[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"pulmonary-arterial-hypertension-pah-who-group-1-ph\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:pulmonary-arterial-hypertension-pah-who-group-1-ph":24},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,7,0,[8,44,75,104,135,161,187],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":18,"targetDuration":4,"studyType":21,"phases":4,"briefSummary":22,"conditions":23,"keywords":27,"overallStatus":31,"whyStopped":4,"lastUpdateSubmitDate":32,"lastUpdatePostDateStruct":33,"startDateStruct":36,"completionDateStruct":38,"leadSponsor":40,"locationsCount":43},"100651912","nailfold-capillaroscopy-in-ph-100651912",false,"NCT07768644","Nailfold Capillaroscopy in PH","Nailfold Capillaroscopy in PH: Are Nailfold Changes Reversible With Pulmonary Vasodilator Therapy?","Inclusion Criteria:\n\nFor inpatient enrolment:\n\n* Suspected diagnosis of group 1,3 or 4 pulmonary hypertension undergoing inpatient assessment at nationally designated centre\n* Age \\> 18 years\n\nFor follow up scanning of those enrolled as an inpatient:\n\n* Confirmed diagnosis of pulmonary hypertension assessed at nationally designated centre fulfilling criteria for group 1, 3 or 4 disease\n* Commencing PH targeted therapy, specifically one or more of: a phosphodiesterase 5 inhibitor (PDE5i), an ET receptor antagonist (ERA), parenteral prostanoid therapy, selexipag, riociguat, sotatercept.\n\nFor outpatient enrolment\n\n* Confirmed diagnosis of pulmonary hypertension assessed at nationally designated centre fulfilling criteria for group 1, 3 or 4 disease\n* Age \\> 18 years\n* Escalating PH targeted therapy, specifically one or more of: a phosphodiesterase 5 inhibitor (PDE5i), an ET receptor antagonist (ERA), parenteral prostanoid therapy, selexipag, riociguat, sotatercept.\n\nTarget of \\> 25% of patients recruited at diagnosis and to have an SSc-spectrum disorder fulfilling either ACR\u002FEULAR 2013 or VEDOSS (very early diagnosis of SSc) criteria.\n\nTarget of \\> 25% of patients recruited at diagnosis and to have non-SSc group 1 (PAH) diagnosis (as determined by national MDT decision).\n\nExclusion Criteria:\n\n* Patients with confirmed group 2 or group 5 pulmonary hypertension\n* Patients \\\u003C 18 years of age\n* Patients unable to participate in nailbed microscopy (through e.g. amputation, severe contractures or other musculoskeletal issues).\n* (From follow up) Patients not being commenced on pulmonary vasodilator therapy at the point of diagnosis.\n* Patients already on maximal PH therapy\n* Patients lacking capacity to consent to participation\n* Patients who are non-fluent English speakers, where suitable translation cannot be found within a time frame that makes the study feasible.\n* Patients with a life expectancy \\\u003C 4 months\n* Patients who have consumed nicotine or caffeine within the last 4 - 6 hours\n* Patients wearing nail polish \u002F varnish\u002F extensions that preclude adequate nailbed visualisation.","ALL","18 Years",{"count":19,"type":20},60,"ESTIMATED","OBSERVATIONAL","Pulmonary hypertension (PH) is an uncommon condition and can often be difficult to diagnose. It means elevated blood pressure in the lungs which can rapidly lead to failure of the right side of the heart and is ultimately fatal. Diagnosing PH currently involves a number of scans, breathing tests and often an invasive test called right heart catheterisation \\[RHC\\] (which involves inserting a tube into a large vein to measure the lung blood pressure from the).\n\nThe investigators propose using a scan of the nailbeds called \"Nailfold (Video) Capillaroscopy\" to see whether this measurements correspond to the pressures that the investigators measure at the time of RHC and whether these measures change with treatment for PH.\n\nThe investigators wish to answer the hypotheses that:\n\n1. The measurements of the nailbed blood vessels are correlated with the invasive right heart catheter values across all groups of PH\n2. The abnormalities seen in systemic sclerosis are reversible with treatment with pulmonary vasodilator drugs\n3. This change will be significantly more than any change seen in other types of pulmonary hypertension with these drugs\n4. Changes in the nailbed blood vessels with treatment will reflect changes in the usual parameters used to measure treatment response in PH:\n\nThe scan uses a camera microscope connected to a laptop, does not involve radiation and can be completed within 30 minutes. The results are sent to the University of Manchester where they are analysed by computer software.\n\nIf patients are found to have pulmonary hypertension and are started on medication (pulmonary vasodilators) to open up the blood vessels of the lungs, the investigators will then repeat the scan at this point. The investigators will compare the results between different forms of PH before and after treatment.",[24,25,26],"Pulmonary Arterial Hypertension (PAH) (WHO Group 1 PH)","Pulmonary Hypertension (World Health Organization Group 3)","Chronic Thromboembolic Pulmonary Hypertension",[28,29,30],"Nailfold Videocapillaroscopy","Pulmonary vasodilator","Pulmonary Hypertension","RECRUITING","2026-08-11",{"date":34,"type":35},"2026-08-17","ACTUAL",{"date":37,"type":35},"2026-05-20",{"date":39,"type":20},"2028-08-02",{"name":41,"class":42},"University of Glasgow","OTHER",1,{"id":45,"slug":46,"hasResults":11,"nctId":47,"briefTitle":48,"officialTitle":49,"acronym":50,"eligibilityCriteria":51,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":52,"targetDuration":4,"studyType":21,"phases":4,"briefSummary":54,"conditions":55,"keywords":58,"overallStatus":31,"whyStopped":4,"lastUpdateSubmitDate":66,"lastUpdatePostDateStruct":67,"startDateStruct":69,"completionDateStruct":71,"leadSponsor":73,"locationsCount":43},"100593954","the-impact-of-era-switching-on-risk-stratification-in-pulmonary-arterial-hypertension-100593954","NCT07013149","The Impact of ERA Switching on Risk Stratification in Pulmonary Arterial Hypertension","ACTION - The Impact of ERA Switching on Risk Stratification in Pulmonary Arterial Hypertension","ACTION","Inclusion Criteria:\n\n* Age ≥ 18 years\n* Confirmed diagnosis of pulmonary arterial hypertension (PAH) by right heart catheterization\n* Documented therapeutic switch from ambrisentan (10 mg once daily) to bosentan (125 mg twice daily) within the previous 6 months for the switch group\n* Treatment with ambrisentan for at least 6 months without switching to bosentan for the maintenance group\n\nExclusion Criteria:\n\n* History of severe hepatic impairment\n* Incomplete clinical or laboratory records that prevent risk score calculation\n* Inability to attend clinical follow-up between 3 and 6 months after medication switch",{"count":53,"type":20},183,"Pulmonary arterial hypertension (PAH) is a rare, progressive, and potentially life-threatening disease characterized by pulmonary vascular remodeling, increased pulmonary vascular resistance, and right ventricular dysfunction. The endothelin pathway plays a central role in its pathophysiology and is targeted by endothelin receptor antagonists (ERAs), including ambrisentan and bosentan.\n\nAmbrisentan is a selective ETA receptor antagonist, whereas bosentan blocks both ETA and ETB receptors. Although transitions between ERAs occur in clinical practice, evidence regarding the clinical impact of switching from ambrisentan to bosentan remains limited.\n\nACTION is a retrospective, observational, single-center cohort study evaluating adult patients with pulmonary arterial hypertension (World Health Organization Group 1) and\u002For chronic thromboembolic pulmonary hypertension (World Health Organization Group 4) confirmed by right heart catheterization. Patients who switched from ambrisentan to bosentan because of a national ambrisentan shortage will be compared with clinically similar patients who remained on ambrisentan.\n\nClinical, functional, and laboratory data recorded at baseline and at 3 to 6 months of follow-up will be assessed. The primary outcome is the proportion of patients with worsening risk stratification after switching from ambrisentan to bosentan compared with patients who continued ambrisentan. Risk will be evaluated using the COMPERA 2.0 and REVEAL Lite 2 assessment tools.\n\nSecondary outcomes include changes in World Health Organization\u002FNew York Heart Association functional class, 6-minute walk distance, BNP levels, individual risk-assessment components, hepatic enzymes, hemoglobin levels, and clinically relevant events such as hospitalization, emergency department visits, initiation of supplemental oxygen, and right heart failure decompensation.",[56,57,24],"Pulmonary Arterial Hypertension","Pulmonary Arterial Hypertension (PAH)",[56,59,60,61,62,63,64,65],"PAH","Endothelin Receptor Antagonists","ERA","Ambrisentan","Bosentan","Drug Switching","Risk Stratification","2026-07-01",{"date":68,"type":35},"2026-07-06",{"date":70,"type":35},"2025-08-20",{"date":72,"type":20},"2026-12-01",{"name":74,"class":42},"University of Sao Paulo General Hospital",{"id":76,"slug":77,"hasResults":11,"nctId":78,"briefTitle":79,"officialTitle":80,"acronym":81,"eligibilityCriteria":82,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":83,"enrollmentInfo":84,"targetDuration":4,"studyType":86,"phases":87,"briefSummary":89,"conditions":90,"keywords":93,"overallStatus":94,"whyStopped":4,"lastUpdateSubmitDate":95,"lastUpdatePostDateStruct":96,"startDateStruct":98,"completionDateStruct":99,"leadSponsor":101,"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","75 Years",{"count":85,"type":20},300,"INTERVENTIONAL",[88],"PHASE3","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.",[56,57,24,91,92],"Pulmonary Arterial Hypertension PAH","Pulmonary Arterial Hypertension WHO Group I",[59,56],"NOT_YET_RECRUITING","2026-05-22",{"date":97,"type":35},"2026-05-29",{"date":97,"type":20},{"date":100,"type":20},"2029-11-30",{"name":102,"class":103},"VIVUS LLC","INDUSTRY",{"id":105,"slug":106,"hasResults":11,"nctId":107,"briefTitle":108,"officialTitle":109,"acronym":110,"eligibilityCriteria":111,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":112,"targetDuration":4,"studyType":86,"phases":114,"briefSummary":116,"conditions":117,"keywords":118,"overallStatus":31,"whyStopped":4,"lastUpdateSubmitDate":127,"lastUpdatePostDateStruct":128,"startDateStruct":130,"completionDateStruct":132,"leadSponsor":134,"locationsCount":43},"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":113,"type":20},215,[115],"NA","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.",[57,24,30],[30,119,120,121,122,123,124,125,126],"Patient Knowledge","Questionnaire Validation","Content Validity","Semantic Analysis","Psychometric Validation","Health Education","Patient Education","Chronic Disease","2026-03-07",{"date":129,"type":35},"2026-03-10",{"date":131,"type":35},"2025-09-01",{"date":133,"type":20},"2026-12",{"name":74,"class":42},{"id":136,"slug":137,"hasResults":11,"nctId":138,"briefTitle":139,"officialTitle":139,"acronym":140,"eligibilityCriteria":141,"healthyVolunteers":142,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":143,"targetDuration":4,"studyType":21,"phases":4,"briefSummary":145,"conditions":146,"keywords":4,"overallStatus":31,"whyStopped":4,"lastUpdateSubmitDate":152,"lastUpdatePostDateStruct":153,"startDateStruct":155,"completionDateStruct":157,"leadSponsor":159,"locationsCount":43},"100609664","rutgers-university-study-of-the-genetics-of-pulmonary-hypertension-100609664","NCT07217522","Rutgers University Study of the Genetics of Pulmonary Hypertension","RUGCC-PH","Inclusion Criteria:\n\n* age 18 years or older\n* currently living in the United States\n* able to understand and follow written instructions in English\n* have access to the internet and a computer, laptop, tablet or smart phone\n* willing to provide written informed consent for participation\n* willing to provide DNA via a saliva sample using a collection kit mailed to the study participant's home\n* willing to complete a survey with questions about health related to the study of pulmonary hypertension.\n\nExclusion Criteria:\n\n* Age 17 or below\n* Not currently living in the United States\n* Not able to participate in an online research study in English",true,{"count":144,"type":20},10000,"The goal of this observational study is to learn more about how genes impact the risk of pulmonary hypertension. Anyone 18 or older living in the US is eligible, and a diagnosis of PH is NOT required. Study participation is online, and it takes about 20 minutes to complete health surveys and request a saliva collection kit sent through US mail. In return, study participants may opt to receive information about their genetic ancestry at no cost.",[30,56,24,147,148,149,150,151],"Pulmonary Arterial Hypertension Associated With Connective Tissue Disease","Pulmonary Arterial Hypertension Associated With Connective Tissue Disease (Disorder)","Pulmonary Arterial Hypertension Associated With Schistosomiasis (Disorder)","Pulmonary Arterial Hypertension of Congenital Heart Disease","Pulmonary Arterial and Chronic Thromboembolic Pulmonary Hypertension","2025-10-30",{"date":154,"type":35},"2025-11-03",{"date":156,"type":35},"2025-08-23",{"date":158,"type":20},"2027-12-31",{"name":160,"class":42},"Rutgers, The State University of New Jersey",{"id":162,"slug":163,"hasResults":11,"nctId":164,"briefTitle":165,"officialTitle":166,"acronym":167,"eligibilityCriteria":168,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":169,"targetDuration":4,"studyType":21,"phases":4,"briefSummary":171,"conditions":172,"keywords":173,"overallStatus":31,"whyStopped":4,"lastUpdateSubmitDate":179,"lastUpdatePostDateStruct":180,"startDateStruct":182,"completionDateStruct":184,"leadSponsor":186,"locationsCount":43},"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":170,"type":20},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.",[57,24,56],[30,174,175,176,177,65,178],"Diagnostic Delay","Right Heart Catheterization","Early Diagnosis","Diagnostic Journey","Differential Diagnoses","2025-09-26",{"date":181,"type":35},"2025-09-30",{"date":183,"type":35},"2025-07-07",{"date":185,"type":20},"2027-12",{"name":74,"class":42},{"id":188,"slug":189,"hasResults":11,"nctId":190,"briefTitle":191,"officialTitle":192,"acronym":193,"eligibilityCriteria":194,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":195,"targetDuration":4,"studyType":86,"phases":197,"briefSummary":198,"conditions":199,"keywords":200,"overallStatus":31,"whyStopped":4,"lastUpdateSubmitDate":214,"lastUpdatePostDateStruct":215,"startDateStruct":217,"completionDateStruct":219,"leadSponsor":221,"locationsCount":43},"100581475","improving-adherence-to-pulmonary-arterial-hypertension-treatment-with-telemedicine-and-patient-guidance-100581475","NCT06850792","imprOving Adherence to Pulmonary artErial hyperteNsion Treatment With teLemedicIne and patieNt guidaNce","imprOving Adherence to Pulmonary artErial hyperteNsion Treatment With teLemedicIne and patieNt guidaNce - A Multicenter Pre- and Post-intervention Evaluation Study","OPENLINE","Inclusion Criteria:\n\n* Adults aged ≥18 years diagnosed with pulmonary arterial hypertension (PAH) (Group 1 of the WHO classification).\n* Stable oral PAH therapy (including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclin pathway agents) for at least four weeks prior to enrollment.\n* Ability to participate in remote teleconsultations (access to a phone or internet).\n* Signed informed consent agreeing to study participation.\n\nExclusion Criteria:\n\n* Severe cognitive impairment or psychiatric disorders that could affect adherence or study participation.\n* Inability to communicate via phone or telemedicine due to technical or personal constraints.\n* Concurrent participation in another interventional clinical trial that could interfere with outcomes.\n* Life expectancy \\\u003C6 months due to any condition unrelated to PAH.\n* Pregnancy or breastfeeding.",{"count":196,"type":20},61,[115],"Pulmonary arterial hypertension (PAH) is a progressive condition with high morbidity, frequent hospitalizations, and risk of right heart failure. Despite advances in treatment, poor adherence remains a major challenge. This randomized controlled study assesses whether remote monitoring can improve treatment adherence, clinical outcomes, and side effect management in PAH patients on oral therapy.",[24,57,56],[57,201,202,125,203,204,205,206,207,208,209,210,211,212,213],"Medication Adherence","Telemedicine","Remote Monitoring","Digital Health Intervention","Randomized Controlled Trial (RCT)","Multicenter Study","COMPERA 2.0","REVEAL Lite","Six-Minute Walk Test (6MWT)","Quality of Life","CAMPHOR Questionnaire","Martín-Bayarre-Grau (MBG) Scale","Oral Therapy for PAH","2025-06-05",{"date":216,"type":35},"2025-06-10",{"date":218,"type":35},"2025-05-07",{"date":220,"type":20},"2028-04",{"name":74,"class":42}]