[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"idiopathic-pulmonary-arterial-hypertension\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:idiopathic-pulmonary-arterial-hypertension":24},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,4,0,[8,40,65,92],{"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":4,"overallStatus":27,"whyStopped":4,"lastUpdateSubmitDate":28,"lastUpdatePostDateStruct":29,"startDateStruct":32,"completionDateStruct":34,"leadSponsor":36,"locationsCount":39},"100474797","right-ventricle-lipid-in-pulmonary-arterial-hypertension-pah-100474797",false,"NCT05462574","Right Ventricle Lipid in Pulmonary Arterial Hypertension (PAH)","Clinical and Mechanistic Understanding of Right Ventricular Steatosis in Pulmonary Arterial Hypertension (PAH)","Inclusion criteria:\n\n* ≥ 18 years old\n* Diagnosed with idiopathic, heritable, connective tissue disease-associated PAH, associated pulmonary arterial hypertension (PAH), or drug-or toxin-associated PAH according to World Health Organization (WHO) consensus recommendations.\n* Stable PAH-specific medication regimen for three months prior to enrollment. Adjustments in IV prostacyclin for side effect management are allowed. Diuretic adjustments are permitted.\n* WHO Functional Class I-III\n* Ambulatory\n* Able to have an MRI\u002FMRS, perform a 6MWD test, and cardiopulmonary exercise test\n\nExclusion criteria:\n\n* Pregnancy\n* Diagnosis of PAH etiology other than idiopathic, heritable, connective tissue disease - associated PAH or associated with drugs and toxins\n* WHO Functional class IV heart failure\n* Requirement for continuous oxygen\n* Unable to have an MRI\u002FMRS, perform a 6MWD test, or cardiopulmonary exercise test.\n* Patients with implanted\u002Fembedded ferromagnetic material that would preclude cardiac MRI","ALL","18 Years",{"count":19,"type":20},75,"ESTIMATED","OBSERVATIONAL","The investigators propose to study the relationship between right ventricle (RV) steatosis and RV function, exercise capacity, and outcomes in humans with pulmonary arterial hypertension (PAH) and to identify potential drivers of lipid accumulation.",[24,25,26],"Idiopathic Pulmonary Arterial Hypertension","Heritable Pulmonary Arterial Hypertension","Pulmonary Arterial Hypertension Associated With Connective Tissue Disease","RECRUITING","2026-05-26",{"date":30,"type":31},"2026-05-29","ACTUAL",{"date":33,"type":31},"2023-01-17",{"date":35,"type":20},"2027-09-30",{"name":37,"class":38},"Vanderbilt University Medical Center","OTHER",1,{"id":41,"slug":42,"hasResults":11,"nctId":43,"briefTitle":44,"officialTitle":44,"acronym":45,"eligibilityCriteria":46,"healthyVolunteers":11,"sex":16,"minAge":47,"maxAge":48,"enrollmentInfo":49,"targetDuration":4,"studyType":21,"phases":4,"briefSummary":51,"conditions":52,"keywords":55,"overallStatus":27,"whyStopped":4,"lastUpdateSubmitDate":57,"lastUpdatePostDateStruct":58,"startDateStruct":60,"completionDateStruct":62,"leadSponsor":64,"locationsCount":39},"100484183","risk-and-resilience-in-pulmonary-arterial-hypertension-and-genetically-susceptible-individuals-100484183","NCT05584722","Risk and Resilience in Pulmonary Arterial Hypertension and Genetically Susceptible Individuals","RARE-PAH","Inclusion Criteria:\n\n* Children and Adults, aged 15 - 80\n* Diagnosed with idiopathic or heritable, pulmonary arterial hypertension (PAH), defined according to standard criteria\n* Unaffected Mutation Carriers: Healthy participants with a known BMPR2 gene mutation and normal pulmonary pressure and RV function on echo\n* Healthy Controls: Healthy individuals without cardiopulmonary disease.\n* WHO functional class I-III\n* Stable PAH-specific medication regimen for three months prior to enrollment. Subjects with only a single diuretic adjustment in the prior three months will be included. Adjustments in IV prostacyclin for side effect management are allowed.\n\nExclusion Criteria:\n\n* Prohibited from normal activity due to wheelchair bound status, bed bound status, reliance on a cane\u002Fwalker, activity-limiting angina, activity-limiting osteoarthritis, or other condition that limits activity.\n* Pregnancy\n* Diagnosis of PAH etiology other than idiopathic, heritable\n* Functional class IV heart failure\n* Requirement of \\> 2 diuretic adjustment in the prior three months.","15 Years","80 Years",{"count":50,"type":20},150,"Pulmonary arterial hypertension (PAH) is a severe disease with a delayed diagnosis and markedly elevated mortality. High-risk populations, such as those with known genetic defects, provide a unique opportunity to determine the features of susceptibility and resilience to PAH. This proposal will fundamentally overturn the prevailing understanding of PAH by creating molecularly-driven signatures of susceptibility and resilience, provide novel insight into disease severity, and potentially identify new therapeutic targets.\n\nFunding Source - FDA OOPD",[24,25,53,54],"Unaffected Mutation Carriers: Healthy Participants With a Known BMPR2 Gene Mutation and Normal Pulmonary Pressure and RV Function on Echo","Healthy Individuals With no Cardiopulmonary Disease",[56],"pulmonary hypertension","2026-03-09",{"date":59,"type":31},"2026-03-10",{"date":61,"type":31},"2022-11-01",{"date":63,"type":20},"2026-08-31",{"name":37,"class":38},{"id":66,"slug":67,"hasResults":11,"nctId":68,"briefTitle":69,"officialTitle":69,"acronym":70,"eligibilityCriteria":71,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":72,"enrollmentInfo":73,"targetDuration":4,"studyType":75,"phases":76,"briefSummary":78,"conditions":79,"keywords":4,"overallStatus":27,"whyStopped":4,"lastUpdateSubmitDate":83,"lastUpdatePostDateStruct":84,"startDateStruct":86,"completionDateStruct":88,"leadSponsor":90,"locationsCount":39},"100524093","phase-2-129-xenon-mri-as-a-biomarker-for-diagnosis-and-response-to-therapy-in-pulmonary-arterial-hypertension-pah-100524093","NCT06104228","129 Xenon MRI as a Biomarker for Diagnosis and Response to Therapy in Pulmonary Arterial Hypertension (PAH)","Xenon PAH Bio","Inclusion Criteria:\n\nArm 1 -IPAH\n\n* Age: 18-75 years\n* WHO functional class 2 or 3\n* Mean pulmonary artery pressures \\> 20 mmHg\n* Pulmonary capillary wedge pressure ≤15 mmHg\n* Pulmonary vascular resistance \\> 2 Wood Units (WU)\n* No other cause identified for PAH\n\nArm 2 -PAH-CTD\n\n* Age: 18-75 years\n* WHO functional class (FC) 2 or 3\n* Mean pulmonary artery pressures \\> 20 mmHg\n* Pulmonary capillary wedge pressure ≤15 mmHg\n* Pulmonary vascular resistance \\> 2 WU\n* Diagnosis of connective tissue disease\n\nExclusion Criteria:\n\n* PH other than Idiopathic PAH or PAH associated with CTD; any conditions that prevent the performance of 129Xe MRI scans will be excluded from the study.","75 Years",{"count":74,"type":20},20,"INTERVENTIONAL",[77],"PHASE2","The overall study objectives outlined in this study are to derive 129Xe MRI pulmonary vascular biomarker signatures that differentiate common subtypes of PAH and to determine the ability of 129Xe MRI to longitudinally monitor disease progression and response to therapy in PAH, with the aid of additional assessments, such as labs, echocardiography, and six-minute walk distance (6MWD).",[80,24,81,82],"Pulmonary Arterial Hypertension","Pulmonary Arterial Hypertension Associated With Connective Tissue Disease (Disorder)","Connective Tissue Diseases","2026-02-23",{"date":85,"type":31},"2026-02-24",{"date":87,"type":31},"2024-08-12",{"date":89,"type":20},"2027-03-31",{"name":91,"class":38},"Bastiaan Driehuys",{"id":93,"slug":94,"hasResults":11,"nctId":95,"briefTitle":96,"officialTitle":97,"acronym":4,"eligibilityCriteria":98,"healthyVolunteers":99,"sex":16,"minAge":4,"maxAge":100,"enrollmentInfo":101,"targetDuration":4,"studyType":21,"phases":4,"briefSummary":103,"conditions":104,"keywords":107,"overallStatus":27,"whyStopped":4,"lastUpdateSubmitDate":109,"lastUpdatePostDateStruct":110,"startDateStruct":112,"completionDateStruct":114,"leadSponsor":116,"locationsCount":39},"100200324","hormonal-metabolic-and-signaling-interactions-in-pah-100200324","NCT01884051","Hormonal, Metabolic, and Signaling Interactions in PAH","Hormonal, Metabolic, and Signaling Interaction in Pulmonary Arterial Hypertension","Inclusion Criteria:\n\nProject 1\n\nInclusion:\n\n1. Diagnosis of IPAH (idiopathic pulmonary arterial hypertension), HPAH (heritable pulmonary arterial hypertension), or APAH (associated pulmonary arterial hypertension), family members of affected persons\n2. Age 0-90, age 12-90 for skin biopsy\n\nExclusion:\n\n1. Other diagnosis\n2. Age greater than 90, age less than 12 or greater than 90 for skin biopsy\n\nProject 2\n\nInclusion:\n\n1. Diagnosis of IPAH, HPAH, or APAH, family members of affected persons\n2. 0-90\n3. Subjects with reasonably easy access to clinic for blood collection and other testing\n4. Subject able to tolerate fasting state prior to sample collection and EndoPAT (endothelial function assessment) testing\n\nExclusion:\n\n1. Other diagnosis\n2. 0-90\n3. Subjects with difficulty reaching clinic for blood collection and other testing\n4. Subjects unable to tolerate fasting state\n\nProject 3\n\nInclusion:\n\n1. Diagnosis of IPAH, HPAH, or APAH, family members of affected persons\n2. 7-90\n\nExclusion:\n\n1. Other diagnosis\n2. Age less than 7 or greater than 90\n\n   \\-\n\n   Exclusion Criteria:\n\n   \\-",true,"90 Years",{"count":102,"type":20},1899,"Our hypothesis is that optimal treatment of the dysfunctional metabolic pathways which underlie PAH will improve pulmonary vascular function and consequences of the disease.",[24,25,105,106],"Scleroderma Associated Pulmonary Arterial Hypertension","Appetite Suppressant Associate PAH",[108],"Pulmonary arterial hypertension","2025-09-15",{"date":111,"type":31},"2025-09-16",{"date":113,"type":4},"2012-09",{"date":115,"type":20},"2032-07",{"name":37,"class":38}]