View clinical trials related to Fibromuscular Dysplasia.
Filter by:PROFILE is a cohort study evaluating the progression of fibromuscular dysplasia lesions. This study is the prospective dimension of ARCADIA registry (ClinicalTrials.gov Identifier: NCT02884141), which aims to document phenotypic and genetic traits in patients with renal and/or cervical artery fibromuscular dysplasia.
ARCADIA is a national registry designed to document phenotypic and genetic traits in patients with renal and/or cervical artery fibromuscular dysplasia (FMD). FMD is a group of arterial diseases that most commonly involve renal and carotid arteries. Patients with FMD may present with renovascular hypertension and/or with cerebrovascular symptoms. Angiographic classification includes the multifocal type and the focal type. FMD may affect one or more vascular beds and progress to more severe stenosis and to renal or cerebrovascular complications. FMD may be familial (OMIM #135580). Our main objective is to create a FMD registry that will collect standardized information from all consenting patients diagnosed with the condition in 16 participating centers. This registry, along with a collection of leukocyte DNA, will constitute a resource for further clinical research on FMD. The first application will be the assessment of the frequency of multi-site FMD, i.e. the frequency of cervical artery FMD in patients presenting with renal artery FMD and vice-versa. The second application will be a case-control study to identify susceptibility genes for FMD. Patients are eligible in the registry if: (a) they have renal or cervical artery FMD with either multifocal or focal lesions at CT-angiography, MR-angiography, or intra-arterial angiography; (b) they give informed consent to leukocyte DNA analysis and to the collection of bioclinical and morphologic information. Phenotypic assessment will be performed in accordance with current recommendations and best clinical practice. Given the multicenter nature of the study and the recruitment capacity of each centre, enrollment of 500 FMD cases is expected over 5 years. This number will 1) allow an accurate estimation of the frequency of multi-site FMD: when the sample size is 500, a two-sided 95% confidence interval will extend 0.035 from the observed proportion for an expected proportion of 0.20 based on a previous report and from our unpublished data. 2) In addition to a collection of 400 renal FMD already collected at HEGP, give sufficient power for a genome-wide association study seeking for susceptibility genes
Spontaneous Coronary Artery Dissection (SCAD) is a rare and often misdiagnosed cause of Acute Coronary Syndrome (ACS) affecting predominantly young women without cardiovascular risk factors. The origin of SCAD remains uncertain but a strong and frequent association with Fibromuscular Dysplasia (FMD) has been recently reported based on imaging evidence only. The aim of our study is to assess the presence of FMD and its genetic determinants i in a sample for haematoma or spontaneous coronary artery dissection. From May 2016 to 2018 we plan to include prospectively and retrospectively 200 patients admitted for ACS with confirmed diagnosis of SCAD. This study will be conducted in more than 30 French interventional cardiology centers. Coronary angiograms or intracoronary imaging data will be reviewed by two experienced interventional cardiologist experts in SCAD diagnosis. For each patient a genetic analysis will be performed. A systematic screening for FMD will be realized by computed tomographic or MRI angiography of renal, cerebrovascular and iliac arteries and reviewed by two experienced radiologists. A one year follow-up is expected. This study aims to confirm the presumed association of FMD and SCAD through the exploration of several artery beds and the study of confirmed genetic determinants, which has never been described previously to our knowledge.
This is a prospective multicenter study based on the validation of diagnostic criteria and predictive factors of treatment efficacy for renal artery stenosis in renal artery dysplasia. This is considered as a rare disease and patients are usually treated in specialized centers involved in a national network. Marseille is a center specialized in FMD and a member of the network that is very active across the country. In order to rapidly recruit patients the investigators propose a multicenter study. All patients included will benefit from an invasive angiography with trans stenotic gradient assessment at rest. In case of bilateral stenosis the investigators will randomly omit data from one side and consider the data from the contralateral artery for further analysis, to avoid statistical interdependency. Patients who require angioplasty and who have unilateral stenosis (unilateral FMD lesion or bilateral but with one non significant stenosis based on duplex ultrasound European recommendations for atherosclerotic renal artery stenosis) will be included in the second part of the study. These patients will in addition benefit from a trans stenotic pressure assessment under vasodilation and intravascular renal artery ultrasound. Patients with severe bilateral renal artery stenosis will not be included in the analysis to assess predictive criteria for treatment efficacy on hypertension, treatment of these patients will followed the current best clinical practice. These patients will be followed during 6 to 8 month to assess potential complication of the pressure assessment renal artery stenosis, but their data will be included to assess the value of FFR and IVUS to guide the procedure. Patients with unilateral stenosis will be followed up 6 month after angioplasty in order to assess hypertension and to look for potential complications.
Fibromuscular dysplasia (FMD) is localized structural defects in the arterial wall, whose innate or acquired character is still unknown. This segmental non atheromatous injury, leads to stenosis of the arteries of small and medium caliber. Renal arteries are the most commonly affected with 60-75% of total fibrodysplasia. Three histological subtypes have been described: intimal, medial and peri-medial. They are not mutually exclusive and can be observed in the same patient. This is a rare blood disease, occurring in children and young adults. In this young population with long life expectancy, these aneurysmal lesion are associated with 10% risk of rupture. To date, no data have shown in the literature that FMD is link to genetic causes, or if there is specific histopathologic lesions for non-atherosclerotic renal artery aneurysms. To answer these questions, Cardiovascular Surgery Unit of the University Hospital of Saint-Etienne, French national reference center for renal artery surgery, in association with the Reference Center for Rare Vascular Disease in Paris, designed the first study for pathological and genetic characteristics of dysplastic renal artery aneurysms in young patients.
Fibromuscular dysplasia is an non inflammatory non atherosclerotic obstructive arterial disease affecting mid-size arteries. It is considered as a rare vascular disease of unknown origin. Fibromuscular dysplasia may become symptomatic depending on location and severity of narrowing of the arterial lumen. for example,when a stenosis develops within a renal artery, arterial hypertension may develop. The cause of fibromuscular dysplasia is unknown. Several factors have been suggested to be associated with it: tobacco abuse or oestrogens. In order to progress into identifying possible causative mechanisms of the disease, we design a pathophysiology study destined to assess endothelial function in patients with fibromuscular dysplasia and to identify possible plasmatic biomarkers of the disease.
The purpose of this project is to see if heritable alterations in the function, biology and vascular repair capacity of vascular cells make a major contribution to the burden of coronary artery disease (CAD), fibromuscular dysplasia (FMD), and other vascular diseases. In more detail, FMD is a nonatherosclerotic vascular disease that primarily affects women aged 20 to 60. It commonly affects the renal and carotid arteries but may involve almost every artery in the body. At the cellular level, FMD is characterized by increased fibroblast proliferation and collagen deposition. This study aims to define some of these cellular problems by directly studying fibroblast cells from FMD patients and healthy control subjects. Similarly, CAD is among the leading causes of death worldwide. However, a large part of the risk for CAD is unexplained. It is thought that a major but undefined risk factor may be gene (genomic) variations causing a change in vascular cell function. Here, we will study important vascular cell types in patients with severe and early onset CAD in an attempt to define these problems. Therefore, in summary, this study will look to define the various cellular-level problems that occur in patients with both in CAD and FMD. These data will be linked to DNA-level analyses to ultimately attempt to define the cause of these conditions.