[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"pulmonary-arterial-hypertension-pah\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:pulmonary-arterial-hypertension-pah":27},{"pageToken":4,"total":5,"offset":6,"count":7,"results":8},null,36,0,25,[9,55,82,114,142,169,192,223,248,273,293,324,343,366,387,418,441,468,491,513,537,564,588,613,639],{"id":10,"slug":11,"hasResults":12,"nctId":13,"briefTitle":14,"officialTitle":15,"acronym":4,"eligibilityCriteria":16,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":23,"briefSummary":25,"conditions":26,"keywords":30,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":43,"lastUpdatePostDateStruct":44,"startDateStruct":47,"completionDateStruct":49,"leadSponsor":51,"locationsCount":54},"100643856","vasopressor-agents-in-pulmonary-hypertension-crisis-100643856",false,"NCT07667829","Vasopressor Agents in Pulmonary Hypertension Crisis","Comparison of the Efficacy and Safety of Dopamine, Norepinephrine and Epinephrine in the Treatment of Pulmonary Hypertensive Crisis Under Hemodynamic Monitoring","Inclusion Criteria:\n\n1. Age \\> 18 years old;\n2. Signed informed consent form;\n3. Confirmed diagnosis of arterial pulmonary hypertension (PAH) and\u002For chronic thromboembolic pulmonary hypertension (CTEPH);\n4. Presenting with manifestations of pulmonary hypertensive crisis;\n5. Receiving both diagnosis and treatment in-hospital;\n6. No intravenous administration of the vasopressor drugs (including dopamine, norepinephrine, and epinephrine) under study at enrollment.\n\nExclusion Criteria:\n\n1. At SCAI stage D or stage E ;\n2. Patients who only receive a diagnosis but no treatment in the hospital;\n3. Uncontrolled hyperthyroidism;\n4. Complicated with angle-closure glaucoma;\n5. Hypersensitivity to the study drug;\n6. Ongoing use of halogenated hydrocarbon general anesthetics such as cyclopropane and halothane;\n7. Ongoing use of monoamine oxidase inhibitor (MAOI) antidepressants or anti-Parkinson drugs (phenelzine, tranylcypromine, isocarboxazid, moclobemide);\n8. Pregnancy;\n9. Already receiving the vasopressor drugs (dopamine, norepinephrine, epinephrine) under study at enrollment.","ALL","18 Years",{"count":20,"type":21},75,"ESTIMATED","INTERVENTIONAL",[24],"NA","Comparison of the efficacy and safety of three vasoactive agents-dopamine, norepinephrine, and epinephrine-in the treatment of patients with pulmonary hypertension crisis: prospective, randomised controlled trial monitored by haemodynamic monitoring",[27,28,29],"Pulmonary Arterial Hypertension (PAH)","Chronic Thromboembolic Pulmonary Hypertension (CTEPH)","Hypertension, Pulmonary",[29,31,32,33,34,35,36,37,38,39,40,41],"Cardiovascular Diseases","Vascular Diseases","Dopamine","Norepinephrine","Epinephrine","Haemodynamic Monitoring","Efficacy and Safety","Chronic thromboembolic pulmonary hypertension","Heart Failure","Pulmonary circulation","Pulmonary Arterial Hypertension","NOT_YET_RECRUITING","2026-06-21",{"date":45,"type":46},"2026-06-25","ACTUAL",{"date":48,"type":21},"2026-06",{"date":50,"type":21},"2029-01-31",{"name":52,"class":53},"China National Center for Cardiovascular Diseases","OTHER_GOV",3,{"id":56,"slug":57,"hasResults":12,"nctId":58,"briefTitle":59,"officialTitle":60,"acronym":61,"eligibilityCriteria":62,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":63,"targetDuration":4,"studyType":22,"phases":65,"briefSummary":66,"conditions":67,"keywords":4,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":72,"lastUpdatePostDateStruct":73,"startDateStruct":75,"completionDateStruct":77,"leadSponsor":79,"locationsCount":81},"100644283","tele-pulmonary-rehabilitation-for-patients-with-chronic-lung-diseases-100644283","NCT07666841","Tele-Pulmonary Rehabilitation For Patients With Chronic Lung Diseases","Tele-Pulmonary Rehabilitation to Improve Adherence and Management of Patients With Chronic Lung Diseases","TELE-PRIME","Inclusion Criteria:\n\n1. Patients meeting the Israeli Health Basket criteria for reimbursement of pulmonary rehabilitation (PR), including one of the following conditions:\n\n   * COPD with an FEV1 below 50% or with severe exacerbation (hospitalization) in the previous year or two moderate exacerbations in the previous year.\n   * Interstitial lung disease with an FVC below 80% or a DLCO below 60%.\n   * Bronchiectasis with at least two exacerbations in the past year or at least one hospitalization, and an FEV1 below 80%.\n   * Pulmonary arterial hypertension.\n2. Willingness to initiate a PR or Tele-PR (TPR) programs.\n3. Ability to perform the TPR program or traditional PR activities as assessed by a member of the study team prior to enrollment.\n4. Agree to participate, with signed informed consent.\n\nExclusion Criteria:\n\n1. Uncontrolled comorbidity (e.g uncontrolled congestive heart failure)\n2. Inability to operate the mobile application.\n3. Without a mobile device or computer.\n4. A hospitalization in the 2-months prior to randomization.\n5. Completed PR program in the last 12 months.\n6. Inability to complete 10 repetitions in the 1-minute sit-to-stand test or 5-second single-leg stance\n7. Pregnancy\n8. Inability to provide informed consent due to impaired decision-making capacity, as determined by the study's capacity assessment.",{"count":64,"type":21},90,[24],"Pulmonary rehabilitation is a key treatment for lung diseases, but many patients struggle to attend sessions due to travel distances, physical limitations, or logistical barriers. The purpose of this study is to evaluate whether a tele-pulmonary rehabilitation (TPR) program can improve the rates of treatment initiation and adherence among patients with chronic lung diseases compared to traditional, center-based pulmonary rehabilitation. Additionally, the study will assess improvements in quality of life, physical symptoms, and safety in both groups.\n\nThis study will enroll 90 patients from two medical centers in Israel (Tel Aviv and Barzilai). Participants will be randomly assigned to one of two groups:\n\n1. The control group will receive usual care with a referral by a pulmonologist to standard pulmonary rehabilitation at a medical center.\n2. The intervention group will participate in a supervised remote tele-rehabilitation program using a dedicated application and remote monitoring.",[68,69,70,27,71],"COPD (Chronic Obstructive Pulmonary Disease)","Rehabilitation","Bronchiectasis Adult","Interstitial Lung Disease (ILD)","2026-06-18",{"date":74,"type":46},"2026-06-24",{"date":76,"type":21},"2026-07-01",{"date":78,"type":21},"2028-12-31",{"name":80,"class":53},"Tel-Aviv Sourasky Medical Center",2,{"id":83,"slug":84,"hasResults":12,"nctId":85,"briefTitle":86,"officialTitle":87,"acronym":88,"eligibilityCriteria":89,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":90,"targetDuration":4,"studyType":22,"phases":92,"briefSummary":94,"conditions":95,"keywords":96,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":103,"lastUpdatePostDateStruct":104,"startDateStruct":106,"completionDateStruct":108,"leadSponsor":110,"locationsCount":113},"100617436","phase-2-study-of-regn13335-in-adult-participants-with-pulmonary-arterial-hypertension-pah-100617436","NCT07318597","Study of REGN13335 in Adult Participants With Pulmonary Arterial Hypertension (PAH)","A Phase 2, Randomized, Double-Blind, Placebo-Controlled Study to Assess the Efficacy, Safety, Pharmacokinetics, and Pharmacodynamic Effects of REGN13335, an Anti-PDGF-B Monoclonal Antibody, in Adults With Pulmonary Arterial Hypertension","ILLUMINATE","Key Inclusion Criteria:\n\n1. Documented clinical diagnosis of PAH (Group 1 PH according to the 7th World Symposium on Pulmonary Hypertension (WSPH))\n2. WHO functional class II or III (slight to marked limitation of functional status due to PAH)\n3. Receiving background Standard Of Care (SOC) therapy for PAH on a stable dose and regimen, as determined by the investigator, as described in the protocol\n4. PVR ≥400 dynes∙sec\u002Fcm\\^5 (5 Wood units) based on Right Heart Catheterization (RHC) during the screening period\n5. Has 6MWD ≥150 and ≤550 meters repeated twice during screening as described in the protocol\n\nKey Exclusion Criteria:\n\n1. Has Group 2 (PH associated with left heart disease), Group 3 (PH associated with lung diseases and\u002For hypoxia), Group 4 (PH associated with pulmonary artery obstructions), or Group 5 (PH with unclear and\u002For multifactorial mechanisms) PH according to the 7th WSPH\n2. Pulmonary Arterial Wedge Pressure (PAWP) \\>15 mm Hg by RHC during the screening period\n3. History of left-sided heart disease and\u002For clinically significant cardiac disease, as described in the protocol\n4. Obstructive lung disease defined as Forced Expiratory Volume in 1 second (FEV1)\u002FForced Vital Capacity \\\u003C0.7 and FEV1 \\\u003C70% of the predicted value as described in the protocol\n5. Evidence of interstitial lung disease as defined in the protocol\n6. Evidence of chronic thromboembolic pulmonary disease or acute pulmonary embolism as described in the protocol\n7. Participants requiring anticoagulation and\u002For antiplatelet therapy for an underlying medical condition as described in the protocol\n8. Has any history of intracranial bleeding or any history of elevated intracranial pressure\n9. Has any history of bleeding meeting criteria as described in the protocol\n\nNote: Other protocol-defined Inclusion\u002F Exclusion criteria apply",{"count":91,"type":21},99,[93],"PHASE2","This study is researching an experimental drug called REGN13335. The study is focused on participants with Pulmonary Arterial Hypertension (PAH). The aim of the study is to see how safe and effective REGN13335 is in participants with PAH who are taking other PAH medicines.\n\nThe study is looking at several other research questions, including:\n\n* What side effects may happen from taking REGN13335\n* How much REGN13335 is in the blood at different times\n* Whether the body makes antibodies against REGN13335 (which could make REGN13335 less effective or could lead to side effects)",[27],[27,97,98,99,100,101],"Pulmonary Hypertension (PH)","Elevated Pulmonary Vascular Resistance (PVR)","World Health Organization (WHO) functional class II or III","Platelet-Derived Growth Factor-B (PDGF-B)","REGN13335","RECRUITING","2026-06-17",{"date":105,"type":46},"2026-06-22",{"date":107,"type":46},"2026-05-06",{"date":109,"type":21},"2028-09-17",{"name":111,"class":112},"Regeneron Pharmaceuticals","INDUSTRY",11,{"id":115,"slug":116,"hasResults":12,"nctId":117,"briefTitle":118,"officialTitle":119,"acronym":120,"eligibilityCriteria":121,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":122,"targetDuration":4,"studyType":22,"phases":124,"briefSummary":126,"conditions":127,"keywords":128,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":131,"lastUpdatePostDateStruct":132,"startDateStruct":134,"completionDateStruct":136,"leadSponsor":138,"locationsCount":141},"100620465","phase-1-impact-of-sotatercept-on-pulmonary-artery-and-right-ventricle-remodeling-imaging-assessed-with-68ga-fapi-petct-in-patients-with-pah-100620465","NCT07357974","Impact of Sotatercept on Pulmonary Artery and Right Ventricle Remodeling Imaging Assessed With 68Ga-FAPI PET\u002FCT in Patients With PAH","Impact of Sotatercept on Pulmonary Artery and Right Ventricle Remodeling Imaging Assessed With 68Ga-FAPI PET\u002FCT in Patients With PAH: a Pilot Study","SOFAPI","Inclusion Criteria:\n\n* Age ≥ 18 years\n* Documented diagnostic right heart catheterization (RHC) within 12 months of screening documenting a minimum PVR of ≥ 4 Wood units and pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) of ≤ 15 mmHg, with the diagnosis of WHO PAH Group 1 in any of the following subtypes:\n\n  * Idiopathic PAH\n  * Heritable PAH\n  * Drug\u002Ftoxine-induced PAH\n  * PAH associated with connective tissue disease\n  * PAH associated with simple, congenital systemic-to- pulmonary shunts at least 1year following repair\n* Patients under bi or tri-background-therapy\n* Symptomatic PAH classified WHO FC II or III\n* Patients will be started on Sotatercept\n* Ability to adhere to study visit schedule and understand and comply with all the protocol requirement.\n* Ability to understand and provide written informed consent\n\nExclusion Criteria:\n\n* Diagnosis of PH WHO Groups 2, 3, 4, or 5\n* Diagnosis of the following PAH Group 1 subtypes: human immunodeficiency virus (HIV)-associated PAH, PAH associated with portal hypertension, schistosomiasis associated PAH, pulmonary veno occlusive disease and pulmonary capillary hemangiomatosis.\n* Hemoglobin at screening above gender-specific ULN, per local laboratory test\n* Pregnant or breastfeeding women\n* Any of the following clinical laboratory values at the Screening visit:\n\n  * eGFR \\\u003C 30 mL\u002Fmin\u002F1.73 m2 (as defined by MDRD equation)\n  * Serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), or total bilirubin levels \\> 3 × ULN\n  * Platelet count \\\u003C 50,000\u002Fmm3 (\\\u003C 50.0 × 109\u002FL)\n* Known allergic reaction to sotatercept (ACE-011), its excipients, or luspatercept",{"count":123,"type":21},15,[125,93],"PHASE1","Pulmonary arterial hypertension (PAH) is a rare, progressive disease characterized by structural changes in the pulmonary arteries, leading to increased pulmonary vascular resistance and elevated pulmonary arterial pressure and, if untreated, right heart failure. Diagnosis requires a comprehensive evaluation, including right heart catheterization performed in specialized centers.\n\nDespite advances in the understanding and management of the disease, PAH remains a severe condition. Current approved therapies primarily target three key pathways involved in endothelial dysfunction: the endothelin, nitric oxide, and prostacyclin pathways. Pulmonary arterial remodeling is characterized by alterations in endothelial cells, smooth muscle cells, and fibroblasts, with fibroblast activation and macrophage involvement contributing to disease progression.\n\nTwo positron emission tomography\u002Fcomputed tomography (PET\u002FCT) imaging approaches are currently under investigation in PAH. \\[⁶⁸Ga\\]Ga-FAPI PET\u002FCT targets activated fibroblasts and enables noninvasive assessment of fibroblast activity and tissue remodeling. \\[⁶⁸Ga\\]Ga-MAA lung perfusion PET\u002FCT is an emerging imaging technique that provides higher spatial resolution and sensitivity than conventional lung perfusion imaging and allows evaluation of regional pulmonary perfusion.\n\nSotatercept is a novel fusion protein that modulates signaling within the transforming growth factor-beta (TGF-β) superfamily by binding select ligands involved in vascular remodeling. Its mechanism of action is distinct from that of currently approved PAH therapies. Sotatercept has been evaluated in clinical development programs, including the PULSAR and STELLAR studies. Reported adverse events include epistaxis, dizziness, increased hemoglobin levels, and changes in blood pressure.\n\nThis study is designed with the following objectives:\n\nPrimary objective: To assess pulmonary vascular remodeling in patients with PAH using \\[⁶⁸Ga\\]Ga-FAPI PET\u002FCT imaging.\n\nSecondary objectives: To evaluate \\[⁶⁸Ga\\]Ga-FAPI uptake and regional lung perfusion using \\[⁶⁸Ga\\]Ga-MAA lung perfusion PET\u002FCT imaging at predefined study time points.",[27],[129,130],"[68Ga]Ga-FAPI PET\u002FCT (FAPI)","[68Ga]Ga-MAA lung perfusion PET\u002FCT","2026-06-15",{"date":133,"type":46},"2026-06-16",{"date":135,"type":21},"2026-07-15",{"date":137,"type":21},"2028-01-01",{"name":139,"class":140},"University Hospital, Brest","OTHER",1,{"id":143,"slug":144,"hasResults":12,"nctId":145,"briefTitle":146,"officialTitle":147,"acronym":4,"eligibilityCriteria":148,"healthyVolunteers":149,"sex":17,"minAge":18,"maxAge":150,"enrollmentInfo":151,"targetDuration":4,"studyType":22,"phases":153,"briefSummary":154,"conditions":155,"keywords":157,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":159,"lastUpdatePostDateStruct":160,"startDateStruct":162,"completionDateStruct":164,"leadSponsor":166,"locationsCount":141},"100513578","phase-1-study-of-intravenous-zma001-in-healthy-subjects-100513578","NCT05967299","Study of Intravenous ZMA001 in Healthy Subjects","A Phase 1 Single-Ascending Dose Study to Evaluate the Safety, Tolerability and Pharmacokinetics of Intravenous ZMA001 in Healthy Subjects","* INCLUSION CRITERIA:\n\nIn order to be eligible to participate in this study, an individual must meet all of the following criteria:\n\n1. Stated willingness to comply with all study procedures and availability for the duration of the study\n2. Male\\* or female, aged 18 to 60 years, inclusive\n3. In good general health as evidenced by medical history\n4. Females of childbearing potential agree to use an accepted method of contraception (see below) throughout study participation and for 120 days after study drug infusion.\n5. Males sexually active with a female partner must agree to use a condom with spermicide for 120 days after study drug infusion or be surgically sterile for at least 90 days before screening. Males must also agree to not donate sperm for 120 days after study drug administration.\n6. Agreement to adhere to Lifestyle Considerations throughout study duration\n7. Ability of subject to understand and the willingness to sign a written informed consent document.\n\n   * Enrollment of healthy male subjects will be limited to no more than 14 out of the total study cohort of 32 in order to ensure an adequate representation of female subjects.\n\nAccepted methods of contraception for females of childbearing potential:\n\n* Use of an implanted or intrauterine hormonal device for at least 30 consecutive days before study drug infusion\n* Use of oral, patch or injectable contraceptives or a vaginal hormonal device for at least 30 consecutive days before study drug infusion\n* Use of a non-hormonal intrauterine device for at least 30 consecutive days before study drug infusion\n* Two barrier methods such as a diaphragm with spermicide or a condom with spermicide\n\nEXCLUSION CRITERIA:\n\nAn individual who meets any of the following criteria prior to informed consent will be excluded from participation in this study:\n\n1. Pregnancy or lactation. Females of childbearing potential must have a negative serum Beta-human chorionic gonadotropin test no more than 48 hours from study drug infusion.\n2. A history of human immunodeficiency virus (HIV) infection.\n3. History of severe drug or excipient allergy or hypersensitivity\n4. Known allergy to any of the components of the investigational drug or placebo\n5. Recent infection or febrile illness within the past 14 days\n6. Treatment with another investigational drug within the past 30 days or 5 half-lives, whichever is longer\n7. Any vaccination within the past 4 weeks or receipt of a live-attenuated vaccine within the past 6 months\n8. Use of tobacco products within the past 3 months\n9. Illicit drug use (e.g. cocaine, opioids, methamphetamine, PCP) within the past 6 months or positive urine drug screen at Screening Visit\n10. Marijuana (cannabis) use within the past 30 days or positive urine drug screen at Screening Visit\n11. History of alcohol abuse within the past 2 years\n12. Current clinically significant medical illness that is uncontrolled despite appropriate medical treatment including (but not limited to) hematologic, oncologic, renal, endocrine, pulmonary, gastrointestinal, cardiovascular, infectious, hepatic, dermatologic, psychiatric, neurologic, autoimmune or allergic disease\n13. Body mass index less than 17 or greater than 32 kg\u002Fm\\^2\n14. Clinically significant abnormal results on clinical blood testing completed at the Screening Visit\n15. Electrocardiographic evidence of clinically relevant heart disease\n16. Diabetes mellitus requiring medical treatment\n17. Received another monoclonal antibody in the past 30 days\n18. Use of herbal supplements, or similar products within the past 2 weeks\n19. Blood donation equal to or above 500 mL within 2 months prior to dosing.\n20. Any other finding that, in the judgment of the Investigator, would increase the risk of having an adverse outcome from participating in the study.\n\nJustification to exclude minors: Given that the vast majority of patients with PAH are \\>18 years of age and the fact that this is a first in human study, the risks of including children\u002Fminors (ie. subjects \\\u003C 18 yrs of age) are not justified given the limited potential benefits of including these subjects.\n\nJustification to exclude adults \\>60 years old: Subjects \\> 60 years of age are more likely to have comorbid medical conditions that could place them at increased risk of participation.",true,"60 Years",{"count":152,"type":21},96,[125],"Background:\n\nA number of diseases can cause a type of lung injury called pulmonary arterial hypertension (PAH). Most people who develop PAH do not survive more than a few years. A new study drug (ZMA001) may help. ZMA001 is a monoclonal antibody. This type of drug consists of proteins, made in a facility, that are very similar to proteins in a human body. But before giving ZMA001 to people sick with PAH, researchers want to find out how the drug affects healthy people.\n\nObjective:\n\nTo test a drug (ZMA001) in healthy volunteers.\n\nEligibility:\n\nHealthy adults aged 18 to 60 years.\n\nDesign:\n\nParticipants will be screened. They will have a physical exam with blood tests. They will have a urine test for drug use. They will have a test of their heart function.\n\nParticipants will come to the clinic for 1 inpatient visit of up to 48 hours.\n\nZMA001 is a liquid administered through a tube attached to a needle inserted into a vein in the arm. Participants will receive this drug only once, during their inpatient stay. Some participants will receive the drug; others will receive a placebo in Cohort 1 only. A placebo is a treatment that looks just like the real drug but contains no medicine. Participants will not know which treatment they are getting in Cohort 1. Cohorts 2-4 will receive a single dose of the study drug, administered through a tube attached to a needle inserted into a vein in the arm.\n\nAfter a screening visit, participants will have 1 inpatient visit and up to 8 outpatient visits over 16 weeks after receiving the treatment. Blood draws and other tests will be repeated. Each outpatient visit is approximately 2 hours long.\n\nThis study is the first time ZMA001 will be administered to people.",[156],"Pulmonary Arterial Hypertension PAH",[158],"human monoclonal antibody (IgG1)","2026-06-04",{"date":161,"type":46},"2026-06-05",{"date":163,"type":46},"2023-11-27",{"date":165,"type":21},"2027-11-30",{"name":167,"class":168},"National Heart, Lung, and Blood Institute (NHLBI)","NIH",{"id":170,"slug":171,"hasResults":12,"nctId":172,"briefTitle":173,"officialTitle":174,"acronym":4,"eligibilityCriteria":175,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":176,"enrollmentInfo":177,"targetDuration":4,"studyType":22,"phases":179,"briefSummary":181,"conditions":182,"keywords":4,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":183,"lastUpdatePostDateStruct":184,"startDateStruct":186,"completionDateStruct":188,"leadSponsor":190,"locationsCount":4},"100643598","phase-3-open-label-study-of-ikt-001-in-pulmonary-arterial-hypertension-pah-100643598","NCT07632898","Open-Label Study of IKT-001 in Pulmonary Arterial Hypertension (PAH)","Open-Label, Long-Term Extension Study to Evaluate the Safety and Tolerability of IKT-001 in Pulmonary Arterial Hypertension","Inclusion Criteria:\n\n* Capable of giving signed informed consent.\n* Participants who have completed Study IKT-001-201 or any other Phase 2 or 3 controlled clinical study with IKT-001.\n\nExclusion Criteria:\n\n* Any of the following clinical laboratory values at screening:\n\n  * ALT or AST levels \\>3× the ULN\n  * Bilirubin levels \\>2× the ULN\n  * ANC \\\u003C1.2 ×10\\^9 cells\u002FL, hemoglobin \\\u003C9 g\u002FdL, hematocrit \\\u003C30%, or platelets \\\u003C75 × 10\\^9 cells\u002FL\n  * Absolute eGFR \\\u003C30 mL\u002Fmin using creatinine or cystatin C as defined by the CKD-EPI equation (2021)\n* Currently lactating, pregnant or planning on becoming pregnant during the study.\n* Receiving treatment with sotatercept.","75 Years",{"count":178,"type":21},484,[180],"PHASE3","This is a multicenter, open-label extension (OLE), single-arm study designed to assess the long-term safety and tolerability of oral IKT-001 administered once daily.",[27],"2026-06-02",{"date":185,"type":46},"2026-06-08",{"date":187,"type":21},"2026-10-23",{"date":189,"type":21},"2030-12-30",{"name":191,"class":112},"Inhibikase Therapeutics",{"id":193,"slug":194,"hasResults":12,"nctId":195,"briefTitle":196,"officialTitle":197,"acronym":198,"eligibilityCriteria":199,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":200,"targetDuration":4,"studyType":22,"phases":202,"briefSummary":203,"conditions":204,"keywords":206,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":213,"lastUpdatePostDateStruct":214,"startDateStruct":216,"completionDateStruct":218,"leadSponsor":220,"locationsCount":222},"100606398","phase-2-a-study-of-roc-101-in-patients-with-pulmonary-arterial-hypertension-pah-and-pulmonary-hypertension-associated-with-interstitial-lung-disease-ild-ph-rocstar-study-100606398","NCT07175038","A Study of ROC-101 in Patients With Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension Associated With Interstitial Lung Disease (ILD-PH) (ROCSTAR STUDY)","A Phase 2A, Open-Label Single Arm Multicenter Exploratory Study to Evaluate the Safety, Tolerability, and Efficacy of Oral Doses of ROC-101 in Patients With Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension Associated With Interstitial Lung Disease (ILD-PH).","ROCSTAR","Key Inclusion Criteria:\n\n1. Must be age 18 or older at the time of signing the informed consent form (ICF). The participant must understand and voluntarily sign an ICF prior to any study-related procedures\n2. Documented findings on a right heart catheterization (RHC) consistent with a diagnosis World Health Organization (WHO) Group 1 PAH or WHO GROUP 3 PAH\n3. Symptomatic Pulmonary Hypertension (PH) classified as WHO Functional Class II or III symptoms\n4. PAH participants: Pulmonary Vascular Resistance (PVR) of ≥ 5 Wood units, Pulmonary Capillary Wedge Pressure (PCWP) ≤ 15 mmHg and Mean Pulmonary Arterial Pressure (mPAP) \\> 20 mm Hg and ILD-PH participants: PVR of ≥ 3 Wood units, PCWP ≤ 15 mmHg and mPAP \\> 20 mm Hg\n5. Participants on stable background therapy for PAH or ILD-PH.\n6. Females of childbearing potential (as defined in protocol) must agree to use highly effective contraception (as defined in the protocol)\n7. Male participants must follow protocol-specified contraception guidance.\n8. Participants must be able to communicate well with Investigators, understand the study procedures in the ICF and are agreeable to complete the study in accordance with the protocol.\n9. Must be able to swallow tablets.\n10. Pulmonary function tests (PFT):\n\n    PAH participants at Screening as follows:\n    1. Forced Vital Capacity (FVC) \\> 70% predicted; or if between 60% to 70% predicted, or if not possible to be determined, confirmatory High-Resolution Computed Tomography (HRCT) indicating no more than mild (\\\u003C10% fibrosis) ILD; and\n    2. The ratio of FEV1 (first second)\u002FFVC \\> 0.70 of predicted.\n\n    ILD-PH participants at Screening as follows:\n    1. PFTs consistent with their ILD diagnosis and showing FEV1\u002F FVC ratio \\> 65% and HRCT \\> 10% fibrosis, based on the proportion of lung parenchyma affected by fibrotic changes.\n\n       and,\n    2. Minimum FVC of 50% and diffusing capacity for carbon monoxide (DLCO) (corrected for Hb g\u002Fdl) \\>25%\n11. In PAH participants, i.e., Cohorts 1 and 2 only, ventilation-perfusion (VQ) scan (or, if unavailable, a negative CT pulmonary angiogram \\[CTPA\\] or pulmonary angiography result), any time prior to Screening or conducted during the Screening Period, with a normal or low probability result that is not clinically significant\n12. Acceptable Electrocardiogram (ECG) findings as assessed by the Investigator or qualified designee at the Screening Visit and at the Baseline Visit (Day 1), including each criterion as listed below:\n\n    * Normal sinus rhythm (HR) between 40 and 100 beats per minute, inclusive);\n    * Corrected QT Interval (QTcF) interval ≤ 450 msec (males) and ≤ 460 msec (females);\n    * QRS interval ≤ 120 msec; if \\> 120 msec, result will be confirmed by a manual over read\n13. Body weight at the Screening visit and at Baseline (Day 1) is greater than 50.0 kg and the body mass index (BMI) is in the range of 19.00 to 36.00 kg\u002Fm2, inclusive\n14. 6MWD ≥ 100 and ≤ 550 meters repeated twice, once during Screening Period and once at the Baseline Visit (Day 1) and both values within 15% of each other, allowing for a third repeat if \\> 15% difference, calculated from the higher\u002Fhighest value\n\nKey Exclusion Criteria:\n\n1. Diagnosis of PH WHO Groups 2, 4, or 5\n2. Diagnosis of the following PAH Group 1 subtypes: human immunodeficiency virus (HIV)-associated PAH, PAH associated with portal hypertension, schistosomiasis-associated PAH and pulmonary veno-occlusive disease and\u002For pulmonary capillary hemangiomatosis\n3. Positive blood test for hepatitis B virus surface antigen (HBsAg), hepatitis C virus (HCV) antibody (HCVAb) (unless participants have had treatment for HCV and have a negative HCV ribonucleic acid \\[RNA\\] polymerase chain reaction \\[PCR\\]) or HIV antibody\n4. Participants with known hypersensitivity to ROC-101 or any components of its formulations\n5. History of malignancy within the last 5 years, with the exception of fully excised or treated basal cell carcinoma, cervical carcinoma in-situ, or ≤ 2 squamous cell carcinomas of the skin\n6. History of clinically significant (as determined by the Investigator) non-PAH related cardiac, endocrine, hematologic, hepatic, immune, metabolic, urologic, pulmonary, neurologic, neuromuscular, dermatologic, psychiatric, renal, and\u002For other diseases that may limit participation in the study\n7. Participation in another clinical trial involving intervention with another investigational drug, approved therapy for investigational use, or investigational device within 4 weeks prior to Baseline Visit (unless it is in the follow-up period of an interventional study), or if the half-life of the previous product is known, within 5× the half-life prior to Baseline Visit (Day 1), whichever is longer\n8. Major surgery within 8 weeks prior to Baseline Visit (Day 1) or major surgery scheduled or planned in the main study. Participants must have completely recovered from any previous surgery prior to the Screening Visit\n9. Prior heart or heart-lung transplants, or a participant listed for heart and\u002For lung transplantation or prior pneumonectomy\n10. Pregnant or breastfeeding females\n11. Males who do not agree to protocol contraception guidelines\n12. Uncontrolled systemic hypertension as evidenced by sitting SBP \\> 160 mm Hg or sitting diastolic BP \\> 100 mm Hg during Screening Visit and Baseline Visit (Day 1) after a period of rest\n13. Systolic BP \\\u003C 90 mm Hg during Screening Visit or at Baseline Visit (Day 1)\n14. History of known pericardial constriction or a clinically significant (more than trace or trivial \\[i.e., ≥10 mm\\]) pericardial effusion seen in diastole or in both systole and diastole on echocardiogram (ECHO) historically and confirmed on screening ECHO\n15. RHC contraindicated during the study per Investigator\n16. Cerebrovascular accident within 3 months (120 days) of start of Screening\n17. History of restrictive or constrictive or congestive cardiomyopathy\n18. Left ventricular ejection fraction (LVEF) \\\u003C 50% on historical echocardiogram (ECHO) performed within 6 months prior to start of Screening period (and confirmed during the Screening ECHO) or grade 2 or higher diastolic dysfunction\n19. Any current symptomatic coronary disease (myocardial infarction, percutaneous coronary intervention, coronary artery bypass graft surgery, or cardiac anginal chest pain in the past 6 months (180 days) prior to start of Screening)\n20. History of acutely decompensated left heart failure or right heart failure within 90 days prior to Baseline, as per Investigator assessment\n21. Significant (≥ 2+ \\[or \\> mild\\] regurgitation) mitral regurgitation or aortic regurgitation valvular disease, or more than mild mitral stenosis or aortic stenosis valvular disease as seen on Screening ECHO\n22. Started or stopped receiving any general supportive therapy for PH (e.g., oxygen, anticoagulants, digoxin) within 30 days prior to start of Screening\n23. Use of supplemental oxygen \\> 10 liters\u002Fminute and SaO2 \\\u003C 90% while receiving typical oxygen supplementation\n24. Received intravenous (IV) inotropes (e.g., dobutamine, dopamine, norepinephrine, vasopressin) within 30 days prior to start of Screening\n25. History of atrial septostomy within 180 days prior to start of Screening\n26. History of portal hypertension or chronic liver disease, defined as mild to severe hepatic impairment (Child-Pugh Classes A to C)\n27. Untreated, severe (defined as apnea hypoxia index of \\> 30) obstructive sleep apnea\n28. Active daily smoker of cannabis or tobacco\n29. Current alcohol abuse or current illicit drug use\n30. WHO Group 3 due to severe chronic obstructive pulmonary disease (COPD) or chronic pulmonary fibrosis and emphysema (CPFE) or PFT with FVC \\\u003C 50% or FEV1\u002FFVC \\\u003C 65% or DLCO \\\u003C 25% (corrected for Hb g\u002Fdl)\n31. Presence of lab abnormalities at Screening\n32. History of greater than severe renal disease, including any episode of acute renal failure, with or without a prior history of renal disease in which acute dialysis (e.g., intermittent hemodialysis or continuous veno-venous hemofiltration) was required\n33. Initiation of an exercise program for cardiopulmonary rehabilitation within 90 days prior to Baseline or planned initiation during the study (participants who are stable in the maintenance phase of a program and who will continue for the duration of the study are eligible)\n34. Participants who plan to continue, or start during the study, medications which are sensitive cytochrome (CYP) 2D6 substrates with a narrow therapeutic index, such as nortriptyline, venlafaxine, and amitriptyline or CYP1A2 substrates with a narrow therapeutic index\n35. History or presence of impaired cardiac function\n36. Participants who plan to donate blood after signing consent for the study and for 28 days after their last dose of study drug\n\nKey Inclusion Criteria for Extension Period:\n\n1. Participants must complete the main study period (defined as completion of assessments through the Week 24 visit)\n2. Women of child-bearing potential (WOCBP) must have negative pregnancy test\n3. All participants must comply with contraceptive guidance until 28 days after last dose of study drug for WOCBP and 90 days after the last dose of study drug for males\n\nKey Exclusion Criteria for Extension Period:\n\n1. Participant withdrew from main study period due to an AE related to study drug\n2. Female participant who is pregnant, breastfeeding, or intends to conceive during the long-term extension period\n3. Males who do not agree to follow protocol contraception guidelines\n4. Any condition that in the opinion of the investigator may pose a risk to the participant, interferes with the participant's participation or confounds assessments of the participant",{"count":201,"type":21},40,[93],"This study evaluates the effect of ROC-101 in adults with either Pulmonary Arterial Hypertension (PAH) or Pulmonary Hypertension Associated with Interstitial Lung Disease (ILD-PH). Each eligible participant will receive standard of care (SOC) plus ROC-101 for a 24-week treatment period, followed by a long-term extension period of the study through the end of the program or marketing approval\u002Fauthorization.",[27,205],"Pulmonary Hypertension, Interstitial Lung Disease",[207,208,209,210,211,212,27,205],"Rho kinase inhibitor","ROCK inhibitor","Pan ROCK Inhibitor","ROCK-1 and ROCK-2 Inhibitor","ROC-101","PVR","2026-05-27",{"date":215,"type":46},"2026-05-28",{"date":217,"type":46},"2025-10-01",{"date":219,"type":21},"2029-03",{"name":221,"class":112},"AllRock Bio, Inc.",19,{"id":224,"slug":225,"hasResults":12,"nctId":226,"briefTitle":227,"officialTitle":228,"acronym":229,"eligibilityCriteria":230,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":176,"enrollmentInfo":231,"targetDuration":4,"studyType":22,"phases":233,"briefSummary":234,"conditions":235,"keywords":238,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":240,"lastUpdatePostDateStruct":241,"startDateStruct":243,"completionDateStruct":244,"leadSponsor":246,"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":232,"type":21},300,[180],"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.",[41,27,236,156,237],"Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH)","Pulmonary Arterial Hypertension WHO Group I",[239,41],"PAH","2026-05-22",{"date":242,"type":46},"2026-05-29",{"date":242,"type":21},{"date":245,"type":21},"2029-11-30",{"name":247,"class":112},"VIVUS LLC",{"id":249,"slug":250,"hasResults":12,"nctId":251,"briefTitle":252,"officialTitle":253,"acronym":254,"eligibilityCriteria":255,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":176,"enrollmentInfo":256,"targetDuration":4,"studyType":22,"phases":257,"briefSummary":258,"conditions":259,"keywords":260,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":264,"lastUpdatePostDateStruct":265,"startDateStruct":266,"completionDateStruct":268,"leadSponsor":270,"locationsCount":272},"100640903","phase-2-tiprelestat-versus-placebo-when-added-to-standard-of-care-for-the-treatment-of-pulmonary-arterial-hypertension-pah-100640903","NCT07601295","Tiprelestat Versus Placebo When Added to Standard of Care for the Treatment of Pulmonary Arterial Hypertension (PAH)","A Phase II, Randomized, Double-Blind, Safety and Efficacy Study of Tiprelestat Versus Placebo When Added to Standard of Care for the Treatment of Pulmonary Arterial Hypertension (PAH)","ATHENA","Inclusion Criteria:\n\n1. Adults age 18 to 75 years.\n2. Willingness to give written informed consent prior to any study-related procedures being performed and to be able to adhere to the study restrictions and examination schedule.\n3. Diagnosis of WHO Group I PAH.\n4. WHO functional class II - IV despite optimized treatment SOC, with 1 or more modalities including phosphodiesterase 5 (PDE5) inhibitor, soluble guanylate cyclase stimulator (sGCS), endothelin receptor antagonist (ERA), and\u002For a prostacyclin analogue or receptor agonist (SC\u002Finhaled\u002FPO) (see #5), as well as Sotatercept (see #6).\n5. On stable doses of PDE5 inhibitor, ERA, sGCS, or prostacyclin analogue\u002Freceptor agonist for at least 90 days prior to screening; for infusion prostacyclins, dose adjustment within 10% of baseline dose during the duration of the study is allowed per medical practice.\n6. On stable doses of Sotatercept therapy for at least 6 months prior to screening, and intended to be continued during the duration of the study.\n7. Screening right heart catheterization mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest; pulmonary wedge pressure (PAWP) ≤ 15 mmHg or left ventricular end diastolic pressure (LVEDP) ≤ 15 mmHg; AND pulmonary vascular resistance (PVR) ≥ 400 dynes•sec\u002Fcm5 (5 Wood Units).\n8. If participant is of childbearing potential, willing to use adequate methods of contraception throughout the course of the study. If participant is of childbearing potential (a participant \\\u003C 55 years of age who has not been postmenopausal for ≥ 5 years or who has not had a bilateral salpingectomy, hysterectomy and\u002For oophorectomy), need to employ two reliable means of contraception which may include surgical sterilization, barrier methods, spermicidals, intrauterine devices, and\u002For hormonal contraception, unless the participant chooses abstinence (to avoid heterosexual intercourse completely). If a participant chooses abstinence, then a second reliable means of contraception is not needed.\n9. 6MWD ≥100 and ≤500 meters at screening.\n10. Willing to adhere to study restrictions and examination schedule.\n\nExclusion Criteria:\n\n1. Diagnosis of WHO Group 2 - 5 Pulmonary Hypertension.\n2. Participation in another clinical trial, or experimental use, involving a PAH investigational drug or device within the last 3 months.\n3. Total lung capacity (TLC) \\\u003C 60% predicted; if TLC is ≥ 60% and \\\u003C 70% predicted, high resolution computed tomography (HRCT) must be available to exclude significant interstitial lung disease.\n4. FEV1 \u002F FVC \\\u003C 70% predicted and FEV1 \\\u003C 60% predicted.\n5. Significant left-sided heart disease (based on screening Echocardiogram):\n\n   1. Moderate or severe aortic or mitral valve disease\n   2. Diastolic dysfunction ≥ Grade II\n   3. LV systolic function \\\u003C 45%\n   4. Pericardial constriction\n   5. Restrictive cardiomyopathy\n   6. Significant coronary disease with demonstrable ischemia\n6. Chronic renal insufficiency defined as an estimated creatinine clearance \\\u003C 30 ml\u002Fmin.\n7. Current atrial arrhythmias not under optimal control.\n8. Uncontrolled systemic hypertension: SBP \\> 160 mmHg or DBP \\> 100mmHg.\n9. Severe hypotension: SBP \\\u003C 80 mmHg.\n10. Pregnant or breast-feeding.\n11. Psychiatric, addictive, or other disorders that compromise the patient's ability to provide informed consent, to follow study protocol, and adhere to treatment instructions.\n12. Known allergy or hypersensitivity to tiprelestat.\n13. Moderate to severe hepatic dysfunction with a Child Pugh score \\>10.\n14. Hyperkalemia defined as Potassium \\> 5.1 mEq\u002FL at screening.\n15. Initiation of an exercise program for cardiopulmonary rehabilitation within 90 days prior to screening or planned during the study. Subjects who are already stable in the maintenance phase of an exercise program which will continue for the duration of the study are eligible.\n16. Known active infection requiring antibiotic, antifungal, or antiviral therapies. Patients may be rescreened at physician discretion after the resolution of infection and discontinuation of antibiotic, antifungal, or antiviral therapies.\n17. Co-morbid conditions that would impair a patient's exercise performance and ability to assess WHO functional class, including but not limited to chronic low-back pain or peripheral musculoskeletal problems, other comorbidities expected to alter the patient's clinical course (i.e. active cancer; \\>3 comorbidities e.g., obesity, systemic HTN, diabetes).",{"count":64,"type":21},[93],"The primary objective of this study is to compare the efficacy, safety, and tolerability of tiprelestat plus Standard of Care (SOC) compared with placebo plus SOC in patients with World Health Organization (WHO) functional class II-IV pulmonary arterial hypertension (PAH).",[27],[261,262,263,239],"tiprelestat","elafin","pulmonary arterial hypertension","2026-05-15",{"date":240,"type":46},{"date":267,"type":21},"2026-07",{"date":269,"type":21},"2030-03",{"name":271,"class":140},"Stanford University",10,{"id":274,"slug":275,"hasResults":12,"nctId":276,"briefTitle":277,"officialTitle":278,"acronym":4,"eligibilityCriteria":279,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":176,"enrollmentInfo":280,"targetDuration":4,"studyType":22,"phases":282,"briefSummary":283,"conditions":284,"keywords":4,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":264,"lastUpdatePostDateStruct":285,"startDateStruct":287,"completionDateStruct":289,"leadSponsor":290,"locationsCount":292},"100638141","phase-1-a-phase-ba-clinical-study-of-imc-003-injection-for-the-treatment-of-pulmonary-arterial-hypertension-receiving-background-therapy-100638141","NCT07593027","A Phase Ⅰb\u002FⅡa Clinical Study of IMC-003 Injection for the Treatment of Pulmonary Arterial Hypertension Receiving Background Therapy.","A Randomized, Double-blind, Placebo-controlled, Multiple-dose, Dose-escalation Phase Ib\u002FIIa Clinical Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Preliminary Efficacy of IMC-003 Injection in Patients With Pulmonary Arterial Hypertension (PAH) Receiving Background Therapy.","Inclusion Criteria:\n\n1. Age screening between 18 and 75 years (inclusive), regardless of gender.\n2. Patients diagnosed with WHO Group 1 pulmonary arterial hypertension (PAH) confirmed by right heart catheterization (RHC) before initial dosing (see Appendix 1), including the following subtypes: idiopathic PAH; heritable PAH; drug- or toxin-induced PAH; PAH associated with connective tissue diseases that is inactive; PAH associated with simple congenital heart defects with systemic-to-pulmonary shunts, at least one year post-correction surgery.\n3. Symptomatic pulmonary arterial hypertension, WHO functional class II or III.\n4. Must also meet the following hemodynamic criteria: mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest; pulmonary artery wedge pressure (PAWP) ≤15 mmHg.\n5. Pulmonary vascular resistance (PVR) ≥5 Wood Units (400 dyn·sec·cm-5) by RHC before the first dose, results from pre-screening RHC are acceptable (if they meet the study requirements).\n6. Receiving stable doses of background PAH therapy (i.e., each therapy's individualized target dose has been achieved and stable for at least 90 days before initial dosing).\n7. Six-minute walk distance (6MWD) during screening ≥150 meters and ≤450 meters.\n8. Female participants of childbearing potential must have a negative serum pregnancy test before dosing and agree to undergo regular urine or serum pregnancy tests (any one) during the treatment period; agree to use highly effective contraception before dosing, during the treatment period (including dosing interruptions), and for 16 weeks (112 days) after the last dose, and to avoid blood donation or egg donation within 16 weeks (112 days) after the last dose.\n9. Male participants agree to use condoms, defined as using latex condoms or non-latex (non-animal membrane) condoms (e.g., polyurethane) during sexual intercourse with pregnant or childbearing potential women during treatment (including dosing interruptions) and for 16 weeks (112 days) after the last dose, even if a successful vasectomy has been performed, and to avoid sperm donation within this period.\n10. Participants understand and comply with the study procedures, voluntarily participate, and sign the informed consent form.\n\nExclusion Criteria:\n\n1. Diagnosed with pulmonary hypertension of WHO Group 2, 3, 4, or 5.\n2. Diagnosed with the following PAH subtypes of WHO Group 1: HIV-associated PAH, portal hypertension-associated PAH, schistosomiasis-associated PAH, PAH related to pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis, patients with PAH known to have a positive acute pulmonary vasoreactivity test.\n3. Poorly controlled hypertension after screening: seated systolic BP \\>160 mmHg or seated diastolic BP \\>100 mmHg, or pre-dose systolic BP \\\u003C90 mmHg on C1D1.\n4. Pre-dose electrocardiogram (ECG) Fridericia-corrected QT interval (QTcF) ≥470 ms for men, ≥480 ms for women; or personal or family history of long QT syndrome (LQTS) or sudden cardiac death.\n5. Untreated mild or more severe obstructive sleep apnea.\n6. Previously underwent or plans to undergo heart or cardiopulmonary transplantation, or expected lifespan \\\u003C12 months as assessed by the investigator.\n7. Diagnosed with chronic obstructive pulmonary disease (COPD) or other clinically significant lung disease.\n8. Evidence of interstitial lung disease (ILD) based on chest CT within 1 year before screening or pulmonary function test (PET) within 6 months before screening will lead to exclusion; if the above tests are not available, or chest CT within 1 year shows mild or greater ILD, a PET or chest CT should be performed during the screening period, and patients with evidence of ILD will be excluded.\n9. Pre-dose hemoglobin (Hb) above the sex-specific upper limit of normal (ULN) or \\\u003C90 g\u002FL, platelet count ≤100 × 10\\^9\u002FL, neutrophils \\\u003C1.5 × 10\\^9\u002FL, aspartate aminotransferase (AST) or alanine aminotransferase (ALT) \\>3 × ULN, total bilirubin \\>1.5 × ULN, estimated glomerular filtration rate (eGFR) \\\u003C60 mL\u002Fmin\u002F1.73m\\^2.\n10. History of portal hypertension or chronic liver disease, including Hepatitis B Surface Antigen (HBsAg) positive, and\u002For Hepatitis B Core Antibody (HBcAb) positive and Hepatitis Bvirus-Deoxyribonucleic acid HBV-DNA) titers outside the normal range; Hepatitis C virus (HCV) positive and hepatitis C virus ribonucleic acid (HCV-RNA) positive; Human Immunodeficiency Virus (HIV) seroresponse; Treponema pallidum antibody (TP-Ab) positive (if Treponema pallidum serology test is positive, further non-Treponema pallidum serology test, which is negative and judged by the investigator to be cured of syphilis in the past is eligible for inclusion).\n11. are participating in other clinical studies; or have participated in clinical studies of medical devices or other drugs within 30 days before screening (except for those who have only signed the ICF and have not received the investigational drug or device intervention), or are still within 5 half-lives (t1\u002F2) of the investigational drug (whichever is longer).\n12. Previous treatment targeting the TGF-β superfamily (e.g., Sotatercept) (including participation in a clinical trial).\n13. Known participant has a prior allergy to macromolecular protein preparations\u002Fmonoclonal antibodies, known hypersensitivity to the investigational drug or its excipients, or the same type of drug.\n14. History of opportunistic infection (e.g., invasive candidiasis or pneumocystis pneumonia within 6 months prior to dosing); Severe local infection (e.g., cellulitis, abscess) or systemic infection (e.g., sepsis) within 4 weeks prior to screening.\n15. History of malignancy or current malignancy (excluding basal cell carcinoma that has been resected with no evidence of metastatic disease for 3 years, or cervical intraepithelial neoplasia that has been treated and is not known to have recurred).\n16. History of chronic kidney disease prior to screening, or acute renal failure requiring acute dialysis within 3 months prior to screening, regardless of whether there was a previous history of kidney disease.\n17. Females who are pregnant or breastfeeding.\n18. Participants who are considered unsuitable for the study in the opinion of the investigator.",{"count":281,"type":21},120,[125,93],"This is a Phase Ib\u002FIIa clinical study of IMC-003 treatment in pulmonary arterial hypertension (PAH) patients receiving background therapy",[156],{"date":286,"type":46},"2026-05-18",{"date":288,"type":46},"2026-04-30",{"date":165,"type":21},{"name":291,"class":140},"ImmuneCare Biopharmaceuticals (Shanghai) Co., Ltd.",32,{"id":294,"slug":295,"hasResults":12,"nctId":296,"briefTitle":297,"officialTitle":298,"acronym":299,"eligibilityCriteria":300,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":301,"targetDuration":4,"studyType":22,"phases":303,"briefSummary":304,"conditions":305,"keywords":308,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":315,"lastUpdatePostDateStruct":316,"startDateStruct":318,"completionDateStruct":320,"leadSponsor":322,"locationsCount":141},"100611830","phase-3-commodities-trial-initial-dual-oral-therapy-vs-monotherapy-in-pah-with-cardiovascular-comorbidities-100611830","NCT07245680","COMMODITIES Trial: Initial Dual Oral Therapy vs Monotherapy in PAH With Cardiovascular Comorbidities","Comparison of Initial Dual Oral COMbination Therapy to MOnotherapy in Pulmonary Arterial Hypertension With Cardiovascular comorbiDITIES","COMMODITIES","Inclusion Criteria:\n\n* Initial PAH diagnosis \\\u003C 6 months preceding randomisation\n\n  * Negative vasoreactivity test\n  * Treatment-naïve PAH (group 1): idiopathic, heritable, associated with drugs and toxin, associated with connective tissue disease, HIV infection or systemic-to-pulmonary congenital shunt corrected for more than one year\n  * Meet all of the following hemodynamic criteria by means of a RHC prior to screening:\n* mPAP≥25 mmHg and\n* PAWP\\\u003C15 mmHg and\n* with PVR≥3 WU\n\n  • Presence of at least two of the following criteria, as listed in the European pulmonary hypertension guidelines:\n* History of essential hypertension\n* Diabetes mellitus (any type)\n* Obesity (defined by a BMI ≥30 kg\u002Fm2)\n* Coronary heart disease (established by any of the following: history of myocardial infarction, history of percutaneous coronary intervention, angiographic evidence of coronary artery disease (\\>50% stenosis in ≥1 vessel), positive ST, previous coronary artery bypass graft, stable angina)\n\n  * Participant able to understand the study procedures\n  * For women of childbearing potential (WOCBP), effective form of contraception\\* from screening up to 1 month following discontinuation of the last study treatment\n  * Affiliation to the french social security regime\n  * Signed written informed consent\n\nExclusion Criteria:\n\n* Porto-pulmonary hypertension\n* Uncorrected systemic-to-pulmonary congenital shunt\n* Evidence of thromboembolic disease assessed by ventilation perfusion (V\u002FQ) lung scan or CT pulmonary angiography\n* Patients listed for lung or heart-lung transplantation at time of screening\n* Patients on any PAH-specific drug therapy at any time preceding randomisation\n* Known moderate-to-severe restrictive lung disease (i.e., total lung capacity \\\u003C 60% of predicted value) or obstructive lung disease (i.e., forced expiratory volume in one second \\[FEV1\\] \\\u003C 60% of predicted, with FEV1 \u002F forced vital capacity \\\u003C 65%) or known significant chronic lung disease diagnosed by chest imaging (e.g., interstitial lung disease, emphysema).\n* Known or suspected pulmonary veno-occlusive disease (PVOD)\n* Severe renal insufficiency (creatinine clearance \\\u003C 30 mL\u002Fmin)\n* Documented severe hepatic impairment (with or without cirrhosis) according to National Cancer Institute organ dysfunction working group criteria, defined as total bilirubin \\> 3 x ULN or serum AST and\u002For ALT \\> 3xULN (assessed by local laboratory at screening) and\u002For Child-Pugh Class C.\n* Haemoglobin \\\u003C 10 g\u002FdL\n* Patient under guardianship curatorship, deprived of liberty\n* Pregnant women, or breast-feeding women\n* Treatment with other PDE-5i for erectile dysfunction\n* Ongoing or planned treatment with nitrates and\u002For doxazosin.\n* Ongoing or planned treatment with riociguat\n* Treatment with strong inducers of CYP3A4 (e.g., carbamazepine, rifampin, rifampicin, rifabutin, rifapentin, phenobarbital, phenytoin, and St. John's wort) ≤28 days preceding randomisation",{"count":302,"type":21},186,[180],"Pulmonary arterial hypertension (PAH) is a rare, progressive disease associated with poor prognosis, especially in patients with cardiovascular comorbidities. Current guidelines recommend initial combination therapy, but evidence is lacking for patients with significant comorbidities who are often excluded from clinical trials.\n\nThe COMMODITIES trial is a multicenter, randomized, controlled study designed to compare the efficacy and safety of initial dual oral combination therapy (tadalafil and ambrisentan) versus oral monotherapy in newly diagnosed PAH patients with at least two cardiovascular comorbidities. The study aims to provide robust evidence to guide treatment strategies in this high-risk population.",[27,306,307],"Comorbidities","Cardiovascular Disease (CVD)",[239,309,310,311,312,313,314],"Endothelin Receptor Antagonist (Ambrisentan)","Phosphodiesterase-5 Inhibitor (Tadalafil)","Cardiovascular Comorbidities","Combination Therapy","Randomized Controlled Trial","Pulmonary arterial hypertension","2026-05-12",{"date":317,"type":46},"2026-05-13",{"date":319,"type":46},"2026-04-28",{"date":321,"type":21},"2029-02-14",{"name":323,"class":140},"Assistance Publique - Hôpitaux de Paris",{"id":325,"slug":326,"hasResults":12,"nctId":327,"briefTitle":328,"officialTitle":329,"acronym":4,"eligibilityCriteria":330,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":331,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":333,"conditions":334,"keywords":4,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":288,"lastUpdatePostDateStruct":337,"startDateStruct":338,"completionDateStruct":339,"leadSponsor":341,"locationsCount":4},"100636817","development-and-validation-of-a-chinese-quality-of-life-scale-for-pulmonary-arterial-hypertension-100636817","NCT07570615","Development and Validation of a Chinese Quality of Life Scale for Pulmonary Arterial Hypertension","Development and Validation of a Chinese Patient-Reported Outcome Measure for Quality of Life in Pulmonary Arterial Hypertension Patients","Inclusion Criteria:\n\n* Age ≥18 years\n* Diagnosed with pulmonary arterial hypertension\n* Able to read and complete questionnaires\n* Provided informed consent\n\nExclusion Criteria:\n\n* Severe physical condition preventing participation\n* History of psychiatric disorders or current use of psychiatric medication\n* Pregnancy or lactation\n* Any condition preventing completion of questionnaires",{"count":232,"type":21},"OBSERVATIONAL","This study aims to develop and validate a culturally appropriate, reliable, and clinically applicable patient-reported outcome (PRO) instrument to assess quality of life in patients with pulmonary arterial hypertension (PAH) in China.\n\nThe study includes two phases: (1) scale development using literature review, Delphi expert consultation, and patient interviews; and (2) multicenter prospective observational validation to evaluate reliability, validity, and responsiveness.\n\nThe final scale is expected to support clinical assessment, treatment monitoring, and health policy decision-making.",[27,335,336],"Quality of Life","Patients Reported Outcomes",{"date":107,"type":46},{"date":288,"type":21},{"date":340,"type":21},"2029-04-30",{"name":342,"class":140},"Chinese Pulmonary Vascular Disease Research Group",{"id":344,"slug":345,"hasResults":12,"nctId":346,"briefTitle":347,"officialTitle":348,"acronym":349,"eligibilityCriteria":350,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":150,"enrollmentInfo":351,"targetDuration":4,"studyType":22,"phases":352,"briefSummary":353,"conditions":354,"keywords":355,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":357,"lastUpdatePostDateStruct":358,"startDateStruct":360,"completionDateStruct":362,"leadSponsor":364,"locationsCount":141},"100583114","phase-1-a-study-evaluating-the-safety-and-tolerability-of-artesunate-in-patients-with-pulmonary-arterial-hypertension-100583114","NCT06872112","A Study Evaluating the Safety and Tolerability of Artesunate in Patients With Pulmonary Arterial Hypertension","Phase I Study: A Dose-Escalation Study Evaluating the Safety and Tolerability of Artesunate in Patients With Pulmonary Arterial Hypertension","(STOP-PAH)","Inclusion Criteria\n\n* Each participant must meet the following criteria to be enrolled in this study:\n* Adults aged 18 to 75 years.\n* WHO functional class I, II\u002FIII despite treatment with maximally tolerated doses of 2 or more treatment modalities including PDE5 inhibitors, guanylate cyclase stimulators, endothelin receptor antagonists, and prostanoids when appropriate.\n\nExclusion Criteria\n\n* Participants who meet any of the following criteria will be excluded from the study.\n* Participants with serious concomitant morbidity per investigator assessment.",{"count":123,"type":21},[125],"This is a 20-week, Phase 1, single-center, open-label, dose-escalation study evaluating the safety and tolerability of daily oral artesunate in patients with PAH.",[27],[41,356,239],"Artesunate","2026-04-21",{"date":359,"type":46},"2026-04-23",{"date":361,"type":46},"2026-04-01",{"date":363,"type":21},"2029-01-01",{"name":365,"class":140},"Joseph C. Wu",{"id":367,"slug":368,"hasResults":12,"nctId":369,"briefTitle":370,"officialTitle":370,"acronym":4,"eligibilityCriteria":371,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":372,"enrollmentInfo":373,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":375,"conditions":376,"keywords":4,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":378,"lastUpdatePostDateStruct":379,"startDateStruct":381,"completionDateStruct":383,"leadSponsor":385,"locationsCount":141},"100630420","reverse-remodeling-of-the-pulmonary-vasculature-a-longitudinal-investigational-study-of-the-effects-of-sotatercept-100630420","NCT07487441","Reverse Remodeling of the Pulmonary Vasculature: a Longitudinal, Investigational Study of the Effects of Sotatercept.","Inclusion Criteria - Feasibility Group:\n\n* Group 1 Pulmonary Hypertension (G1PH)-must be idiopathic, drug induced, or hereditary (D\u002FH\u002FI)\n* Age \\\u003C 65 years at time of diagnosis\n* Must be 18 years of age or older\n\nInclusion Criteria - \"Real-world\" treatment naïve cohort:\n\n* Group 1 Pulmonary Hypertension (G1PH)-all types\n* Must be treatment naïve\n* Must be 18 years of age or older\n\nInclusion Criteria - \"Real-world\" prevalent cohort:\n\n* Group 1 Pulmonary Hypertension (G1PH)-all types\n* Must be 18 years of age or older\n\nExclusion Criteria:\n\n* Contraindication to right heart catheterization (per PI)\n* Prior exposure to sotatercept\n* Hemoglobin \\> 18 g\u002FdL at screening\n* Estimated GFR \\\u003C 30 mL\u002Fmin at screening\n* Platelet count \\\u003C30 at screening\n* Positive pregnancy test","65 Years",{"count":374,"type":21},30,"The goal of this study is to learn more about how sotatercept works and if it helps the lung arteries become healthier. Sotatercept will be associated with the following:\n\n1. Improvement in capillary blush, reduce the tapering and tortuosity of affected vessels on pulmonary wedge angiography and decreased wall thickness on intravascular ultrasound in previously affected areas.\n2. Improvement in previously poorly or non-perfused areas rather than increased perfusion to previously perfused areas.\n3. No changes in baseline ventilation and improvement and ventilation\u002Fperfusion matching.",[27,377],"Pulmonary Hypertension","2026-03-17",{"date":380,"type":46},"2026-03-23",{"date":382,"type":21},"2026-03-15",{"date":384,"type":21},"2028-06-15",{"name":386,"class":140},"Franz Rischard, DO",{"id":388,"slug":389,"hasResults":12,"nctId":390,"briefTitle":391,"officialTitle":392,"acronym":393,"eligibilityCriteria":394,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":395,"targetDuration":4,"studyType":22,"phases":397,"briefSummary":398,"conditions":399,"keywords":400,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":409,"lastUpdatePostDateStruct":410,"startDateStruct":412,"completionDateStruct":414,"leadSponsor":416,"locationsCount":141},"100602229","validation-of-a-patient-knowledge-questionnaire-for-pulmonary-hypertension-100602229","NCT07120789","Validation of a Patient Knowledge Questionnaire for Pulmonary Hypertension","Content, Semantic, and Psychometric Validation of a Patient Knowledge Questionnaire for Pulmonary Hypertension","SAVOIR","Inclusion Criteria:\n\n* Adults aged 18 years or older\n* Confirmed diagnosis of Group 1 Pulmonary Arterial Hypertension (PAH)\n* Ability to read and understand the questionnaire language\n* Provided informed consent to participate in the study\n* For expert judges (content validation phase): professionals with recognized expertise in pulmonary hypertension, with academic or clinical experience in the field\n\nExclusion Criteria:\n\n* Cognitive, visual, or auditory impairments that hinder questionnaire comprehension\n* Refusal to participate or withdrawal of informed consent\n* Inability to complete the questionnaire due to clinical instability",{"count":396,"type":21},215,[24],"This methodological study aims to develop and validate a questionnaire named for the Assessment of Patient Knowledge in Pulmonary Arterial Hypertension. The study will be conducted in four phases: (1) questionnaire development based on guidelines and literature; (2) content validation by expert judges; (3) semantic validation with patients; and (4) psychometric testing in a sample of up to 200 patients with confirmed pulmonary arterial hypertension (PAH).\n\nIn addition to validation, the study will collect clinical and functional data from medical records, including risk stratification using the COMPERA 2.0 method The final instrument is expected to support patient education strategies and contribute to improved clinical management of PAH.",[27,236,377],[377,401,402,403,404,405,406,407,408],"Patient Knowledge","Questionnaire Validation","Content Validity","Semantic Analysis","Psychometric Validation","Health Education","Patient Education","Chronic Disease","2026-03-07",{"date":411,"type":46},"2026-03-10",{"date":413,"type":46},"2025-09-01",{"date":415,"type":21},"2026-12",{"name":417,"class":140},"University of Sao Paulo General Hospital",{"id":419,"slug":420,"hasResults":12,"nctId":421,"briefTitle":422,"officialTitle":422,"acronym":423,"eligibilityCriteria":424,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":425,"targetDuration":4,"studyType":22,"phases":427,"briefSummary":428,"conditions":429,"keywords":430,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":433,"lastUpdatePostDateStruct":434,"startDateStruct":436,"completionDateStruct":438,"leadSponsor":440,"locationsCount":141},"100628140","airdrop-can-we-improve-adherence-to-inhaled-treatment-for-pulmonary-arterial-hypertension-100628140","NCT07457762","AIRDROP: Can we Improve Adherence to Inhaled Treatment for Pulmonary Arterial Hypertension?","AIRDROP","Inclusion Criteria:\n\n* \"Adult patients aged ≥ 18 years, treated at the referral center for PH management at the Heart Institute of the University of São Paulo Medical School, with an invasive confirmed diagnosis of PAH (mean pulmonary arterial pressure \\[mPAP\\] greater than 20 mmHg at rest, pulmonary vascular resistance \\[PVR\\] equal to or greater than 2 Wood units, and pulmonary capillary wedge pressure \\[PCWP\\] equal to or less than 15 mmHg), and who have been using Iloprost.\n\nExclusion Criteria:\n\n* Patients who are unable to perform the 6-minute walk test (6MWT) or who have experienced gastrointestinal bleeding within the 12 weeks prior to the start of the study will be excluded.",{"count":426,"type":21},72,[24],"Pulmonary Arterial Hypertension is a rare and progressive condition that compromises pulmonary circulation and can lead to right ventricular failure. Despite recent advances in diagnosis and treatment, the median survival of patients is only 2.8 years. The treatment for this disease is based on drugs that act on three main pathways: prostacyclin, endothelin, and nitric oxide. Iloprost, a prostacyclin analogue available in an inhaled form, is an important and well-established treatment. However, its mandatory frequent administration, the need for a specific inhalation technique, and its adverse event profile make its use complex. Although pharmacotherapeutic and inhalation technique follow-up by a qualified professional is widely studied in diseases like asthma and COPD, its application in Pulmonary Arterial Hypertension still lacks evidence. Thus, this study aims to evaluate how a pharmacist's intervention can improve treatment adherence, mitigate side effects and difficulties associated with inhalation, in addition to optimizing clinical and hemodynamic outcomes in patients with Pulmonary Arterial Hypertension using iloprost.",[27],[239,431,432],"adherence","iloprost","2026-03-03",{"date":435,"type":46},"2026-03-09",{"date":437,"type":46},"2025-05-01",{"date":439,"type":21},"2026-12-28",{"name":417,"class":140},{"id":442,"slug":443,"hasResults":12,"nctId":444,"briefTitle":445,"officialTitle":446,"acronym":447,"eligibilityCriteria":448,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":449,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":450,"conditions":451,"keywords":452,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":459,"lastUpdatePostDateStruct":460,"startDateStruct":462,"completionDateStruct":464,"leadSponsor":466,"locationsCount":141},"100627776","long-term-outcomes-of-selexipag-in-schistosomiasis-associated-pulmonary-arterial-hypertension-100627776","NCT07453030","Long-Term Outcomes of Selexipag in Schistosomiasis-Associated Pulmonary Arterial Hypertension","PROPULSE-Sch: Long-Term Evaluation of Selexipag in Schistosomiasis-Associated Pulmonary Arterial Hypertension Using a Propensity Score-Matched Mirror Cohort","PROPULSE-Sch","Inclusion Criteria\n\nAdults aged 18 years or older.\n\n* Confirmed diagnosis of pulmonary arterial hypertension associated with schistosomiasis (PAH-Sch).\n* Diagnosis of pre-capillary pulmonary arterial hypertension confirmed by right heart catheterization, performed at any time prior to the index date (T0), as documented in the medical record.\n* Evidence of schistosomiasis infection, including epidemiological history and ultrasonographic findings compatible with hepatosplenic schistosomiasis.\n* Previous antiparasitic treatment for schistosomiasis.\n* Clinical stability at the index date, defined as absence of progressive right heart failure or clinical worsening within the previous 12 weeks.\n* World Health Organization (WHO) functional class I-III at the index date.\n* Stable background pulmonary arterial hypertension-specific therapy with a phosphodiesterase-5 inhibitor and\u002For endothelin receptor antagonist for at least 12 weeks prior to the index date.\n* For the treated cohort: initiation of oral selexipag as part of routine clinical care.\n* For the mirror cohort: eligibility for therapeutic escalation at the index date without exposure to selexipag.\n\nExclusion Criteria\n\n* World Health Organization (WHO) functional class IV at the index date.\n* Progressive right heart failure or clinical deterioration within the 12 weeks prior to the index date.\n* Documented formal contraindication to selexipag in the medical record.\n* Insufficient baseline data at the index date to allow clinical characterization or inclusion in propensity score analyses.",{"count":374,"type":21},"Schistosomiasis-associated pulmonary arterial hypertension is a serious condition that can lead to shortness of breath, heart failure, frequent hospitalizations, and early death. Although treatments for pulmonary arterial hypertension have improved over time, patients with this specific cause of the disease are often not included in long-term studies.\n\nSelexipag is an oral medication used to treat pulmonary arterial hypertension and is part of routine clinical care in Brazil. Its long-term effects in patients with schistosomiasis-associated pulmonary arterial hypertension are not well understood.\n\nThe PROPULSE-Sch study aims to evaluate long-term clinical outcomes in patients with schistosomiasis-associated pulmonary arterial hypertension who received selexipag, compared with similar patients who did not receive this medication before it became available at the study center.\n\nThis is an observational study using data from routine medical care. All treatments are prescribed by the treating physicians, and participation in the study does not change patient care. The results may help improve understanding of long-term outcomes and support treatment decisions in this population.",[27],[41,453,454,455,456,457,458],"Schistosomiasis","Prostacyclin Pathway","Real-World Evidence","Propensity Score Matching","Long-Term Outcomes","Observational Study","2026-02-27",{"date":461,"type":46},"2026-03-05",{"date":463,"type":21},"2026-03-01",{"date":465,"type":21},"2027-03-01",{"name":467,"class":140},"Caio Júlio César dos Santos Fernandes",{"id":469,"slug":470,"hasResults":12,"nctId":471,"briefTitle":472,"officialTitle":472,"acronym":473,"eligibilityCriteria":474,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":475,"targetDuration":477,"studyType":332,"phases":4,"briefSummary":478,"conditions":479,"keywords":480,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":483,"lastUpdatePostDateStruct":484,"startDateStruct":486,"completionDateStruct":487,"leadSponsor":489,"locationsCount":4},"100606190","what-is-the-role-of-the-exposome-in-pulmonary-hypertension-100606190","NCT07172334","What is the Role of the Exposome in Pulmonary Hypertension","EXPOSPAH","Inclusion Criteria:\n\n* Consent signed by the participant\n* Age ≥ 18 years\n* Free subject, under no legal protection\n* Good understanding of the French language, allowing to answer the questionnaires\n* Incident patients with pre-capillary PH confirmed by cardiac catheterization (PAPm \\> 20mmHg, PCP ≤ 15 mmHg, RVP \\> 2UW) having had the right diagnostic cardiac catheterization within one year (≤1 year) :\n\n  * Cases: Patients with PAH without associated pathology: idiopathic or heritable, or with features of venous\u002Fcapillary involvement, according to the clinical classification of PH\n  * Controls: Patients with chronic thromboembolic PH (CTE-PTH, class 4), according to the clinical classification of PH.\n\nExclusion Criteria:\n\n* Patients classified as HTP groups 2, 3 or 5\n* Patients with a diagnosis of PAH associated with a connective tissue disease, HIV, portal hypertension, congenital heart disease, bilharzia, or a drug or toxic cause considered certain according to the international classification (Aminorex, Benfluorex, Carfilzomib, Dasatinib, Dexfenfluramine, Fenfluramine, Methamphetamines, Mitomycin C, adulterated rapeseed oil)\n* Patients with signs of right heart failure requiring amines and\u002For intensive care hospitalization at time of visit (may be included at a later visit)\n* Patients refusing hair sampling or having no hair \\> 2 cm to allow sampling\n* Patients benefiting from enhanced protection, i.e. minors, persons deprived of their liberty by a judicial or administrative decision, persons staying in a health or social establishment, adults under legal protection, and patients in emergency situations",{"count":476,"type":21},150,"1 Day","Pulmonary arterial hypertension (PAH) is a rare and incurable disease affecting people of all ages. It is characterized by obstructive remodeling of the small pulmonary arteries, responsible for an increase in pulmonary arterial pressure, leading to right heart failure and death in the absence of treatment. PAH can be associated with a variety of diseases, but around half of all PAH cases are idiopathic or hereditary, and may develop on predisposed terrain following a \"second hit\", as suggested by the identification of PAH cases associated with the use of anorectic drugs, methamphetamine and occupational exposure to organic solvents. No study has systematically analyzed the exposome of patients with PAH, combining environmental and occupational exposures as well as drugs and medications.\n\nThe exposome of patients with PAH without associated causes will be compared with that of patients with another form of pulmonary hypertension (PH), linked to thromboembolic risk factors: chronic thromboembolic PH (CTEPH), which will constitute the control group.",[27,28],[481,482],"Pulmonary hypertension","Exposome","2026-02-12",{"date":485,"type":46},"2026-02-13",{"date":267,"type":21},{"date":488,"type":21},"2028-07",{"name":490,"class":140},"Poitiers University Hospital",{"id":492,"slug":493,"hasResults":12,"nctId":494,"briefTitle":495,"officialTitle":496,"acronym":497,"eligibilityCriteria":498,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":499,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":500,"conditions":501,"keywords":502,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":507,"lastUpdatePostDateStruct":508,"startDateStruct":509,"completionDateStruct":510,"leadSponsor":512,"locationsCount":4},"100620342","patient-reported-outcomes-and-adherence-after-transition-from-inhaled-iloprost-to-oral-selexipag-in-pulmonary-arterial-hypertension-100620342","NCT07356375","Patient-Reported Outcomes and Adherence After Transition From Inhaled Iloprost to Oral Selexipag in Pulmonary Arterial Hypertension","PROMISE: Evaluation of Patient-Reported Outcomes and Adherence After Transition From Inhaled Iloprost to Oral Selexipag in Pulmonary Arterial Hypertension","PROMISE","Inclusion Criteria\n\n* Adults aged 18 years or older.\n* Confirmed diagnosis of pulmonary arterial hypertension (PAH, Group 1) by right heart catheterization.\n* Current treatment with inhaled iloprost as part of routine clinical care.\n* Clinical decision by the treating physician to transition from inhaled iloprost to oral selexipag.\n* Ability to understand and complete patient-reported outcome questionnaires.\n* Provision of written informed consent.\n\nExclusion Criteria\n\n* Significant cognitive impairment or sensory deficits that prevent completion of study questionnaires.\n* Unstable or decompensated comorbid conditions that may interfere with treatment adherence or study participation.\n* Refusal or inability to provide informed consent.",{"count":292,"type":21},"Pulmonary arterial hypertension (PAH) is a rare and serious condition that affects the blood vessels of the lungs and can significantly limit daily activities and quality of life. Some patients with PAH use inhaled iloprost, a medication that requires several inhalations per day, which can be difficult to maintain over time. Oral selexipag is an alternative treatment that may reduce treatment burden and improve adherence.\n\nThe PROMISE study aims to evaluate how switching from inhaled iloprost to oral selexipag affects patients' quality of life, satisfaction with treatment, and adherence in real-world clinical practice. Patient-reported outcome questionnaires will be used to understand patients' perceptions of symptoms, daily functioning, and overall improvement after the transition.\n\nAdult patients with PAH who are receiving inhaled iloprost and whose physicians decide to switch treatment to oral selexipag will be followed over time. A comparison group of patients who continue using inhaled iloprost will also be observed. The study does not involve any experimental treatment or changes to routine clinical care. All medications are prescribed as part of standard medical practice.\n\nThe results of this study may help improve understanding of the patient experience during treatment transitions in PAH and support more patient-centered treatment decisions.",[27],[41,454,503,335,504,455,505,506,458],"Patient-Reported Outcomes","Treatment Adherence","Iloprost","Selexipag","2026-02-10",{"date":485,"type":46},{"date":463,"type":21},{"date":511,"type":21},"2026-12-31",{"name":417,"class":140},{"id":514,"slug":515,"hasResults":12,"nctId":516,"briefTitle":517,"officialTitle":518,"acronym":519,"eligibilityCriteria":520,"healthyVolunteers":12,"sex":17,"minAge":477,"maxAge":521,"enrollmentInfo":522,"targetDuration":4,"studyType":22,"phases":524,"briefSummary":525,"conditions":526,"keywords":4,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":528,"lastUpdatePostDateStruct":529,"startDateStruct":531,"completionDateStruct":533,"leadSponsor":535,"locationsCount":141},"100621243","phase-1-pneumostem-for-improving-respiratory-outcomes-in-very-premature-infants-diagnosed-with-early-pulmonary-arterial-hypertension-100621243","NCT07368088","PNEUMOSTEM® for Improving Respiratory Outcomes in Very Premature Infants Diagnosed With Early Pulmonary Arterial Hypertension","A Clinical Study of Advanced Regenerative Medicine to Evaluate the Safety and Potential Efficacy of PNEUMOSTEM® for Improving Respiratory Outcomes in Very Premature Infants Diagnosed With Early Pulmonary Arterial Hypertension","REVIVE-PH","Inclusion Criteria:\n\n1. Premature infants within 2 weeks of birth with a gestational age of 28 weeks or less or birth weight of less than 1,250g who require continuous invasive mechanical ventilation\n2. When diagnosed with early pulmonary arterial hypertension satisfying condition ① or ② up to 14 days after birth:\n\n   * When on or more of the following abnormal findings are present on echocardiography performed between 4 and 14 days after birth (findings at 1-3 days after birth correspond to early neonatal transition):\n\n     1. Sytemic or suprasystemic pulmonary artery pressure \\>40mmHg(based on peak Doppler velocity of tricuspid regurgitation)\n     2. Right-to-left or bidirectional shunt through patent ductus arteriosus, foramen ovale, or atrial septal defect\n     3. Flattened interventricular septum or D-shaped left ventricle at end systole ② When receiving nitric oxide(NO) inhalation therapy for persistent pulmonary hypertension of the newborn(PPHN) within 3 days after birth\n\nExclusion Criteria:\n\n1. Those witth cyanotic congenital heart defects or acyanotic congenital heart defects causing heart failure, excluding patent ductus arteriosus in premature infatns\n2. Those with severe pulmonary malformations such as congenital diaphragmatic hernia or congenital cystic lung disease\n3. Those who underwent surgery within 72 hours before or after administration of the investigational cell product, or those for whom surgery is anticipated\n4. Those who received surfactant within 24 hours prior to administration of the investigattional cell product\n5. Those with chromosomal abnormalities accompanied by severe malformations(such as Edwards syndrome, Patau syndrome, Down syndrome, etc.) and severe congenital malformations(such as hydrocephalus, encephalocele, etc.)\n6. Those with severe congenital infectious diseases(such as herpes, toxoplasmosis, rubella, syphilis, AIDS, etc.)\n7. Those with severe sepsis or shock due to active infection not adequately treated with antibiotics\n8. Those who have a history of participation in other advanced regenerative medicine clinical studies or clinical trials\n9. Others deemed inappropriate by tthe investigator to participate in this advanced regenerative medicine clinical study","14 Days",{"count":523,"type":21},12,[125],"The goal of this clinical trial is to evaluate the safety and potential efficacy of PNEUMOSTEM® for improving respiratory outcomes in very premature infants diagnosed with Early Pulmonary Arterial Hypertension. The main questions it aims to answer are:\n\n* In very premature infants diagnosed with early pulmonary arterial hypertension, will a single intratracheal administration of PNEUMOSTEM®(Allogeneic umbilical cord blood-derived mesenchymal stem cells) result in improvement of pulmonary arterial hypertension based on echocardiographic assessment?\n* In very premature infants diagnosed with early pulmonary arterial hypertension who show improvement of pulmonary arterial hypertension based on echocardiographic assessment following a single intratracheal administration of PNEUMOSTEM®(Allogeneic umbilical cord blood-derived mesenchymal stem cells), at what time point does this improvement occur?\n\nParticipants will:\n\n* Single intratracheal dose of PNEUMOSTEM® at 2.0 x 10,000,000 cells\u002Fkg\n* Acute adverse event monitoring: 24 hours post-administration for safety assessment\n* Follow- up time points: Day 1(Baseline, PNEUMOSTEM® administration), Day 2, Week 1, Week 2, Postnatal Day 28, PMA 36\\~40 weeks",[27,527],"Premature Infants","2026-01-20",{"date":530,"type":46},"2026-01-26",{"date":532,"type":21},"2026-03",{"date":534,"type":21},"2027-07",{"name":536,"class":140},"Samsung Medical Center",{"id":538,"slug":539,"hasResults":12,"nctId":540,"briefTitle":541,"officialTitle":541,"acronym":542,"eligibilityCriteria":543,"healthyVolunteers":149,"sex":17,"minAge":477,"maxAge":544,"enrollmentInfo":545,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":546,"conditions":547,"keywords":550,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":556,"lastUpdatePostDateStruct":557,"startDateStruct":558,"completionDateStruct":560,"leadSponsor":562,"locationsCount":141},"100617145","genetic-hallmarks-of-patients-with-congenital-portosystemic-shunts-and-portopulmonary-hypertension-100617145","NCT07314814","Genetic Hallmarks of Patients With Congenital Portosystemic Shunts and Portopulmonary Hypertension","Gen-PoPH-CPSS","Inclusion Criteria:\n\n* Patient is a participant to the IRCPSS with history of PoPH\n* Trios composed of CPSS PoPH patients and their parents (trios are mandatory)\n* Brother\u002Fsister of an enrolled patient\n* Trios accept to provide biological samples (blood), sign the inform consent.\n* Siblings and\u002For siblings' legal representatives accept to provide biological samples (blood), sign the inform consent.\n\nExclusion Criteria:\n\n* Trio condition is not met.\n* No genuine parent-offspring trios (check for medically assisted procreation with donors, and adoption)\n* For siblings, half-brothers or half-sisters are excluded, as well as adopted children, or children issued from medically assisted procreation with donors.\n* Secondary portosystemic shunts\n* The refusal by the patient or the patient's legal representatives to provide biological samples or agree with the proposed procedure or after voluntary withdrawal from the project.\n* The refusal of one of the parents to provide biological samples or to agree with the proposed procedure or after voluntary withdrawal from the project.","99 Years",{"count":281,"type":21},"Congenital portosystemic shunt (CPSS) are rare vascular malformations causing blood from the intestines to bypass the liver and directly flow into body's general circulation. Such liver bypass can cause several health problems, one of the most severe being portopulmonary hypertension (PoPH).\n\nThe goal of this study is to identify pathogenic and potentially pathogenic genetic variants in patients who have both CPSS and PoPH. Future research will assess the contribution of these genetic variants to the development of PoPH.\n\nThe long-term goal is to use genetic information to identify patients with congenital portosystemic shunts (CPSS) or chronic liver disease who are at risk of developing PoPH to offer anticipatory management.\n\nChildren and adult patients with both CPSS and PoPH, as well as their close relatives (patient's parents and siblings) can take part in the study. Genetic variations within each family will be studied.",[548,27,549],"Portopulmonary Hypertension","Congenital Portosystemic Shunt",[551,552,239,553,554,555],"CPSS","PoPH","Genetic","Genomic","IRCPSS","2026-01-15",{"date":528,"type":46},{"date":559,"type":21},"2026-02-01",{"date":561,"type":21},"2030-01-31",{"name":563,"class":140},"Prof. Valérie Mc Lin",{"id":565,"slug":566,"hasResults":12,"nctId":567,"briefTitle":568,"officialTitle":569,"acronym":570,"eligibilityCriteria":571,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":572,"enrollmentInfo":573,"targetDuration":4,"studyType":22,"phases":574,"briefSummary":575,"conditions":576,"keywords":577,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":580,"lastUpdatePostDateStruct":581,"startDateStruct":583,"completionDateStruct":584,"leadSponsor":586,"locationsCount":4},"100613432","phase-2-sirius---initial-combination-therapy-with-an-endothelin-receptor-antagonist-a-phosphodiesterase-5-inhibitor-and-sotatercept-in-patients-with-newly-diagnosed-pulmonary-arterial-hypertension-100613432","NCT07266519","SIRIUS - Initial Combination Therapy With an Endothelin Receptor Antagonist, a Phosphodiesterase-5 Inhibitor and Sotatercept in Patients With Newly Diagnosed Pulmonary Arterial Hypertension","SIRIUS - A Prospective, Non-randomized, Open-label, Proof-of-concept Study of Initial Combination Therapy With an Endothelin Receptor Antagonist, a Phosphodiesterase-5 Inhibitor and Sotatercept in Patients With Newly Diagnosed Pulmonary Arterial Hypertension A Single-arm, Multi-center, Interventional Phase II Study","SIRIUS","Inclusion Criteria:\n\n* Age 18- 70 years\n* Treatment-naïve patients diagnosed within 12 weeks prior to screening with I\u002FH\u002FD-PAH or PAH associated with corrected congenital heart disease (disease (≥12 months after correction). Patients who have received treatment with PDE5is and\u002For ERAs for up to 6 weeks after diagnosis are eligible provided that state-of-the-art right heart catheterization has been performed at PAH diagnosis.\n* PVR \\>5 WU, mPAP ≥35 mmHg and PAWP or LVEDP ≤15 mmHg at PAH diagnosis.\n* Signed written informed consent\n* Normal blood counts for platelets and erythrocytes\n* Women of childbearing potential must be willing to remain abstinent or use adequate and reliable contraception throughout the study and for at least 4 months after the last dose of study medication has been received.\n\n  * WOCBP must use one of the following highly effective methods of birth control that result in a low failure rate of less than 1% per year when used consistently and correctly - according to recommendations by the European Heads of Medicines Agencies - from at least 14 days before the first administration of study medication until 4 month after the last administration of study medication:\n  * combined (estrogen and progestogen containing) hor-monal contraception associated with inhibition of ovulation:\n\n    * oral\n    * intravaginal\n    * transdermal\n  * progestogen-only hormonal contraception associatedwith inhibition of ovulation:\n\n    * oral\n    * injectable\n    * implantable\n  * intrauterine device (IUD)\n  * intrauterine hormone-releasing system (IUS)\n  * bilateral tubal occlusion\n  * vasectomized partner\n  * sexual abstinence\n* Male participants with female partner(s) of childbearing potential are eligible to participate in the study if they agree to the following during treatment and until 304 daysmonths after the last administration of study medication:\n\n  * Inform any and all partner(s) of their participation in a clinical drug study and the need to comply with contraception instructions as directed by the investigator.\n  * Male participants are required to use a condom during treatment and until 30 days4 months after the last administration of study medication.\n  * Female partners of male participants who have not undergone a vasectomy with the absence of sperm confirmed or a bilateral orchiectomy should consider use of effective methods of contraception during treatment and until 30 days4 months after the last administration of study medication.\n* Sperm donation is not allowed during treatment and until 4 months after the last administration of study medication.\n\nExclusion Criteria:\n\n* Other Forms of PH or PAH\n* Use of PAH medications for more than 6 weeks prior to screening\n* Symptoms or signs of clinically relevant lung disease, including TLC \\\u003C 70%, FEV1\u002FFVC \\\u003C60%, and DLCO \\\u003C45%, respectively\n* Signs of left heart failure with reduced or preserved ejection fraction, including LVEF \\\u003C50%, LAVI \\>34 ml\u002Fm2, E\u002Fé ≥15, or permanent atrial fibrillation, respectively.\n* History of severe bleeding\u002Fhaemorrhage\n* Therapy with prostacyclin and\u002For antithrombotic agents\n* eGFR \\\u003C30 ml\u002Fmin\u002Fm2.\n* Pregnancy or breastfeeding\n* Hypersensitivity to the active substance or to any of the excipients","70 Years",{"count":7,"type":21},[93],"The study aims to see how 24 weeks of triple therapy-an endothelin receptor antagonist (ERA), a phosphodiesterase-5 inhibitor (PDE5i), and sotatercept-affects pulmonary vascular resistance (PVR) in patients with newly diagnosed pulmonary arterial hypertension (PAH). SIRIUS is a 24-week, single-arm, open-label study with up to 42 days of screening and a 28-day safety follow-up. It will enroll 25 patients and will be conducted only in countries where all treatments are available and covered. After 24 weeks, PAH treatment is decided by the doctor.",[27],[578,239,579],"triple therapy","treatment-naiive","2025-11-24",{"date":582,"type":46},"2025-12-05",{"date":76,"type":21},{"date":585,"type":21},"2028-10",{"name":587,"class":140},"Philipps University Marburg",{"id":589,"slug":590,"hasResults":12,"nctId":591,"briefTitle":592,"officialTitle":593,"acronym":594,"eligibilityCriteria":595,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":596,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":598,"conditions":599,"keywords":600,"overallStatus":42,"whyStopped":4,"lastUpdateSubmitDate":605,"lastUpdatePostDateStruct":606,"startDateStruct":608,"completionDateStruct":610,"leadSponsor":612,"locationsCount":141},"100608897","information-sources-in-pulmonary-hypertension-100608897","NCT07207525","Information Sources in Pulmonary Hypertension","Sources of Information and Search Patterns on Pulmonary Hypertension in Patients With Confirmed Diagnosis and Their Caregivers (inPHorma)","inPHorma","Inclusion Criteria:\n\n* Age ≥ 18 years\n* Confirmed diagnosis of pulmonary hypertension (any clinical group) or informal caregiver of a patient with pulmonary hypertension\n* Ability to read and understand the questionnaire\n* Signed informed consent (ICF\u002FTCLE)\n\nExclusion Criteria:\n\n* Inability to understand or complete the questionnaire\n* Refusal to participate or not signing the informed consent",{"count":597,"type":21},100,"Pulmonary hypertension is a serious disease that affects patients' health, daily life, and emotional well-being. Many patients and their caregivers actively look for information to better understand the condition and its treatment. However, the quality of information found in different sources, such as the internet, social media, health professionals, and patient groups, can vary.\n\nThis study will use a short questionnaire to learn where patients and caregivers search for information, what topics they look for, how satisfied they are with what they find, and which sources they trust most. The results will help improve communication strategies, educational materials, and support programs for people living with pulmonary hypertension and their families.",[27],[377,601,602,603,604],"Patients","Information Needs","Information Sources","Health Information Seeking","2025-09-26",{"date":607,"type":46},"2025-10-06",{"date":609,"type":21},"2025-11",{"date":611,"type":21},"2027-03",{"name":417,"class":140},{"id":614,"slug":615,"hasResults":12,"nctId":616,"briefTitle":617,"officialTitle":618,"acronym":619,"eligibilityCriteria":620,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":621,"targetDuration":623,"studyType":332,"phases":4,"briefSummary":624,"conditions":625,"keywords":626,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":605,"lastUpdatePostDateStruct":633,"startDateStruct":634,"completionDateStruct":636,"leadSponsor":638,"locationsCount":141},"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\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":622,"type":21},121,"6 Months","Pulmonary arterial hypertension (PAH) is a rare, progressive, and potentially fatal disease characterized by increased pulmonary vascular resistance and right ventricular dysfunction. Among the four major molecular pathways involved in PAH pathophysiology-nitric oxide, prostacyclin, activin, and endothelin-1 (ET-1)-the endothelin pathway plays a central role. Endothelin-1 acts on ETA and ETB receptors, inducing vasoconstriction and vascular remodeling.\n\nEndothelin receptor antagonists (ERAs) are cornerstone therapies in PAH. Ambrisentan is selective for ETA and associated with a lower risk of hepatotoxicity. Bosentan, a dual ERA (ETA\u002FETB), has well-established efficacy but a higher incidence of liver enzyme elevation, with approximately 9% of patients experiencing hepatic side effects and about 2% discontinuing therapy due to hepatotoxicity.\n\nWhile transitions between ERAs occur in routine clinical practice, data on their clinical impact are scarce. This prospective, observational, single-center cohort study aims to evaluate the effect of switching from ambrisentan to bosentan on risk stratification using the COMPERA 2.0 and REVEAL Lite 2.0 scores at 3-6 months post-switch.\n\nSecondary outcomes include variations in functional class (WHO\u002FNYHA), 6-minute walk distance (6MWD), NT-proBNP levels, incidence of adverse events (with a focus on hepatotoxicity), and hematologic parameters such as anemia.\n\nThe study will enroll adult patients (≥18 years) with confirmed PAH by right heart catheterization who have undergone a documented switch from ambrisentan 10 mg to bosentan 125 mg within the last 6 months. The primary endpoint is the proportion of patients whose risk category changes post-transition according to COMPERA 2.0 and REVEAL Lite 2.0. The results are expected to provide clinically relevant insights into therapeutic decisions involving ERA transitions in PAH management.",[41,27,236],[41,239,627,628,629,630,631,632],"Endothelin Receptor Antagonists","ERA","Ambrisentan","Bosentan","Drug Switching","Risk Stratification",{"date":217,"type":46},{"date":635,"type":46},"2025-08-20",{"date":637,"type":21},"2026-12-01",{"name":417,"class":140},{"id":640,"slug":641,"hasResults":12,"nctId":642,"briefTitle":643,"officialTitle":644,"acronym":645,"eligibilityCriteria":646,"healthyVolunteers":12,"sex":17,"minAge":18,"maxAge":4,"enrollmentInfo":647,"targetDuration":4,"studyType":332,"phases":4,"briefSummary":649,"conditions":650,"keywords":651,"overallStatus":102,"whyStopped":4,"lastUpdateSubmitDate":605,"lastUpdatePostDateStruct":657,"startDateStruct":659,"completionDateStruct":661,"leadSponsor":663,"locationsCount":141},"100592814","pulmonary-hypertension-diagnosis-a-national-cohort-research-100592814","NCT06998329","PulmonAry hyperteNsion DiagnOsis: a National cohoRt reseArch","PANDORA: PulmonAry hyperteNsion DiagnOsis: a National cohoRt reseArch","PANDORA","Inclusion Criteria:\n\n* Availability of documented or estimated date of first symptom suggestive of PAH.\n* Availability of documented date of confirmed PAH diagnosis.\n\nExclusion Criteria:\n\n* Patients with pulmonary hypertension not classified as Group 1 (e.g., due to left heart disease, chronic lung disease, or chronic thromboembolic disease).\n* Patients with insufficient data to determine either the date of symptom onset or the date of confirmed diagnosis.\n* Patients diagnosed only clinically or by echocardiography, without confirmation by right heart catheterization.",{"count":648,"type":21},139,"Understanding Delays in the Diagnosis of Pulmonary Arterial Hypertension and Rare Diseases in Brazil: A Multicenter Observational Study\n\n\\--- Pulmonary arterial hypertension (PAH) is a rare, progressive, and life-threatening disease that affects the arteries of the lungs and the right side of the heart. Early diagnosis is essential to initiate appropriate treatment and improve patient outcomes. However, worldwide studies show that there is often a significant delay between the onset of symptoms and the final diagnosis. This delay may lead to disease progression and worse survival.\n\nThis multicenter observational study aims to understand the time from the first symptoms to the diagnosis of PAH and other rare diseases across several Brazilian reference centers. By analyzing medical records and patient journeys, the investigators intends to identify factors contributing to delayed diagnosis and potential opportunities for earlier detection.\n\nThe study includes adult patients diagnosed with PAH or other selected rare diseases within the last five years. The investigators will analyze time to diagnosis, number and type of physicians consulted, tests performed, and possible misdiagnoses. Our goal is to support the development of strategies that reduce diagnostic delay and improve access to specialized care for people living with rare diseases.\n\nThis study does not involve any intervention and poses no additional risk to participants, as it is based solely on retrospective data from medical records.",[27,236,41],[377,652,653,654,655,632,656],"Diagnostic Delay","Right Heart Catheterization","Early Diagnosis","Diagnostic Journey","Differential Diagnoses",{"date":658,"type":46},"2025-09-30",{"date":660,"type":46},"2025-07-07",{"date":662,"type":21},"2027-12",{"name":417,"class":140}]