[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"pulmonary-arterial-hypertension\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:pulmonary-arterial-hypertension":32},{"pageToken":4,"total":5,"offset":6,"count":7,"results":8},null,67,0,25,[9,50,72,121,146,166,188,210,232,252,273,303,328,349,378,395,420,442,466,496,526,553,573,589,613],{"id":10,"slug":11,"hasResults":12,"nctId":13,"briefTitle":14,"officialTitle":15,"acronym":4,"eligibilityCriteria":16,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":19,"enrollmentInfo":20,"targetDuration":4,"studyType":23,"phases":24,"briefSummary":26,"conditions":27,"keywords":34,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":38,"lastUpdatePostDateStruct":39,"startDateStruct":42,"completionDateStruct":44,"leadSponsor":46,"locationsCount":49},"100641631","fibrotic-disease-activity-in-cardiopulmonary-disorders-using-18f-fibroblast-activation-protein-inhibitor-18f-fapi-74-petct-imaging-100641631",false,"NCT07613099","Fibrotic Disease Activity in Cardiopulmonary Disorders Using 18F-Fibroblast Activation Protein Inhibitor (18F-FAPI-74) PET\u002FCT Imaging","Evaluation of Fibrotic Disease Activity in Cardiopulmonary Disorders Using 18F-Fibroblast Activation Protein Inhibitor (18F-FAPI-74 PET\u002FCT Imaging)","* INCLUSION CRITERIA\n\nIn order to be eligible to participate in this study, an individual must meet all of the following criteria:\n\n1. Provision of signed and dated informed consent form\n2. Stated willingness to comply with all study procedures and availability for the duration of the study\n3. Participant \\>=18 years old\n4. Has a specific diagnosis of a cardiopulmonary and\u002For vascular disease that puts them at risk of developing or having developed tissue fibrosis.\n5. Has undergone prior imaging of lungs, heart, and\u002For vasculature with chest CT\n6. Able to lie on the PET\u002FCT scanner for imaging up to 45 minutes.\n7. For females of reproductive potential: use of highly effective contraception for at least 1 month prior to screening and agreement to use such a method during study participation and for an additional 4 weeks after the end of FAPI-PET\u002FCT or the FDG PET\u002FCT scan.\n8. For males of reproductive potential: use of condoms or other methods to ensure effective contraception with partner during study participation and for an additional 4 weeks after the end of FAPI-PET\u002FCT or the FDG PET\u002FCT scan.\n9. Ability of subject to understand and the willingness to sign a written informed consent document.\n\nEXCLUSION CRITERIA\n\nAn individual who meets any of the following criteria will be excluded from participation in this study:\n\n1. History of allergic reactions attributed to compounds of similar chemical or biologic composition to 18F-FAPI-74 or other agents used in the study.\n2. Uncontrolled intercurrent illness, factors, or social situations that would limit compliance with study requirements\n3. Pregnancy or lactation\n4. Women able to become pregnant or men actively trying to father a child and are unwilling to use an effective form of birth control during the study and 4 weeks after the last 18F-FAPI-74 PET\u002FCT scan or the FDG PET\u002FCT scan.\n5. Subjects with severe claustrophobia unresponsive to oral anxiolytics.\n6. Subjects weighing \\> 350 lbs (weight limit for PET scanner table), or unable to fit within the imaging gantry","ALL","18 Years","100 Years",{"count":21,"type":22},210,"ESTIMATED","INTERVENTIONAL",[25],"PHASE3","Background:\n\nInjury or diseases of the heart and lung can sometimes cause scar tissue (fibrosis) to build up in those organs. Current imaging scans can see this scar tissue once it has formed, but researchers want to find a way to detect the fibrosis in its earliest stages, while there might still be time to prevent serious damage. A new tracer (a radioactive substance injected during imaging scans) may be able to help.\n\nObjective:\n\nTo test a new tracer (18F-FAPI-74) during imaging scans in people with heart or lung disease.\n\nEligibility:\n\nPeople aged 18 years and older with lung or heart disease that may cause scarring in those organs.\n\nDesign:\n\nParticipants will have 6 clinic visits over 2 years.\n\nParticipants will be screened: They will have blood tests and tests of their heart and lung function. Those with heart disease will have a magnetic resonance imaging (MRI) scan of the heart.\n\nThe study tracer will be used with positron emission tomography (PET)\u002Fcomputed tomography (CT) scans. The study tracer will be injected into a vein in the arm. Participants will lie on a padded bed that slides through a donut-shaped machine.\n\nParticipants will have scans with the study tracer 2 times, 8 to 12 months apart. They will also have standard CT scans and blood tests during these visits. They will also have blood tests at 3 and 6 months between these visits.\n\nParticipants will have a follow-up visit after 18 to 24 months. The study scans, MRI and standard CT scans, and lung function tests may be repeated....",[28,29,30,31,32,33],"Allogeneic Stem Cell Transplantation","Lung Allograft Transplantation","Interstitial Lung Disease","Acute Lung Injury","Pulmonary Arterial Hypertension","Cardiovascular Diseases",[35,36],"Fibroblast activation protein (FAP) FAPI\u002FPET","Fibrotic Disease Activity","RECRUITING","2026-08-20",{"date":40,"type":41},"2026-08-21","ACTUAL",{"date":43,"type":22},"2026-08-26",{"date":45,"type":22},"2033-05-26",{"name":47,"class":48},"National Heart, Lung, and Blood Institute (NHLBI)","NIH",1,{"id":51,"slug":52,"hasResults":12,"nctId":53,"briefTitle":54,"officialTitle":55,"acronym":56,"eligibilityCriteria":57,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":58,"targetDuration":4,"studyType":23,"phases":60,"briefSummary":61,"conditions":62,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":38,"lastUpdatePostDateStruct":63,"startDateStruct":64,"completionDateStruct":66,"leadSponsor":68,"locationsCount":71},"100609703","a-clinical-study-of-sotatercept-mk-7962-in-people-with-pulmonary-arterial-hypertension-mk-7962-038-100609703","NCT07218029","A Clinical Study of Sotatercept (MK-7962) in People With Pulmonary Arterial Hypertension (MK-7962-038)","An Open-label Long-term Follow-up Study to Evaluate the Effects of Sotatercept When Added to Background Pulmonary Arterial Hypertension (PAH) Therapy for the Treatment of PAH (MK-7962-038)","SOTERIA","Inclusion Criteria:\n\nThe main inclusion criteria include but are not limited to the following:\n\n* Has completed their current respective PAH sotatercept clinical study and its requirements, and must not have discontinued early\n* Is willing to adhere to the study visit schedule, and understands and will comply with all protocol requirements\n* Must have the ability to understand and provide documented informed consent\n\nExclusion Criteria:\n\nThe main exclusion criteria include but are not limited to the following:\n\n* Did not participate in a sotatercept PAH parent study\n* Missed more than the equivalent of 4 consecutive doses between the end of parent study and the start of this study.\n* Presence of an ongoing serious adverse event that occurred during a PAH sotatercept clinical study that is assessed to be possibly or probably related to sotatercept\n* Is a female who is pregnant or breastfeeding\n* Is or has an immediate family member who is investigational site or Sponsor staff directly involved with this study\n* Is currently enrolled in another investigational product study other than a sotatercept study\n* Is incapacitated",{"count":59,"type":22},815,[25],"Researchers are looking for more ways to treat PAH. In PAH, the blood vessels in the lungs become thick and narrow, which makes it harder for blood to flow. This causes high blood pressure in the lungs and overworks the heart. PAH can make it hard to breathe and be active. Some standard (usual) treatments for PAH can treat symptoms of PAH but do not stop PAH from getting worse.\n\nSotatercept is a study medicine designed to treat PAH. It is a targeted therapy, which is a treatment that works on certain proteins that play a role in causing PAH.\n\nThis is a long-term follow-up (LTFU) study. People who took part in certain other studies testing sotatercept for PAH may be able to join this study. The goal of this study is to learn about the long-term safety of sotatercept and if people tolerate it when taken with standard PAH treatment over a longer period of time.",[32],{"date":40,"type":41},{"date":65,"type":41},"2021-05-12",{"date":67,"type":22},"2028-12-07",{"name":69,"class":70},"Merck Sharp & Dohme LLC","INDUSTRY",134,{"id":73,"slug":74,"hasResults":12,"nctId":75,"briefTitle":76,"officialTitle":77,"acronym":78,"eligibilityCriteria":79,"healthyVolunteers":80,"sex":17,"minAge":81,"maxAge":82,"enrollmentInfo":83,"targetDuration":4,"studyType":85,"phases":4,"briefSummary":86,"conditions":87,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":38,"lastUpdatePostDateStruct":112,"startDateStruct":113,"completionDateStruct":115,"leadSponsor":117,"locationsCount":120},"100383878","pharmacokinetics-pharmacodynamics-and-safety-profile-of-understudied-drugs-administered-to-children-per-standard-of-care-pops-100383878","NCT04278404","Pharmacokinetics, Pharmacodynamics, and Safety Profile of Understudied Drugs Administered to Children Per Standard of Care (POPS)","Pharmacokinetics, Pharmacodynamics, and Safety Profile of Understudied Drugs","POPS or POP02","Inclusion Criteria:\n\n1. Participant is \\\u003C 21 years of age\n2. Parent\u002F Legal Guardian\u002F Adult Participant can understand the consent process and is willing to provide informed consent\u002FHIPAA:\n3. (a) Participant is receiving one or more of the study drugs of interest at the time of enrollment or (b) Participant is NOT receiving one or more of the study drugs of interest but is SARS-COV-2 positive within 60 days prior to enrollment\n\nExclusion Criteria:\n\n1. Participant has a known pregnancy\n\n   Below exclusion criteria apply only to:\n\n   Participants receiving one or more of the study drugs of interest at the time of enrollment, DOI administration or PK sampling: (Refer to DOI specific appendices for details on enrollment cohort specifications and additional eligibility criteria)\n2. Has had intermittent dialysis within previous 24 hours\n3. Has had a kidney transplant within previous 30 days\n4. Has had a liver transplant within previous 1 year\n5. Has had a stem cell transplant within previous 1 year\n6. Has had therapeutic hypothermia within previous 24 hours\n7. Has had plasmapheresis within the previous 24 hours\n8. Has a Ventricular Assist Device\n9. Has any condition which would make the participant, in the opinion of the investigator, unsuitable for the study",true,"0 Years","20 Years",{"count":84,"type":22},5000,"OBSERVATIONAL","The study investigators are interested in learning more about how drugs, that are given to children by their health care provider, act in the bodies of children and young adults in hopes to find the most safe and effective dose for children. The primary objective of this study is to evaluate the PK of understudied drugs currently being administered to children per SOC as prescribed by their treating provider.",[88,32,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109,110,111],"Coronavirus Infection (COVID-19)","Urinary Tract Infections in Children","Hypertension","Pain","Hyperphosphatemia","Primary Hyperaldosteronism","Edema","Hypokalemia","Heart Failure","Hemophilia","Menorrhagia","Insomnia","Pneumonia","Skin Infection","Arrythmia","Asthma in Children","Bronchopulmonary Dysplasia","Adrenal Insufficiency","Fibrinolysis; Hemorrhage","Attention Deficit Hyperactivity Disorder","Multisystem Inflammatory Syndrome in Children (MIS-C)","Kawasaki Disease","Coagulation Disorder","Down Syndrome",{"date":40,"type":41},{"date":114,"type":41},"2020-03-05",{"date":116,"type":22},"2026-12",{"name":118,"class":119},"Duke University","OTHER",52,{"id":122,"slug":123,"hasResults":12,"nctId":124,"briefTitle":125,"officialTitle":126,"acronym":127,"eligibilityCriteria":128,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":129,"enrollmentInfo":130,"targetDuration":4,"studyType":23,"phases":132,"briefSummary":133,"conditions":134,"keywords":135,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":136,"lastUpdatePostDateStruct":137,"startDateStruct":139,"completionDateStruct":141,"leadSponsor":143,"locationsCount":145},"100630000","phase-3-a-study-to-evaluate-the-efficacy-and-safety-of-once-daily-treprostinil-palmitil-inhalation-powder-tpip-in-participants-with-pulmonary-arterial-hypertension-pah-100630000","NCT07481981","A Study to Evaluate the Efficacy and Safety of Once Daily Treprostinil Palmitil Inhalation Powder (TPIP) in Participants With Pulmonary Arterial Hypertension (PAH)","A Phase 3, Randomized, Double-Blind, Placebo-controlled, Multicenter, Parallel-group Study to Evaluate the Efficacy and Safety of Once Daily Treprostinil Palmitil Inhalation Powder in Participants With Pulmonary Arterial Hypertension","PALM-PAH","Inclusion Criteria\n\n* Participants must have a diagnosis of World Health Organisation (WHO) Group 1 pulmonary hypertension (PAH) in any of the following subtypes, in accordance with European Society of Cardiology European Respiratory Society (ESC\u002FERS) Guidelines:\n\n  * Idiopathic PAH\n  * Heritable PAH\n  * Drug\u002Ftoxin-induced PAH\n  * Connective tissue disease (CTD)-associated PAH\n  * PAH associated with congenital heart disease-related to simple systemic-to-pulmonary shunt at least 1 year following repair.\n* PAH diagnosis for at least 3 months prior to Screening.\n* New York Heart Association (NYHA) or World Health Organization (WHO) functional class II-IV.\n* Participants must be on stable PAH therapy consisting of 1 to 3 medications from the following classes:\n\n  * Endothelin receptor antagonists (eg, ambrisentan, bosentan, macitentan) for at least 90 days prior to Screening with the last 30 days on stable dose\n  * Phosphodiesterase type 5 inhibitors (eg, sildenafil, tadalafil) for at least 90 days prior to Screening with the last 30 days on stable dose\n  * Guanylate cyclase stimulator (eg, riociguat) for at least 90 days prior to Screening with the last 30 days on stable dose\n  * Activin signaling inhibitor (e.g., sotatercept) for at least 6 months prior to Screening, with the last 3 months on stable dose and meeting all the following conditions:\n\n    * no active clinically significant bleeding (eg, epistaxis and gingival bleeding requiring medical interventions) within the past 3 months.\n    * no history of major bleeding events or risks (eg, gastrointestinal or intracranial bleeding) within the past 6 months.\n    * platelet counts ≥100,000 per microlitre (μL) at Screening\n  * For both 6-minute walk tests (6MWTs), the values of 6-minute walk distance (6MWD) should be ≥ 150 and ≤ 450 meters at Screening.\n* Right heart catheterization (RHC) at Screening (or within 6 months prior to Screening, if available). Prior RHC may be used provided there has been no change in background PAH therapy and doses. The RHC must meet all of the following hemodynamic criteria:\n\n  * Mean pulmonary arterial pressure (PAP) \\>20 millimetre of mercury (mmHg) at rest.\n  * pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) ≤15 mmHg.\n  * pulmonary vascular resistance (PVR) of ≥5 wood units (WU).\n\nExclusion Criteria\n\n* Diagnosis of PH WHO Groups 2, 3, 4, or 5, or subtypes of PH WHO Group 1 other than described in inclusion criterion 2 (eg, human immunodeficiency virus (HIV), complex congenital heart disease-associated PAH, portal hypertension-associated PAH, pulmonary veno-occlusive disease, Schistosomiasis associated PAH).\n* Clinically significant left heart disease, including left-sided valvular disease, left ventricular systolic or diastolic dysfunction, echocardiographic findings suggestive of post-capillary pulmonary hypertension, unstable ischemic heart disease, or unstable arrhythmias.\n* Evidence of airflow obstruction defined by forced expiratory volume in 1 second (FEV1) per forced vital capacity (FVC) \\\u003C0.7.\n* Evidence of significant restrictive lung disease as evidenced by FVC \\\u003C70% predicted normal.\n* Evidence of chronic thromboembolic disease or recent (within 6 months of Screening) acute pulmonary embolism.\n* Known hypersensitivity or contraindication to treprostinil or TPIP or TPIP formulation excipients (e.g., mannitol, leucine).\n* Any other medical or psychological condition including relevant laboratory abnormalities at Screening that, in the opinion of the Investigator, suggest a new and\u002For insufficiently understood disease and\u002For may present an unreasonable risk to the study participant as a result of his\u002Fher participation in this clinical trial, may impede their ability complete the study or the study assessments or confound the outcomes of the trial.\n\nNote: Other protocol-defined inclusion\u002Fexclusion criteria may apply.","75 Years",{"count":131,"type":22},344,[25],"The primary objective of this study is to evaluate the effect of 24-weeks of once daily treatment with TPIP compared with placebo on exercise capacity in adults with PAH.",[32],[32],"2026-08-18",{"date":138,"type":41},"2026-08-19",{"date":140,"type":41},"2026-07-21",{"date":142,"type":22},"2029-01-28",{"name":144,"class":70},"Insmed Incorporated",3,{"id":147,"slug":148,"hasResults":12,"nctId":149,"briefTitle":150,"officialTitle":150,"acronym":151,"eligibilityCriteria":152,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":153,"targetDuration":4,"studyType":23,"phases":155,"briefSummary":157,"conditions":158,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":136,"lastUpdatePostDateStruct":159,"startDateStruct":160,"completionDateStruct":162,"leadSponsor":164,"locationsCount":49},"100474914","the-mobile-health-intervention-in-pulmonary-arterial-hypertension-move-pah-study-100474914","NCT05464095","The MObile Health InterVEntion in Pulmonary Arterial Hypertension (MOVE PAH) Study","MOVE PAH)","Inclusion Criteria:\n\n* Adults aged 18 or older.\n* Diagnosed with idiopathic, heritable, or associated (connective tissue disease, drugs, or toxins) pulmonary arterial hypertension (PAH), or PAH due to simple congenital heart disease (i.e. atrial septal defect).\n* WHO functional class I-III\n* Stable PAH-specific medication regimen for three months prior to enrollment. Subjects with only a single diuretic adjustment in the prior three months will be included. Adjustments in IV prostacyclin for side effect management are allowed.\n* Forced vital capacity \\>65% predicted with no or minimal interstitial lung disease based on reviews of imaging studies by PI and medical monitor.\n\nExclusion Criteria:\n\n* Prohibited from normal activity due to wheelchair bound status, bed bound status, reliance on a cane\u002Fwalker, activity-limiting angina, activity-limiting osteoarthritis, or other condition that limits activity.\n* Pregnancy\n* Diagnosis of PAH etiology other than idiopathic, heritable, or associated.\n* Functional class IV heart failure\n* Requirement of \\> 2 diuretic adjustment in the prior three months.\n* Preferred form of activity is not measured by an activity tracker (swimming, yoga, ice skating, stair master, or activities on wheels such as bicycling or rollerblading).",{"count":154,"type":22},100,[156],"NA","Patients with pulmonary arterial hypertension (PAH) have reduced health related quality of life (HRQOL) and impaired exercise capacity. Despite fourteen approved therapies, most patients die within ten years. Increasing physical activity is highly efficacious in PAH, resulting in six-minute walk distance (6MWD) and HRQOL improvement that often exceeds the effect of medications. Prior activity studies required inpatient rehabilitation, which is impractical, hard to sustain, and poorly scalable to a rare disease.\n\nThe Investigators propose a randomized trial of smart texts versus usual care for 6 months. The Investigators will randomize 100 PAH patients to the mHealth intervention or usual care. The Investigators will test the effect of a text-based mHealth intervention on HRQOL in PAH using the PAH-specific emPHasis-10 questionnaire. The Investigators will also test the effect of an mHealth intervention on exercise capacity, measured by a supervised home-based 6MWD test. Finally, the Investigators will examine the effect of the intervention on time to clinical worsening (composite of PAH therapy escalation, PAH hospitalization, and death) one year after randomization.",[32],{"date":38,"type":41},{"date":161,"type":41},"2023-01-01",{"date":163,"type":22},"2027-08-31",{"name":165,"class":119},"Vanderbilt University Medical Center",{"id":167,"slug":168,"hasResults":12,"nctId":169,"briefTitle":170,"officialTitle":171,"acronym":4,"eligibilityCriteria":172,"healthyVolunteers":12,"sex":17,"minAge":173,"maxAge":174,"enrollmentInfo":175,"targetDuration":4,"studyType":23,"phases":177,"briefSummary":179,"conditions":180,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":181,"lastUpdatePostDateStruct":182,"startDateStruct":183,"completionDateStruct":185,"leadSponsor":187,"locationsCount":49},"100641996","phase-2-a-study-of-sotatercept-mk-7962-in-japanese-children-with-pulmonary-arterial-hypertension-pah-mk-7962-032-100641996","NCT07646860","A Study of Sotatercept (MK-7962) in Japanese Children With Pulmonary Arterial Hypertension (PAH) (MK-7962-032)","A Phase 2 Open-label Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Efficacy of Sotatercept (MK-7962) in Japanese Children From 1 to Less Than 18 Years of Age With PAH on Standard of Care.","Inclusion Criteria:\n\nThe main inclusion criteria include but are not limited to the following:\n\n* Has pulmonary arterial hypertension (PAH, World Health Organization Group 1) confirmed by a prior right heart catheterization\n* Has idiopathic, heritable, drug- or toxin-induced PAH, connective tissue disease-associated PAH, repaired congenital heart disease-associated PAH, or PAH with coincidental shunt\n* Has PAH classified as World Health Organization Functional Class I, or symptomatic World Health Organization Functional Class II to IV\n* Has been receiving stable standard-of-care background therapy for PAH for at least 90 days\n* Is Japanese\n\nExclusion Criteria:\n\nThe main exclusion criteria include but are not limited to the following:\n\n* History of left-sided heart disease\n* Has severe congenital or developmental abnormalities of the lung, thorax, and\u002For diaphragm\n* History of Eisenmenger syndrome, Potts shunt, or recent atrial septostomy within 180 days\n* Has unrepaired or residual cardiac shunt with Qp\u002FQs \\>1.5\n* Has pulmonary veno-occlusive disease, pulmonary capillary hemangiomatosis, or overt signs of pulmonary capillary and\u002For venous involvement\n* PAH associated with portal hypertension\n* Known visceral arteriovenous malformations\n* History of full or partial pneumonectomy\n* Untreated more than mild obstructive sleep apnea\n* History of known pericardial constriction\n* Family history of sudden cardiac death or long QT syndrome\n* History of symptomatic coronary disease within 6 months or cerebrovascular accident within 3 months\n* Prior treatment with sotatercept or luspatercept","1 Year","17 Years",{"count":176,"type":22},6,[178],"PHASE2","The goal of this study is to learn about the safety of sotatercept and how well Japanese children tolerate it, when taken along with standard (usual) pulmonary arterial hypertension (PAH) treatment. Researchers also want to learn what happens to it in a person's body over time and whether it lowers resistance in blood vessels in the lungs.",[32],"2026-08-17",{"date":138,"type":41},{"date":184,"type":22},"2026-08-31",{"date":186,"type":22},"2031-05-16",{"name":69,"class":70},{"id":189,"slug":190,"hasResults":12,"nctId":191,"briefTitle":192,"officialTitle":193,"acronym":4,"eligibilityCriteria":194,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":19,"enrollmentInfo":195,"targetDuration":4,"studyType":23,"phases":197,"briefSummary":198,"conditions":199,"keywords":200,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":202,"lastUpdatePostDateStruct":203,"startDateStruct":204,"completionDateStruct":206,"leadSponsor":208,"locationsCount":49},"100187213","phase-2-spironolactone-for-pulmonary-arterial-hypertension-100187213","NCT01712620","Spironolactone for Pulmonary Arterial Hypertension","A Pilot Study of the Effect of Spironolactone Therapy on Exercise Capacity and Endothelial Dysfunction in Pulmonary Arterial Hypertension","* INCLUSION CRITERIA:\n\n  1. WHO Group 1 PH patients on either no medical therapy or stable medical therapy for at least the past 4 weeks (defined as no new PAH-specific therapy, no change in the dose of current PAH-specific therapy and no change in NYHA\u002FWHO functional classification within the past 4 weeks) are eligible. The following parameters on RHC are required to meet the hemodynamic definition of PAH:\n\n  \u003C!-- -->\n\n  1. mean pulmonary artery pressure of \\> 25 mmHg at rest,\n  2. pulmonary capillary wedge pressure of less than or equal to 15 mmHg (or a left ventricular end-diastolic pressure of less than or equal to 12 mmHg) and\n  3. pulmonary vascular resistance of \\> 3 Wood units (240 dyn.s.cm\\^-5).\n\nIf clinically indicated at the time of enrollment, then a RHC will be performed at the NIH Clinical Center upon study entry under a procedural consent.\n\n2\\) Females who are able to become pregnant (i.e., are not postmenopausal, have not undergone surgical sterilization, and are sexually active with men) must agree to use adequate contraception (hormonal or barrier method of birth control; abstinence) prior to and for the duration of study participation.\n\nEXCLUSION CRITERIA:\n\n1. Patients with WHO Group 1 PH and evidence of right heart failure as defined by:\n\n   1. NYHA\u002FWHO class IV symptoms and\n   2. Echocardiographic evidence of severe RV dysfunction and\n   3. Clinical evidence of right heart failure which may include, but is not limited to elevated jugular venous pressure, ascites, and lower extremity edema\n2. Patients with WHO Group 1 pulmonary hypertension and a prior diagnosis of cirrhosis with portal hypertension as evidenced by a history of ascites, hepatic encephalopathy and\u002For varices prior to enrollment\n3. Patients with WHO Group 1 pulmonary hypertension and evidence of active infection, (HIV patients with two consecutive viral loads of \\\u003C 500 on their most recent determinations within the past 12 months will be considered to have inactive infection)\n4. Patients with WHO Group 1 pulmonary hypertension who have taken spironolactone or eplerenone within the last 30 days\n5. Known or suspected allergy to spironolactone\n6. Pregnant or breastfeeding women (all women of childbearing potential will be required to have a screening urine or blood pregnancy test)\n7. Age \\\u003C18 years\n8. Inability to provide informed written consent for participation in the study\n9. Chronic kidney disease (an estimated glomerular filtration rate of \\\u003C 35 mL\u002Fmin\u002F1.73m\\^2 of body surface area)\n10. Serum potassium at the time of enrollment of \\> 5 mEq\u002FL\n11. Concurrent use of an ACE inhibitor and angiotensin II receptor blocker\n\n    OR\n\n    Patients currently taking the maximum recommended dose of an ACE inhibitor or an angiotensin II receptor blocker \\[For patients taking one of these medicines (ACE-Inhibitors or ARBs), the investigators agree to do due diligence by consulting a clinical center pharmacist and\u002For a standard pharmacy reference (i.e. Micromedex) to certify whether or not the patient is on a maximum dose of the drug.\\]\n12. Women currently taking drospirenone-containing oral contraceptives\n\nThe estimated glomerular filtration rate (eGFR) will be calculated using the IDMS-traceable Modification of Diet in Renal Disease (MDRD) equation and corrected for body surface area.\n\neGFR = 175 x (serum creatinine in mg\u002FdL)\\^-1.154 x (age in years)\\^-0.203 x \\[1.212 if African-American\\] x \\[0.742 if female\\]\n\n(http:\u002F\u002Fwww.nkdep.nih.gov\u002Fprofessionals\u002Fgfr\\_calculators\u002Fidms\\_con.htm)\n\nExclusion Criteria for MRI\n\nThese contraindications include but are not limited to the following devices or conditions:\n\n1. Implanted cardiac pacemaker or defibrillator\n2. Cochlear Implants\n3. Ocular foreign body (e.g. metal shavings)\n4. Embedded shrapnel fragments\n5. Central nervous system aneurysm clips\n6. Implanted neural stimulator\n7. Any implanted device that is incompatible with MRI\n8. Unsatisfactory performance status as judged by the referring physician such that the subject could not tolerate an MRI scan. Examples of medical conditions that would not be accepted would include unstable angina and severe dyspnea at rest\n9. Subjects requiring monitored sedation for MRI studies\n10. Subjects with a condition precluding entry into the scanner (e.g. morbid obesity, claustrophobia, etc.)\n11. Subjects with severe back-pain or motion disorders who will be unable to tolerate supine positioning within the MRI scanner and hold still for the duration of the examination.\n\nEXCLUSION CRITERIA FOR GADOLINIUM BASED MRI STUDIES ONLY:\n\n1. History of severe allergic reaction to gadolinium contrast agents despite pre- medication with diphenhydramine and prednisone\n2. Chronic kidney disease (an estimated glomerular filtration rate of \\\u003C 60 mL\u002Fmin\u002F1.73m\\^2 of body surface area)",{"count":196,"type":22},70,[178],"Background:\n\n\\- High blood pressure in the lungs, known as pulmonary arterial hypertension (PAH), is a rare disorder. In spite of recent advances in treatment, the death rate remains unacceptably high. Lung blood vessel function can be harmed by progressive injuries, such as inflammation, leading to worsening of the disease. A drug called spironolactone has been known to improve blood vessel function and reduce inflammation. Some people with PAH take spironolactone to help treat fluid retention. However, its effect on inflammation and blood vessel function in patients with PAH is not known. Researchers want to see if spironolactone can help these conditions in people with PAH.\n\nObjectives:\n\n\\- To test the effectiveness of spironolactone in treating pulmonary arterial hypertension.\n\nEligibility:\n\n\\- Individuals at least 18 years of age with pulmonary arterial hypertension.\n\nDesign:\n\n* This study will last for 24 weeks. Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected.\n* Participants will take either spironolactone or a placebo. They will take their study drug or placebo for 7 weeks. Treatment will be monitored with regular blood tests.\n* In Week 8, participants who have had no reaction to the treatment will receive a higher dose of the drug or placebo.\n* In Week 12, participants will have a study visit with heart and lung function tests. They will also have a 6-minute walk test, and provide blood and urine samples.\n* After additional study visits for blood samples, participants will have a final visit in Week 24. The tests from Week 12 will be repeated at this visit.",[32],[201],"Magnetic Resonance Imaging","2026-08-14",{"date":181,"type":41},{"date":205,"type":41},"2014-01-10",{"date":207,"type":22},"2026-12-31",{"name":209,"class":48},"National Institutes of Health Clinical Center (CC)",{"id":211,"slug":212,"hasResults":12,"nctId":213,"briefTitle":214,"officialTitle":215,"acronym":4,"eligibilityCriteria":216,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":217,"targetDuration":4,"studyType":23,"phases":219,"briefSummary":221,"conditions":222,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":223,"lastUpdatePostDateStruct":224,"startDateStruct":225,"completionDateStruct":227,"leadSponsor":229,"locationsCount":231},"100603971","phase-1-a-study-of-hs235-for-treatment-of-pulmonary-arterial-hypertension-pah-in-adults-100603971","NCT07143448","A Study of HS235 for Treatment of Pulmonary Arterial Hypertension (PAH) in Adults","Phase 1b\u002F2, Multicenter, Randomized, Double-Blind, Placebo-Controlled, Dose-Ranging Study Assessing the Safety, Pharmacokinetics, Pharmacodynamics, and Efficacy of HS235 Added to Background Treatment in Adults With PAH","Inclusion Criteria:\n\nParticipants are eligible to be included in the study only if they meet all of the following criteria:\n\n1. Male and female participants, 18 years of age and older.\n2. Body Mass Index (BMI) 18.5 and 40 kg\u002Fm2 and body weight at or below 120 kg.\n3. Baseline RHC performed during the Screening Period documenting:\n\n   1. A minimum PVR of ≥ 5 Wood units (WU) or ≥ 400 dyn·sec·cm-5 and,\n   2. A pulmonary artery wedge pressure (PAWP) of ≤ 15 mm Hg.\n4. Diagnosis of WHO pulmonary arterial hypertension (PAH) Group 1.\n5. Symptomatic PAH classified as WHO Functional class II to IV.\n6. On stable doses of at least 2 approved background PAH therapies and diuretics.\n7. 6-Minute Walk Distance (6MWD) ≥ 150 m and ≤ 500 m repeated twice during screening.\n8. Ability to adhere to study visit schedule and understand and comply with all protocol requirements.\n\nExclusion Criteria:\n\n1. PH WHO Groups 2, 3, 4 or 5.\n2. Hospitalization for any worsening medical condition or major surgery within 4 weeks prior to screening.\n3. Any symptomatic coronary disease events.\n4. History of heart transplant or on heart transplant list.\n5. Uncontrolled systemic hypertension.\n6. History of restrictive, constrictive or congestive cardiomyopathy.\n7. History of significant mitral regurgitation or aortic regurgitation valvular disease.\n8. Untreated or poorly controlled moderate or severe obstructive sleep apnea.\n9. A diagnosis of worse than moderate COPD or worse than mild ILD.\n10. Cerebrovascular event within 90 days prior to screening\n11. A pneumonectomy or lobectomy\n12. Having received an activin signaling inhibitor such as sotatercept at any time prior to screening\n13. A Hgb \\> sex-specific ULN\n14. Platelets count \\\u003C 100,000\u002Fmm3\n15. Severe (stage ≥4) renal failure with eGFR \\\u003C 30 mL\u002Fmin\u002Fm2\n16. Abnormally elevated liver function enzymes (ALT or AST \\> 3X ULN, or total bilirubin \\> 1.5X ULN).",{"count":218,"type":22},90,[220,178],"PHASE1","Phase 1b\u002F2 Study of HS235 Assessing the Safety, Pharmacokinetics, Pharmacodynamics, and Efficacy of the Activin Signaling Inhibitor HS235 Added to Background Treatment in Adults with Pulmonary Arterial Hypertension (PAH)",[32],"2026-08-13",{"date":181,"type":41},{"date":226,"type":22},"2026-08",{"date":228,"type":22},"2028-11",{"name":230,"class":70},"35Pharma Inc",2,{"id":233,"slug":234,"hasResults":12,"nctId":235,"briefTitle":236,"officialTitle":236,"acronym":237,"eligibilityCriteria":238,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":239,"targetDuration":4,"studyType":23,"phases":241,"briefSummary":242,"conditions":243,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":244,"lastUpdatePostDateStruct":245,"startDateStruct":246,"completionDateStruct":248,"leadSponsor":250,"locationsCount":145},"100592362","empagliflozin-to-improve-right-ventricular-function-in-pulmonary-arterial-hypertension-100592362","NCT06992440","Empagliflozin to Improve Right Ventricular Function in Pulmonary Arterial Hypertension","EmPATH","Inclusion Criteria:\n\n* In order to be eligible to participate in this study, an individual must meet all the following criteria:\n\n  1. Provision of signed and dated informed consent form\n  2. Ability and stated willingness to comply with all study procedures and availability for the duration of the study\n  3. Ability to read and write in English\n  4. Male or female, aged 18 years or older, with group 1 PAH, idiopathic, heritable, associated with drugs and toxins, associated with connective tissue disease and with congenital heart disease (simple repaired or unrepaired defects) according to the current guidelines and adjudicated by the local PI\n  5. PAH confirmed by right heart catheterization in the last 5 years\n  6. RV dysfunction defined as FAC ≤ 40.0% on echocardiography performed during the screening visit. In PVDOMICS, FAC has a strong correlation with CMR RV ejection fraction; group 1 PAH patients with an FAC ≤ 40% (mean 27.0 ± 8.0%) had a mean RV ejection fraction of 35.5 ± 11.3% CMR RV ejection fraction ≤ 54% classifies PAH as intermediate (37-54%) or high (\\\u003C37%) risk under current guidelines. However, to ensure we recruit patients with significantly impaired RV function, patients with RV FAC between 35.0-40.0% on the screening echocardiogram will have to meet at least one of the following additional criteria: a) TAPSE\u002FsPAP ratio \\\u003C 0.31 mm\u002FmmHg; b) elevated right atrial pressure defined as inferior vena cava (IVC) \\> 2.1 cm in diameter and the IVC does not collapse with sniff\u002Frespiration; c) RV enlargement defined as RV basal diameter ≥ 4.1 cm; d) RV free wall longitudinal strain ≥ -22%; e) Interventricular septal flattening; and f) RV outflow tract mid or late systolic notching.\n  7. On FDA-approved PAH-targeted therapy (any combination including infused prostacyclin analogues and sotatercept) with stable doses for at least 8 weeks or 24 weeks for sotatercept prior to the screening visit and no clinical plans to change this therapy.\n  8. Diuretic doses stable for at least 4 weeks prior to screening. After screening, diuretic doses may be changed as directed by the site PIs and\u002For the treating physician.\n  9. Ability to take oral medication and willingness to adhere to the study drug regimen.\n  10. For females of reproductive potential: use of highly effective contraception for at least 4 weeks prior to screening and agreement to use such a method during study participation and for an additional 2 weeks after the end of study drug administration\n  11. Able to have baseline and week 24 CMR according to Imaging Core criteria, as adjudicated by the Site PI\n\nExclusion Criteria:\n\n* An individual who meets any of the following criteria will be excluded from participation in this study:\n\n  1. Current use of insulin, insulin secretagogues (sulfonylureas and meglitinides), lithium or an SGLT2 inhibitor\n  2. Use of an SGLT2 inhibitor within the past 3 months prior to screening\n  3. Prior documented inability to tolerate an SGLT2 inhibitor\n  4. Volume depletion, as ascertained by the site PI, at screening or baseline\n  5. History of diabetic ketoacidosis or type 1 diabetes mellitus\n  6. Chronic alcohol or drug abuse\n  7. More than one bacterial or yeast genitourinary tract infection in the year prior to enrollment\n  8. Estimated glomerular filtration rate under 30 mL\u002Fminute\u002F1.73m2 or on renal replacement therapy\n  9. Pregnancy or lactation\n  10. Known allergy or hypersensitivity to empagliflozin or another SGLT-2 inhibitor\n  11. Currently taking or has taken another investigational drug within the past 4 weeks\n  12. Enrollment in another randomized intervention trial. (Participants participating in observational trials will not be excluded).\n  13. Decompensated right heart failure, as adjudicated by the site PI.\n  14. Screening HbA1c \\>10% with symptoms such as polyuria and polydipsia",{"count":240,"type":22},78,[178],"Randomized, triple-masked, parallel arm clinical trial of empagliflozin versus placebo in pulmonary arterial hypertension (PAH) participants on stable approved PAH-targeted medical therapy.",[32],"2026-08-11",{"date":223,"type":41},{"date":247,"type":41},"2025-06-26",{"date":249,"type":22},"2030-09",{"name":251,"class":119},"Gustavo A Heresi, MD, MS",{"id":253,"slug":254,"hasResults":12,"nctId":255,"briefTitle":256,"officialTitle":257,"acronym":4,"eligibilityCriteria":258,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":129,"enrollmentInfo":259,"targetDuration":4,"studyType":23,"phases":261,"briefSummary":262,"conditions":263,"keywords":4,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":265,"lastUpdatePostDateStruct":266,"startDateStruct":267,"completionDateStruct":269,"leadSponsor":271,"locationsCount":49},"100650834","phase-1-microbiota-transplant-with-antibiotic-preconditioning-and-fiber-supplementation-in-pah-100650834","NCT07751120","Microbiota Transplant With Antibiotic Preconditioning and Fiber Supplementation in PAH.","A Pilot Study of Microbiota Transplant With Antibiotic Preconditioning and Fiber Supplementation in Pulmonary Arterial Hypertension (GUT RESTORE-PAH).","Inclusion Criteria:\n\n* Provision of signed and dated consent form\n* Ages 18-75\n* Diagnosis of PAH\n* On stable treatment for PAH for one month prior to enrollment\n* Able to swallow capsules\n* Able to provide blood sample and fecal sample\n* Stated willingness to comply with all study procedures and availability for the duration of trial to follow-up by telephone, in-person, email, and\u002For video visits or correspondence.\n\nExclusion Criteria:\n\n* Dysphagia to pills\n* Clinically active inflammatory bowel disease\n* Pregnancy or breastfeeding. A pregnancy test will be obtained from females of child- bearing potential at the screening visit or day 1 (prior to the receipt of MTT). Patients with a positive pregnancy test will be excluded. A negative result will be required for subjects who are females of child-bearing potential to receive MTT. Patients will be counseled to avoid pregnancy which is the standard of care for patients with PAH.\n* Life expectancy of \\\u003C 6 months\n* Presence of ileostomy or colostomy\n* Patients on immunosuppressants (calcineurin inhibitors, prednisone ≥ 20 mg\u002Fday, methotrexate, azathioprine, immunosuppressive biologics, JAK inhibitors).\n* Patients with neutropenia (an absolute neutrophil count \\\u003C 0.5 x 10 9 cells\u002FL)",{"count":260,"type":22},24,[220],"This pilot clinical trial analysis will evaluate the initial safety, feasibility, and pharmacokinetics of microbiota transplant therapy (MTT) with antibiotic pre-conditioning and fiber supplementation vs. placebo in patients with pulmonary arterial hypertension (PAH). This trial will inform development of future trials of MTT as a treatment for PAH. 24 PAH patients will be randomized to receive either MTT with antibiotic preconditioning + fiber supplementation, MTT with antibiotic preconditioning + placebo supplementation, or placebo + placebo supplementation. MTT will in a capsule form composed of freeze-dried, encapsulated intestinal microbiota from healthy donors. Fiber supplementation will be 10-14 gm oral fiber supplement. Patients will be followed at week 1, week 2, week 4, week 12, and week 24. Patient will undergo stool sample collection at baseline, week 1, week 4, and week 12, blood sample collection at baseline, week 4, and week,12. In addition, patient will undergo an echocardiogram, six-minute walk test (6MWT) and quality of life questionnaire at baseline and at week 12.",[32],"NOT_YET_RECRUITING","2026-08-10",{"date":223,"type":41},{"date":268,"type":22},"2026-09-01",{"date":270,"type":22},"2030-04-30",{"name":272,"class":119},"University of Minnesota",{"id":274,"slug":275,"hasResults":12,"nctId":276,"briefTitle":277,"officialTitle":278,"acronym":279,"eligibilityCriteria":280,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":281,"enrollmentInfo":282,"targetDuration":4,"studyType":23,"phases":284,"briefSummary":285,"conditions":286,"keywords":287,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":294,"lastUpdatePostDateStruct":295,"startDateStruct":297,"completionDateStruct":299,"leadSponsor":301,"locationsCount":4},"100651323","phase-2-cs1-added-to-standard-of-care-therapy-for-pulmonary-arterial-hypertension-100651323","NCT07761572","CS1 Added to Standard of Care Therapy for Pulmonary Arterial Hypertension","A Phase 2b, Double-Blind, Randomized, Placebo-Controlled, Dose-Finding Study, to Compare the Efficacy and Safety\u002FTolerability of CS1 Versus Placebo When Added to Standard of Care for the Treatment of Pulmonary Arterial Hypertension (PAH)","EPIMODE","Inclusion Criteria:\n\n* Age 18 to 80 years\n* WHO Group 1 pulmonary arterial hypertension\n* WHO Functional Class II or III\n* REVEAL Lite 2 score ≥6\n* Receiving stable standard-of-care PAH therapy for at least 90 days prior to screening\n* Hemodynamic confirmation of PAH by right heart catheterization\n* 6-minute walk distance ≥150 m and \\\u003C550 m\n\nExclusion Criteria:\n\n* Pulmonary hypertension WHO Group 2, 3, 4, or 5\n* Significant left-sided heart disease\n* Recent major cardiovascular or cerebrovascular event\n* Prior heart or heart-lung transplantation or active lung transplant listing\n* Significant renal or hepatic dysfunction\n* Current use of prohibited medications that cannot be discontinued\n* Pregnant or breastfeeding","80 Years",{"count":283,"type":22},126,[178],"This Phase 2 study (EPIMODE) will evaluate the efficacy and safety of CS1 compared with placebo when added to standard-of-care therapy in participants with pulmonary arterial hypertension.\n\nParticipants completing Treatment Period 1 (Week 36) will be re-randomized to continue or switch study treatment for Treatment Period 2 through Week 52.",[32],[288,32,289,290,291,292,293],"CS1","PAH","Sodium Valproate","Valproic Acid","Pulmonary Vascular Resistance","6-Minute Walk Distance","2026-08-06",{"date":296,"type":41},"2026-08-12",{"date":298,"type":22},"2026-09-15",{"date":300,"type":22},"2029-04-30",{"name":302,"class":70},"Cereno Scientific AB",{"id":304,"slug":305,"hasResults":12,"nctId":306,"briefTitle":307,"officialTitle":308,"acronym":309,"eligibilityCriteria":310,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":129,"enrollmentInfo":311,"targetDuration":4,"studyType":23,"phases":313,"briefSummary":314,"conditions":315,"keywords":317,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":294,"lastUpdatePostDateStruct":321,"startDateStruct":322,"completionDateStruct":324,"leadSponsor":326,"locationsCount":49},"100438605","xenon-mri-pulm-hypertension-100438605","NCT04991454","Xenon MRI Pulm Hypertension","Xenon MRI in Pulmonary Hypertension","Jupiter PH","Inclusion Criteria of Cohort 1\n\nSubjects must meet all of the following inclusion criteria to be eligible for enrollment into the trial:\n\n1. Outpatients of either gender, Age 18-75\n2. Awaiting a lung transplant\n3. Diagnosis of precapillary PH (right heart catheterization demonstrating hemodynamic criteria of a mean pulmonary artery pressure (mPAP) ≥ 25 mmHg, pulmonary vascular resistance ≥ 3 WU, pulmonary capillary wedge pressure ≤ 15 mmHg) in the setting of Group 1 (PAH), 3 (PH due to chronic lung disease, 4 (PH due to pulmonary artery obstructions), or 5 (PH due to miscellaneous causes)\n4. Willing and giving informed consent and adhere to visit\u002Fprotocol schedules (consent must be given before any study procedures are performed).\n5. Women of childbearing potential must have a negative urine pregnancy test before MRI\n\nExclusion Criteria of Cohort 1\n\nSubjects presenting with any of the following will not be included in the trials:\n\n1. Moderate to severe heart disease (LVEF \\\u003C45%, Severe LV hypertrophy, Moderate to severe valvular disease)\n2. PH due to schistosomiasis\n3. Active cancer\n4. Sickle cell anemia\n5. Prisoners and pregnant women will not be approached for the study\n6. Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to lie supine)\n7. Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the subject or interfere with the subject's ability to comply with the protocol requirements\n\nInclusion Criteria of Cohort 2\n\nSubjects must meet all of the following inclusion criteria to be eligible for enrollment into the trial:\n\n1. Treatment naïve or treatment started within the last 3 months\n2. Outpatients of either gender, Age 18-75\n3. WHO functional class (FC) 2-3 symptoms with a diagnosis of group 1 PH (mean pulmonary artery pressures (mPAP) \\> 20 mmHg, pulmonary capillary wedge pressure (PCWP) ≤ 15mmHg and pulmonary vascular resistance (PVR) ≥3 WU)\n4. Willing and able to give informed consent and adhere to visit\u002Fprotocol schedules (consent must be given before any study procedures are performed).\n5. Women of childbearing potential must have a negative urine pregnancy test before MRI\n\nExclusion Criteria of Cohort 2\n\nSubjects presenting with any of the following will not be included in the trials:\n\n1. Sarcoidosis\n2. Active cancer\n3. Sickle cell anemia\n4. Liver disease (Childs-Pugh class C)\n5. Any conditions that prevent the performance of 129Xe MRI scans.\n6. Prisoners and pregnant women will not be approached for the study.\n7. Conditions that will prohibit MRI scanning (metal in eye, claustrophobia, inability to lie supine).\n8. Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the subject or interfere with the subject's ability to comply with the protocol requirements",{"count":312,"type":22},20,[178],"The overall objective outlined in this study is to determine how pulmonary vascular remodeling in PAH at a cellular and pathological level is associated with changes in gas exchange physiology and hemodynamics (monitored with 129Xe MRI\u002FMRS) and how these signals change with disease progression or treatment.",[316,32],"Pulmonary Hypertension",[318,319,320],"Lung Transplant","Xenon","MRI",{"date":265,"type":41},{"date":323,"type":41},"2021-09-01",{"date":325,"type":22},"2027-07-31",{"name":327,"class":119},"Bastiaan Driehuys",{"id":329,"slug":330,"hasResults":12,"nctId":331,"briefTitle":332,"officialTitle":333,"acronym":4,"eligibilityCriteria":334,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":335,"targetDuration":4,"studyType":23,"phases":337,"briefSummary":339,"conditions":340,"keywords":4,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":341,"lastUpdatePostDateStruct":342,"startDateStruct":344,"completionDateStruct":346,"leadSponsor":348,"locationsCount":4},"100638004","phase-4-a-study-of-sotatercept-mk-7962-in-people-with-pulmonary-arterial-hypertension-pah-in-india-mk-7962-037-100638004","NCT07600723","A Study of Sotatercept (MK-7962) in People With Pulmonary Arterial Hypertension (PAH) in India (MK-7962-037)","A Phase 4, Prospective, Open-label, Single-Arm Study to Evaluate the Safety of Sotatercept in Adults With Pulmonary Arterial Hypertension (PAH) in India","Inclusion Criteria:\n\nThe main inclusion criteria include but are not limited to the following:\n\n* Has documented historical diagnostic right heart catheterization (RHC), with the diagnosis of pulmonary arterial hypertension (PAH), also known as Group 1 pulmonary hypertension (PH), in any of the following subtypes: Idiopathic PAH, Heritable PAH, Drug\u002Ftoxin-induced PAH, PAH associated with connective tissue disease, and PAH associated with simple, congenital systemic-to-pulmonary shunts at least 1 year following repair\n* Has been on stable doses of PAH background therapies and diuretics (if applicable)\n* Has symptomatic PAH classified as World Health Organization (WHO) Functional Classification (FC) II or III\n\nExclusion Criteria:\n\nThe main exclusion criteria include but are not limited to the following:\n\n* Has a diagnosis of Groups 2, 3, 4, or 5 PH\n* Has a diagnosis of the following PAH (Group 1 PH) subtypes: human immunodeficiency virus (HIV)-associated PAH, PAH associated with portal hypertension, schistosomiasis-associated PAH, PAH diagnosed with pulmonary veno occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH)\n* Has uncontrolled systemic hypertension\n* Has a history of full or partial pneumonectomy\n* Has untreated more than mild obstructive sleep apnea\n* Has known history of portal hypertension or chronic liver disease, including hepatitis B and\u002For hepatitis C\n* Has a history of restrictive, constrictive, or congestive cardiomyopathy\n* Has significant mitral regurgitation or aortic regurgitation valvular disease, mitral stenosis, and more than mild aortic valve stenosis\n* Has known malignancy that is progressing or has required active treatment within the past 5 years",{"count":336,"type":22},30,[338],"PHASE4","Researchers are looking for other ways to treat people in India with pulmonary arterial hypertension (PAH), also known as Group 1 pulmonary hypertension (PH). In PAH, the blood vessels in the lungs become thick and narrow, which makes it harder for blood to flow to the lungs. This causes high blood pressure in the lungs and can overwork the heart. PAH can make it hard to breathe and be active.\n\nResearchers want to learn if sotatercept, the study medicine, can be given with standard treatment to help treat PAH. The standard treatment (the usual treatment) for PAH includes one or multiple medicines. However, these may not fully work or treat the symptoms of PAH in some people.\n\nThe goal of this study is to learn about the safety and tolerability of sotatercept when it is given with standard treatment to people in India.",[32],"2026-08-05",{"date":343,"type":41},"2026-08-07",{"date":345,"type":22},"2026-11-04",{"date":347,"type":22},"2028-04-03",{"name":69,"class":70},{"id":350,"slug":351,"hasResults":12,"nctId":352,"briefTitle":353,"officialTitle":354,"acronym":355,"eligibilityCriteria":356,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":357,"targetDuration":4,"studyType":23,"phases":359,"briefSummary":360,"conditions":361,"keywords":363,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":368,"lastUpdatePostDateStruct":369,"startDateStruct":371,"completionDateStruct":373,"leadSponsor":375,"locationsCount":377},"100638083","phase-1-diag723-in-adults-with-hereditary-hemorrhagic-telangiectasia-100638083","NCT07623525","DIAG723 in Adults With Hereditary Hemorrhagic Telangiectasia","A Phase 1\u002F2, First-in-Human, Multicenter, Ascending Single-Dose and Multi-Dose Study to Assess the Safety of DIAG723, a Novel Bispecific ALK-1 and BMPRII Agonist Antibody in Adult Patients With Hereditary Hemorrhagic Telangiectasia (DIAMOND Trial)","DIAMOND","Inclusion Criteria:\n\n* Adult patients ≥18 years with a clinical or genetic diagnosis of HHT\n* Adequate hepatic and renal function\n* Part B: Epistaxis and anemia or transfusion\u002Firon history\n* Part C: HHT with documented pre-capillary pulmonary arterial hypertension\n\nExclusion Criteria:\n\n* Active or recent systemic infection\n* Recent thromboembolic events\n* Use of anti-angiogenic drugs within 6 weeks\n* Pregnancy or lactation\n* Recent participation in another investigational study",{"count":358,"type":22},93,[220,178],"This is a Phase 1\u002F2, randomized, double-blind, placebo-controlled, first-in-human study evaluating the safety, tolerability, pharmacokinetics, and preliminary efficacy of subcutaneously administered DIAG723 in adult patients with hereditary hemorrhagic telangiectasia (HHT).\n\nThe study consists of three parts:\n\nPart A (dose escalation): Single ascending subcutaneous doses of DIAG723 are evaluated in sequential cohorts to assess safety, tolerability, and pharmacokinetics.\n\nPart B (dose expansion): Multiple doses of DIAG723 administered over 13 weeks are evaluated in patients with HHT to assess safety and preliminary efficacy.\n\nPart C (dose expansion): Multiple doses of DIAG723 administered over 13 weeks are evaluated in patients with HHT and concomitant pulmonary arterial hypertension to assess safety and exploratory clinical effects in this population.\n\nParticipants will be randomized within each study part to receive DIAG723 or placebo. The study includes dose escalation in Part A and dose expansion in Parts B and C.",[362,32],"Hereditary Hemorrhagic Telangiectasia",[362,364,365,366,367],"HHT","BMPRII","ALK1","Bone morphogenetic protein receptor type II","2026-07-31",{"date":370,"type":41},"2026-08-03",{"date":372,"type":41},"2026-06-04",{"date":374,"type":22},"2027-12-31",{"name":376,"class":70},"Diagonal Therapeutics, Inc.",12,{"id":379,"slug":380,"hasResults":12,"nctId":381,"briefTitle":382,"officialTitle":382,"acronym":4,"eligibilityCriteria":383,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":129,"enrollmentInfo":384,"targetDuration":4,"studyType":23,"phases":385,"briefSummary":386,"conditions":387,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":388,"lastUpdatePostDateStruct":389,"startDateStruct":390,"completionDateStruct":392,"leadSponsor":394,"locationsCount":49},"100553112","phase-1-a-pilot-study-of-microbiota-transplant-with-antibiotic-preconditioning-and-fiber-supplementation-in-pulmonary-arterial-hypertension-gut-restore-pah-100553112","NCT06481852","A Pilot Study of Microbiota Transplant With Antibiotic Preconditioning and Fiber Supplementation in Pulmonary Arterial Hypertension (GUT RESTORE-PAH)","Inclusion Criteria:\n\n* Provision of signed and dated consent form\n* Ages 18-75\n* Diagnosis of PAH\n* On stable treatment for PAH for one month prior to enrollment\n* Able to swallow capsules\n* Able to provide blood sample and fecal sample\n* Stated willingness to comply with all study procedures and availability for the duration of trial to follow-up by telephone, in-person, email, and\u002For video visits or correspondence.\n\nExclusion Criteria:\n\n* Dysphagia to pills\n* Clinically active inflammatory bowel disease\n* Pregnancy or breastfeeding. A pregnancy test will be obtained from females of child- bearing potential at the screening visit or day 1 (prior to the receipt of MTT). Patients with a positive pregnancy test will be excluded. A negative result will be required for subjects who are females of child-bearing potential to receive MTT. Patients will be counseled to avoid pregnancy which is the standard of care for patients with PAH.\n* Life expectancy of \\\u003C 6 months\n* Presence of ileostomy or colostomy\n* Patients on immunosuppressants (calcineurin inhibitors, prednisone ≥ 20 mg\u002Fday, methotrexate, azathioprine, immunosuppressive biologics, JAK inhibitors).\n* Patients with neutropenia (an absolute neutrophil count \\\u003C 0.5 x 10 9 cells\u002FL) obtained on a complete blood count with differential at screening\n* History of solid organ or bone marrow transplant\n* Anticipated recurrent antibiotic use (patients with frequent urinary tract infections or sinusitis)\n* History of severe anaphylactic food allergy\n* History of celiac disease\n* Patients receiving cancer chemotherapy, immunotherapy, or radiation\n* Patients with severe Irritable bowel syndrome (IBS) defined by an IBS score \\>250",{"count":260,"type":22},[220],"This pilot clinical trial will evaluate the initial safety, feasibility, and pharmacokinetics of microbiota transplant therapy (MTT) with antibiotic pre-conditioning and fiber supplementation vs. placebo in patients with pulmonary arterial hypertension (PAH). This trial will inform development of future trials of MTT as a treatment for PAH. 24 PAH patients will be randomized to receive either MTT with antibiotic preconditioning + fiber supplementation, MTT with antibiotic preconditioning + placebo supplementation, or placebo + placebo supplementation. MTT will in a capsule form composed of freeze-dried, encapsulated intestinal microbiota from healthy donors. Fiber supplementation will be 10-14 gm oral fiber supplement. Patients will be followed at week 1, week 2, week 4, week 12, and week 24. Patient will undergo stool sample collection at baseline, week 1, week 4, and week 12, blood sample collection at baseline, week 4, and week,12. In addition, patient will undergo an echocardiogram, six-minute walk test (6MWT) and quality of life questionnaire at baseline and at week 12.",[32],"2026-07-29",{"date":368,"type":41},{"date":391,"type":41},"2024-10-28",{"date":393,"type":22},"2027-04-30",{"name":272,"class":119},{"id":396,"slug":397,"hasResults":12,"nctId":398,"briefTitle":399,"officialTitle":400,"acronym":4,"eligibilityCriteria":401,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":129,"enrollmentInfo":402,"targetDuration":4,"studyType":23,"phases":404,"briefSummary":405,"conditions":406,"keywords":407,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":412,"lastUpdatePostDateStruct":413,"startDateStruct":414,"completionDateStruct":416,"leadSponsor":417,"locationsCount":419},"100537205","phase-3-open-label-extension-study-of-seralutinib-in-adult-subjects-with-pah-prosera-ext-100537205","NCT06274801","Open-label Extension Study of Seralutinib in Adult Subjects With PAH (PROSERA-EXT)","An Open-label Extension Study Evaluating the Long-term Safety and Efficacy of Seralutinib Orally Inhaled for the Treatment of Pulmonary Arterial Hypertension (PAH)","Inclusion Criteria:\n\n1. Subjects must have completed a qualifying last visit in a prior seralutinib PAH study on investigational product (IP) and in accordance with the protocol.\n2. Evidence of an informed consent document, signed and dated by the subject, indicating that the subject has been informed of all pertinent aspects of the study prior to initiation of any subject-mandated procedures.\n3. Willingness and ability to comply with scheduled visits, treatment plan, laboratory tests, and other study procedures\n4. Women of childbearing potential (WOCBP) must have a negative urine or serum pregnancy test at enrollment visit before first administration of Investigational Product (IP) in this study.\n5. WOCBP who are not abstinent and intend to be sexually active with a non-sterilized male partner must be willing to use a highly effective method of contraception from consent through 30 days following the last administration of IP.\n6. Male subjects: Non-sterilized male subjects who are not abstinent and intend to be sexually active with a female partner of childbearing potential must use a male condom from consent through 90 days after the last dose of IP.\n\nExclusion Criteria:\n\n1. Severe acute or chronic medical or laboratory abnormality that may increase the risk associated with study participation or seralutinib administration, unless associated with an ongoing AE and discussed with the Sponsor's medical monitor (MM) (or designee).\n2. Have any other condition or reason that, in the opinion of the Investigator, would prohibit the subject from participating in the study.",{"count":403,"type":22},330,[25],"This open-label extension study will evaluate the long-term safety, tolerability and efficacy of orally inhaled seralutinib in subjects who have completed a previous seralutinib study",[32],[408,409,410,411],"seralutinib","GB002","PROSERA","PROSERA-EXT","2026-07-28",{"date":388,"type":41},{"date":415,"type":41},"2024-09-03",{"date":116,"type":22},{"name":418,"class":70},"Gossamer Bio USA, Inc.",120,{"id":421,"slug":422,"hasResults":12,"nctId":423,"briefTitle":424,"officialTitle":425,"acronym":4,"eligibilityCriteria":426,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":427,"enrollmentInfo":428,"targetDuration":4,"studyType":23,"phases":430,"briefSummary":431,"conditions":432,"keywords":4,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":433,"lastUpdatePostDateStruct":434,"startDateStruct":436,"completionDateStruct":438,"leadSponsor":440,"locationsCount":4},"100649660","phase-2-myocardial-metabolic-flux-in-pulmonary-arterial-hypertension-100649660","NCT07737522","Myocardial Metabolic Flux in Pulmonary Arterial Hypertension","Myocardial Metabolic Flux in Patients With Pulmonary Arterial Hypertension (PAH) in Response to GLP-1 Agonist Therapy: a Physiological Study Using 31-Phosphorus MR Spectroscopy","Inclusion Criteria:\n\n\\- 1. Diagnosis of Group 1 PAH confirmed by right heart catheterisation under the National Pulmonary Hypertension Service, Royal Brompton Hospital, part of GSTT Foundation Trust 2. Age over 18, less than 85 years 3. Able to give informed consent 4. On a stable dose of PAH-specific therapies (e.g., ERA, PDE5i) for at least 3 months.\n\n5\\. Clinically justified prescription of GLP-1 agonist Semaglutide based on following criteria: BMI \\> 30 or BMI \\> 27 with at least one cardiovascular co-morbidity (systemic hypertension, diabetes, pre-diabetes, COPD, atrial fibrillation, dyslipidaemia, sleep disordered breathing)\n\nExclusion Criteria:\n\n* • 1. Pregnancy\n\n  * 2\\. Myocardial infarction within the previous 3 months\n  * 3\\. Contraindications to MRI: Pacemakers, metallic implants, or severe claustrophobia.\n  * 4\\. Severe renal impairment: eGFR \\\u003C 15ml\u002Fmin\u002F1.73m.\n  * 5\\. Current use of SGLT2 inhibitors or GLP-1 agonist therapy (which significantly alter fuel substrate preference) or insulin therapy that cannot be held for the fasting scan","85 Years",{"count":429,"type":22},32,[178],"The rationale for this study is that GLP-1 agonist treatment is likely to influence myocardial substrate utilisation, changing the predominant source of metabolic energy within the heart to a more energetically efficient form. This is represented by a surrogate for improved mitochondrial efficiency with reduction in myocardial lactate levels (produced by inefficient myocardial glycolysis, prevalent in the ventricles of patients with pulmonary hypertension), which can be measured by 31P-magnetic resonance spectroscopy (31P-MRS).",[32],"2026-07-27",{"date":435,"type":41},"2026-07-30",{"date":437,"type":22},"2026-10-01",{"date":439,"type":22},"2029-04-01",{"name":441,"class":119},"Imperial College London",{"id":443,"slug":444,"hasResults":12,"nctId":445,"briefTitle":446,"officialTitle":447,"acronym":448,"eligibilityCriteria":449,"healthyVolunteers":80,"sex":17,"minAge":450,"maxAge":129,"enrollmentInfo":451,"targetDuration":4,"studyType":23,"phases":453,"briefSummary":454,"conditions":455,"keywords":4,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":459,"lastUpdatePostDateStruct":460,"startDateStruct":461,"completionDateStruct":462,"leadSponsor":464,"locationsCount":4},"100648010","extracellular-vesicle-dynamics-predicting-vascular-complications-and-treatment-response-in-systemic-sclerosis-100648010","NCT07717060","Extracellular Vesicle Dynamics Predicting Vascular Complications and Treatment Response in Systemic Sclerosis","Extracellular Vesicle Dynamics Across the Circulatory System as Predictors of Major Vascular Complications and Therapeutic Response in Systemic Sclerosis Patients","EVOLVE-SSc","Inclusion Criteria:\n\nMale and female patients aged 45-75 years Diagnosis of systemic sclerosis according to the 2013 ACR\u002FEULAR classification criteria High risk of pulmonary arterial hypertension based on the DETECT algorithm Stable treatment with vasoactive, vasodilator, and immunosuppressive therapies for at least 3 months prior to blood sampling\n\nExclusion Criteria:\n\nPrevious diagnosis of pulmonary arterial hypertension confirmed by right heart catheterization Interstitial lung involvement affecting more than 10% of the lung parenchyma Left-sided heart failure (NYHA class 3-4) Evidence of chronic thromboembolic pulmonary disease on contrast-enhanced CT scan Major contraindications to right heart catheterization or coronary angiography Inability to provide informed consent","45 Years",{"count":452,"type":22},60,[156],"Systemic sclerosis is a multisystem autoimmune disease characterized by vascular dysfunction, immune dysregulation, and progressive tissue fibrosis. Cardiopulmonary complications and peripheral vascular involvement are the principal causes of disability and mortality.\n\nExtracellular vesicles (EVs) have emerged as key mediators of paracrine intercellular communication. Preclinical studies further suggest that EVs mediate long-range inter-organ communication through the circulation. However, the inability to directly track EV trafficking in vivo in humans has limited the understanding of their contribution to systemic inter-organ communication.\n\nThe investigators propose that systemic sclerosis provides a unique human model for investigating circulating EV-mediated inter-organ communication in a multisystem disease. The central hypothesis is that arteriovenous differences in the molecular and cellular characteristics of circulating EVs reflect their dynamic exchange between individual organs and the bloodstream, and that these differences are associated with disease severity. Comparison of EVs across the circulation, rather than relying exclusively on peripheral blood samples, enables a more direct assessment of organ-specific EV release and uptake.\n\nCharacterizing EV dynamics along the circulatory pathway has the potential to identify novel biomarkers and therapeutic targets for systemic sclerosis while providing fundamental insights into EV-mediated inter-organ communication in humans.",[456,32,457,458],"Systemic Sclerosis","Digital Ulcers","Vascular Complications","2026-07-16",{"date":140,"type":41},{"date":268,"type":22},{"date":463,"type":22},"2028-09-01",{"name":465,"class":119},"Fondazione Policlinico Universitario Agostino Gemelli IRCCS",{"id":467,"slug":468,"hasResults":12,"nctId":469,"briefTitle":470,"officialTitle":471,"acronym":472,"eligibilityCriteria":473,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":474,"targetDuration":4,"studyType":23,"phases":475,"briefSummary":476,"conditions":477,"keywords":478,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":487,"lastUpdatePostDateStruct":488,"startDateStruct":490,"completionDateStruct":492,"leadSponsor":494,"locationsCount":49},"100645749","withdrawal-of-prostacyclin-pathway-therapy-in-patients-with-pulmonary-arterial-hypertension-receiving-sotatercept-waterloo-100645749","NCT07700303","Withdrawal of Prostacyclin Pathway Therapy in Patients With Pulmonary Arterial Hypertension Receiving Sotatercept (WATERLOO)","Withdrawal of Background Prostacyclin Pathway Therapy in Patients With Pulmonary Arterial Hypertension Receiving Sotatercept: an Open Label Non-inferiority Trial","WATERLOO","Inclusion Criteria: In order to be eligible for this study, a participant must meet all of the following criteria:\n\n1. Adults ≥ 18 years old diagnosed with PAH.\n2. Treatment with sotatercept for ≥6 months.\n3. Background therapy with ≥2 PAH vasodilator therapies, one of which is a parenteral prostacyclin or selexipag.\n4. At low or intermediate-low risk, defined using the 2022 ESC\u002FERS guidelines 4-strata risk assessment tool (Table 1).\n5. RHC at screening or historical within 8 weeks of screening, and after at least 6 months of sotatercept treatment, demonstrating a mPAP ≤40 mmHg and PVR ≤5 WU\n6. The ability to adhere to the study visit schedule and to comprehend and comply with all protocol requirements.\n7. Ability to provide informed consent.\n\nExclusion Criteria: A potential participant who meets any of the following criteria will be excluded from participation in this study:\n\n1. Known intolerance to sotatercept\n2. A RHC at screening or historical within 8 weeks of screening and after ≥6 months of sotatercept treatment demonstrating mPAP \\> 40 mmHg or PVR \\> 5 WU.\n3. Hospitalization for worsening PAH or right heart failure within the 3 months prior to screening.\n4. Active listing for lung or heart\u002Flung transplantation.\n5. Metastatic cancer or any other condition with a life expectancy \\\u003C 6 months,\n6. Female patients who are pregnant or who are of childbearing age and are unwilling to use contraception during the study.\n7. History of ≥ 3 interruptions or missed doses of sotatercept for any reason within the previous 6 months prior to screening.\n8. Patients who received any investigational medication within 1 month prior to screening (unless known to be placebo) or who are scheduled to receive another investigational drug during the course of this study.",{"count":240,"type":22},[156],"Pulmonary arterial hypertension (PAH) is a rare lung disease that leads to elevated blood pressure in the lungs and strain on the right side of the heart. For many years, treatments for PAH have included drugs that target the prostacyclin pathway using intravenous, subcutaneous, oral, and inhaled drugs. These drugs help widen the blood vessels in the lungs so the heart does not have to work as hard. However, these medicines can cause side effects such as jaw pain, flushing, diarrhea, and nausea, and the pump therapy can be very hard to manage day-to-day.\n\nA newer medicine called sotatercept works in a different way. It helps fix some of the root causes of PAH. Early reports suggest that some people do very well on sotatercept and may not need to keep taking their prostacyclin therapy. However, investigators do not yet know if it is safe to stop prostacyclin therapies or how to do so. This study, called WATERLOO, is designed to find out whether slowly stopping prostacyclin therapy while the participant is doing well on sotatercept is safe. Investigators will compare people who stop their prostacyclin therapy to people who keep taking it. This study is being done at PAH expert centres in Canada and Europe.",[32],[479,480,481,482,483,484,485,486],"Prostacyclin","sotatercept","open label","non-inferiority","Weatherald","Canadian VIGOUR Centre","CVC","pulmonary arterial hypertension","2026-07-13",{"date":489,"type":41},"2026-07-15",{"date":491,"type":22},"2026-09-30",{"date":493,"type":22},"2030-03-30",{"name":495,"class":119},"University of Alberta",{"id":497,"slug":498,"hasResults":12,"nctId":499,"briefTitle":500,"officialTitle":501,"acronym":502,"eligibilityCriteria":503,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":504,"targetDuration":4,"studyType":85,"phases":4,"briefSummary":506,"conditions":507,"keywords":510,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":517,"lastUpdatePostDateStruct":518,"startDateStruct":520,"completionDateStruct":522,"leadSponsor":524,"locationsCount":49},"100593954","the-impact-of-era-switching-on-risk-stratification-in-pulmonary-arterial-hypertension-100593954","NCT07013149","The Impact of ERA Switching on Risk Stratification in Pulmonary Arterial Hypertension","ACTION - The Impact of ERA Switching on Risk Stratification in Pulmonary Arterial Hypertension","ACTION","Inclusion Criteria:\n\n* Age ≥ 18 years\n* Confirmed diagnosis of pulmonary arterial hypertension (PAH) by right heart catheterization\n* Documented therapeutic switch from ambrisentan (10 mg once daily) to bosentan (125 mg twice daily) within the previous 6 months for the switch group\n* Treatment with ambrisentan for at least 6 months without switching to bosentan for the maintenance group\n\nExclusion Criteria:\n\n* History of severe hepatic impairment\n* Incomplete clinical or laboratory records that prevent risk score calculation\n* Inability to attend clinical follow-up between 3 and 6 months after medication switch",{"count":505,"type":22},183,"Pulmonary arterial hypertension (PAH) is a rare, progressive, and potentially life-threatening disease characterized by pulmonary vascular remodeling, increased pulmonary vascular resistance, and right ventricular dysfunction. The endothelin pathway plays a central role in its pathophysiology and is targeted by endothelin receptor antagonists (ERAs), including ambrisentan and bosentan.\n\nAmbrisentan is a selective ETA receptor antagonist, whereas bosentan blocks both ETA and ETB receptors. Although transitions between ERAs occur in clinical practice, evidence regarding the clinical impact of switching from ambrisentan to bosentan remains limited.\n\nACTION is a retrospective, observational, single-center cohort study evaluating adult patients with pulmonary arterial hypertension (World Health Organization Group 1) and\u002For chronic thromboembolic pulmonary hypertension (World Health Organization Group 4) confirmed by right heart catheterization. Patients who switched from ambrisentan to bosentan because of a national ambrisentan shortage will be compared with clinically similar patients who remained on ambrisentan.\n\nClinical, functional, and laboratory data recorded at baseline and at 3 to 6 months of follow-up will be assessed. The primary outcome is the proportion of patients with worsening risk stratification after switching from ambrisentan to bosentan compared with patients who continued ambrisentan. Risk will be evaluated using the COMPERA 2.0 and REVEAL Lite 2 assessment tools.\n\nSecondary outcomes include changes in World Health Organization\u002FNew York Heart Association functional class, 6-minute walk distance, BNP levels, individual risk-assessment components, hepatic enzymes, hemoglobin levels, and clinically relevant events such as hospitalization, emergency department visits, initiation of supplemental oxygen, and right heart failure decompensation.",[32,508,509],"Pulmonary Arterial Hypertension (PAH)","Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH)",[32,289,511,512,513,514,515,516],"Endothelin Receptor Antagonists","ERA","Ambrisentan","Bosentan","Drug Switching","Risk Stratification","2026-07-01",{"date":519,"type":41},"2026-07-06",{"date":521,"type":41},"2025-08-20",{"date":523,"type":22},"2026-12-01",{"name":525,"class":119},"University of Sao Paulo General Hospital",{"id":527,"slug":528,"hasResults":12,"nctId":529,"briefTitle":530,"officialTitle":531,"acronym":532,"eligibilityCriteria":533,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":534,"targetDuration":4,"studyType":85,"phases":4,"briefSummary":536,"conditions":537,"keywords":539,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":544,"lastUpdatePostDateStruct":545,"startDateStruct":547,"completionDateStruct":549,"leadSponsor":551,"locationsCount":49},"100643944","multimodality-rv-phenotyping-for-risk-stratification-and-short-term-outcomes-in-group-1-pah-100643944","NCT07667673","Multimodality RV Phenotyping for Risk Stratification and Short-Term Outcomes in Group 1 PAH","Multimodality Imaging-Based Right Ventricular Phenotyping for Risk Stratification and Short-Term Outcomes in Group 1 Pulmonary Arterial Hypertension: The MIRROR-PAH Study","MIRROR-PAH","Inclusion Criteria:\n\n* Adults aged 18 years or older\n* Diagnosis of Group 1 pulmonary arterial hypertension (PAH) according to ESC\u002FERS guidelines\n* Followed at the study center with a diagnosis of PAH\n* Availability of right heart catheterization (RHC) and cardiac magnetic resonance (CMR) imaging data obtained within a clinically relevant time interval\n* Availability of analyzable clinical, imaging, and hemodynamic data\n* Willingness to participate in the study and provision of written informed consent\n\nExclusion Criteria:\n\n* Age younger than 18 years\n* Pulmonary hypertension groups other than Group 1 PAH (Groups 2-5 PH)\n* Absence of either right heart catheterization or cardiac magnetic resonance imaging data within a clinically relevant time interval\n* Incomplete clinical or imaging data preventing analysis\n* Unavailable follow-up data",{"count":535,"type":22},50,"The MIRROR-PAH is a single-center, prospective, observational cohort study evaluating the incremental value of multimodality imaging-derived right ventricular characteristics for risk stratification in patients with Group 1 pulmonary arterial hypertension (PAH). The study aims to determine whether incorporation of echocardiographic and cardiac magnetic resonance (CMR)-derived right ventricular parameters into established non-invasive risk assessment models results in risk reclassification and improves identification of patients at risk for short-term clinical worsening.\n\nAdult patients with established Group 1 PAH undergoing routine follow-up and with available right heart catheterization (RHC) and CMR data will be consecutively enrolled. Clinical, laboratory, echocardiographic, and follow-up data will be prospectively collected over a 6-month period. Associations between multimodality imaging findings, invasive hemodynamic measurements, risk classification, and short-term clinical outcomes will be evaluated.",[316,32,538],"Right Ventricular Function",[486,540,541,542,543],"transthoracic echocardiography","cardiac magnetic resonance imaging","right heart catheterization","risk stratification","2026-06-19",{"date":546,"type":41},"2026-06-25",{"date":548,"type":41},"2026-05-11",{"date":550,"type":22},"2027-05",{"name":552,"class":119},"Istanbul University - Cerrahpasa",{"id":554,"slug":555,"hasResults":12,"nctId":556,"briefTitle":557,"officialTitle":557,"acronym":4,"eligibilityCriteria":558,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":559,"targetDuration":4,"studyType":23,"phases":560,"briefSummary":561,"conditions":562,"keywords":563,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":566,"lastUpdatePostDateStruct":567,"startDateStruct":569,"completionDateStruct":570,"leadSponsor":572,"locationsCount":4},"100529618","phase-2-clinical-trial-of-2-hoba-in-pulmonary-arterial-hypertension-100529618","NCT06176118","Clinical Trial of 2-HOBA in Pulmonary Arterial Hypertension","Inclusion Criteria:\n\n* Adults aged 18 or older\n* Diagnosed with idiopathic, heritable, simple congenital heart defect, or drug- or toxin-associated pulmonary arterial hypertension (PAH) according to World Health Organization consensus recommendations.\n* Stable PAH-specific medication regimen for three months prior to enrollment. Subjects with only a single diuretic adjustment in the prior three months will be included. Adjustments in IV prostacyclin for side effect management are allowed.\n* FEV1\\> or = 60% predicted and no more than mild abnormalities on lung imaging\n* WHO Functional Class II-IV\n* Ambulatory\n\nExclusion Criteria:\n\n* Sensitivity to 2-HOBA\n* Sensitivity to aspirin or salicylates\n* Aspirin-related rhinitis or wheezing\n* Prohibited from normal activity due to wheelchair bound status, bed bound status, reliance on a cane\u002Fwalker, activity-limiting angina, activity-limiting osteoarthritis, or other condition that limits activity\n* Pregnancy\n* Diagnosis of PAH etiology other than idiopathic, heritable, simple congenital heart defect, or associated with drugs or toxins\n* Drug and toxin associated PAH patients with active drug use\n* Prior diagnosis of cirrhosis\n* Malignancy\n* eGFR by MDRD \\\u003C60mL\u002Fmin\n* Non-english speaking",{"count":377,"type":22},[178],"Based on existing literature and clinical trials, 2- hydroxbenzylamine (2-HOBA) has clear impact on mechanisms that much of the international field of pulmonary hypertension (PH) research agrees are central to disease progression. The investigator's preliminary data and Phase I studies demonstrate not only a clear positive impact on reducing pulmonary vascular resistances in Group I and II PH, and both cytokine and molecular biomarkers of disease, but also indicated the potential for a substantial positive effect on heart function under load stress. In this Phase II project, investigators will test the safety and efficacy of 2-HOBA in PH patients, improving the function of the right ventricle under stress in a large animal model, and effectiveness in the context of standard-of-care in mouse models and large animals, to establish the remaining data needed to proceed to commercialization.",[32],[564,316,565],"2-hoba","Right Ventricular","2026-06-15",{"date":568,"type":41},"2026-06-17",{"date":268,"type":22},{"date":571,"type":22},"2027-12",{"name":165,"class":119},{"id":574,"slug":575,"hasResults":12,"nctId":576,"briefTitle":577,"officialTitle":578,"acronym":4,"eligibilityCriteria":579,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":4,"targetDuration":4,"studyType":580,"phases":4,"briefSummary":581,"conditions":582,"keywords":4,"overallStatus":584,"whyStopped":4,"lastUpdateSubmitDate":585,"lastUpdatePostDateStruct":586,"startDateStruct":4,"completionDateStruct":4,"leadSponsor":588,"locationsCount":4},"100588303","an-expanded-access-study-to-assess-treprostinil-palmitil-inhalation-powder-tpip-for-participants-with-pulmonary-arterial-hypertension-pah-and-pulmonary-hypertension-associated-with-interstitial-lung-disease-ph-ild-100588303","NCT06939647","An Expanded Access Study to Assess Treprostinil Palmitil Inhalation Powder (TPIP) for Participants With Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)","Treprostinil Palmitil Inhalation Powder (TPIP) in Patients With Pulmonary Arterial Hypertension or Pulmonary Hypertension Associated With Interstitial Lung Disease","Inclusion Criteria\n\n* The participant is ineligible for or cannot be treated satisfactorily with alternative commercially available therapy for PAH or PH-ILD.\n* Participant provides their informed consent to participate as per local requirements.\n* Participant must have successfully completed the OLE INS1009-203 or INS1009-212 studies.\n* Based on the treating physician's judgement on participant's medical history and an evaluation of the overall risk-benefit profile, the participant will be determined to be suitable for continued TPIP treatment within this program.\n* Requests for the post-OLE INS1009-203 and INS1009-212 TPIP studies must originate from the investigators of INS1009-203 and INS1009-212 TPIP studies, respectively.\n* Female participants must be postmenopausal (defined as no menses for 12 months without an alternative medical cause), surgically sterile, or using highly effective contraception (failure rate \\\u003C1% per year when used consistently and correctly) from Day 1 to at least 90 days after the last dose.\n* Male participants with female partners must adhere to contraception requirements to avoid potential exposure to the embryo\u002Ffetus based on the partner's reproductive status. For partners of childbearing potential, effective contraception must be used from Day 1 to at least 90 days after the last dose.\n\nExclusion Criteria:\n\n• No specific exclusion criteria.","EXPANDED_ACCESS","The purpose of this study is to provide continued access to TPIP for participants who have successfully completed the open-label extension (OLE) studies of INS1009-203 for PAH or INS1009-212 for PH-ILD.",[32,583],"Pulmonary Hypertension, Interstitial Lung Disease","AVAILABLE","2026-06-09",{"date":587,"type":41},"2026-06-10",{"name":144,"class":70},{"id":590,"slug":591,"hasResults":12,"nctId":592,"briefTitle":593,"officialTitle":594,"acronym":595,"eligibilityCriteria":596,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":129,"enrollmentInfo":597,"targetDuration":4,"studyType":23,"phases":599,"briefSummary":600,"conditions":601,"keywords":604,"overallStatus":264,"whyStopped":4,"lastUpdateSubmitDate":605,"lastUpdatePostDateStruct":606,"startDateStruct":608,"completionDateStruct":609,"leadSponsor":611,"locationsCount":4},"100639334","phase-3-a-phase-3-study-of-extended-release-tacrolimus-in-subjects-with-pulmonary-arterial-hypertension-and-functional-limitations-100639334","NCT07612657","A Phase 3 Study of Extended-release Tacrolimus in Subjects With Pulmonary Arterial Hypertension and Functional Limitations","A Randomized, Double-blind, Placebo-controlled, Safety and Efficacy Study of VI-0106 (Extended-release Tacrolimus) in Subjects With PAH and Functional Limitations Despite Optimized Treatment With Available PAH Medications","TRANSCEND","Inclusion Criteria:\n\n* WHO Group 1 PH: Pulmonary Arterial Hypertension;\n* WHO functional class II - IV despite optimized treatment with one or more modalities. Treatments for PAH must be stable for at least 3 months at the time of screening;\n* Right heart catheterization (RHC) at screening (or within 3 months prior to screening);\n* Screening 6MWD \\>75 meters to ≤450 meters.\n\nExclusion Criteria:\n\n* PAH due to pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis\n* Chronic thromboembolic or portopulmonary hypertension\n* Total Lung Capacity (TLC) \\\u003C60% predicted;\n* FEV1\u002FFVC \\\u003C70% predicted or FEV1 \\\u003C60% predicted;\n* Evidence of left-sided heart disease;\n* Inability to safely attempt completion of the 6MWD;\n* Life expectancy \\\u003C6 months;\n* eGFR \\\u003C30 mL\u002Fmin\u002F1.73 m2 (CKD-EPI equation);\n* Moderate to severe hepatic dysfunction (Child-Pugh score \\>10);\n* Serum potassium \\>5.1 mEq\u002FL;\n* Use of experimental PAH treatments within the past 3 months;\n* Active infection requiring antibiotic, antifungal, or antiviral therapies;\n* Current systemic treatment with cyclosporine;\n* Known allergy or hypersensitivity to tacrolimus;\n* Significant psychiatric, addictive, or other disorder that compromises the subject's ability to provide informed consent, follow study protocol, or adhere to study treatment",{"count":598,"type":22},300,[25],"This study evaluates the effects of VI-0106 (an extended-release formulation of tacrolimus) in participants with pulmonary arterial hypertension (PAH) who continue to have functional limitations despite being on optimized background PAH therapy. Participants will be randomly assigned with equal chance to receive either VI-0106 or placebo in a double-blind fashion to assess whether VI-0106 improves outcomes in this population.",[32,508,509,602,603],"Pulmonary Arterial Hypertension PAH","Pulmonary Arterial Hypertension WHO Group I",[289,32],"2026-05-22",{"date":607,"type":41},"2026-05-29",{"date":607,"type":22},{"date":610,"type":22},"2029-11-30",{"name":612,"class":70},"VIVUS LLC",{"id":614,"slug":615,"hasResults":12,"nctId":616,"briefTitle":617,"officialTitle":617,"acronym":4,"eligibilityCriteria":618,"healthyVolunteers":80,"sex":619,"minAge":18,"maxAge":450,"enrollmentInfo":620,"targetDuration":4,"studyType":85,"phases":4,"briefSummary":622,"conditions":623,"keywords":4,"overallStatus":37,"whyStopped":4,"lastUpdateSubmitDate":626,"lastUpdatePostDateStruct":627,"startDateStruct":629,"completionDateStruct":631,"leadSponsor":633,"locationsCount":231},"100616946","a-prospective-longitudinal-observational-cohort-study-of-pregnant-women-residing-at-high-altitude-in-bolivia-100616946","NCT07312227","A Prospective Longitudinal Observational Cohort Study of Pregnant Women Residing at High Altitude in Bolivia","Inclusion Criteria:\n\n* women delivering at participating hospitals at more than 3500 meters or women delivering at participating hospitals at sea level\n* signed informed consent\n\nExclusion Criteria:\n\n* preexisting cardiopulmonary pathologies (CHD, COPD, CKD, NYHA \\> III)","FEMALE",{"count":621,"type":22},27,"In this study, the investigators will follow two small cohorts of pregnant women: a cohort of healthy women with uncomplicated pregnancies residing at high altitude, a control group of healthy women with uncomplicated pregnancies residing at sea level, to characterize differences in cardiopulmonary adaptation and nitric oxide (NO) pathway expression at elevations \\>3,500 m throughout pregnancy and into the postpartum period. The investigators aim to investigate right-sided cardiac impairment induced by chronic hypobaric hypoxemia, its effects on fetal growth, and the potential contribution of cardiovascular nitric oxide depletion to obstetric complications.",[624,32,625],"Hypertensive Disorder of Pregnancy","Intrauterine Growth Restriction","2026-05-21",{"date":628,"type":41},"2026-05-26",{"date":630,"type":41},"2026-02-11",{"date":632,"type":22},"2027-02",{"name":634,"class":119},"Massachusetts General Hospital"]