndrome in functional class II, III or IV (World Health Organization for Pulmonary Hypertension).\n2. Using or not oral anticoagulation with warfarin.\n\nExclusion Criteria:\n\n1. Hospitalized.\n2. History of relevant and\u002For repetitive bleeding.\n3. Relevant comorbidities with specific treatments.\n4. Systemic syndromes, except Down syndrome.\n5. Candidates for surgical treatment of any nature, except dental.\n6. Clinically manifest systemic infectious or inflammatory disease.\n7. Thrombocytopenia (\\\u003C80x10\\*9 platelets\u002FL).\n8. Patients in chronic anticoagulation regimen other than warfarin.\n9. Diabetics individuals.\n10. Pregnancy in progress, interruption of contraception or amenorrhea.\n11. History of intolerance of pentoxifylline or other xanthine derivatives.\n12. \"Creatinine clearance\" less than or equal to 30 mL\u002Fminute.","10 Years","80 Years",{"count":87,"type":21},48,[89],"NA","The Eisenmenger syndrome corresponds to the most advanced form of pulmonary arterial hypertension associated with congenital heart disease. The syndrome causes chronic hypoxemia, with an increase in erythrocyte mass, which predisposes to thrombotic complications. Pentoxifylline is a xanthine derivative and it is considered as a hemorrheological agent with described effects of reduction in erythrocyte and platelet aggregation, adhesion and activation of leukocytes, and endothelial damage. The main objective of this study is to verify if the chronic oral administration of pentoxifylline to Eisenmenger patients induces an increase in the circulating levels of thrombomodulin, a naturally occurring proteoglycan with anticoagulant, anti thrombotic and anti-inflammatory properties.",[28],[93,94,95,96,97,98],"Pulmonary Hypertension","Congenital Heart Disease","Thrombosis","Pentoxifylline","Thrombomodulin","Tissue Factor","2023-12-14",{"date":101,"type":38},"2023-12-15",{"date":103,"type":38},"2022-06-03",{"date":105,"type":21},"2026-09-30",{"name":107,"class":45},"University of Sao Paulo General Hospital",1]