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INTRODUCTION: Although renal stenting is the standard revascularization method for atherosclerotic renal artery stenosis (RAS) (FMD-RAS), stenting in fibromuscular dysplasia (FMD) RAS is usually limited to periprocedural complications of angioplasty and primary arterial dissection. The main aim of the study was to retrospectively analyze the immediate and long-term results of renal stenting versus angioplasty in patients with FMD. METHODS: Of 343 patients in the ARCADIA-POL registry, 58 patients underwent percutaneous treatment due to FMD-RAS (in 70 arteries). Percutaneous transluminal renal angioplasty (PTRA) was performed as an initial treatment in 61 arteries (PTRA-group), whereas primary stenting was undertaken in nine arteries (stent-group). Stent-related complications were defined as: in-stent restenosis > 50% (ISR); stent fracture; under-expansion; or migration. RESULTS: In the PTRA-group, the initial restenosis rate was 50.8%. A second procedure was then performed in 22 arteries: re-PTRA (12 arteries) or stenting (10 arteries). The incidence of recurrent restenosis after re-PTRA was 41.7%. Complications occurred in seven of 10 (70%) arteries secondarily treated by stenting: two with under-expansion and five with ISR. In the stent-group, stent under-expansion occurred in one case (11.1%) and ISR in three of nine stents (33.3%). In combined analysis of stented arteries, either primarily or secondarily, stent-related complications occurred in 11/19 stenting procedures (57.9%): three due to under-expansion and eight due to ISRs. Finally, despite several revascularization attempts, four of 19 (21%) stented arteries were totally occluded and one was significantly stenosed at follow-up imaging. CONCLUSION: Our study indicates that renal stenting in FMD-RAS may carry a high risk of late complications, including stent occlusion. Further observational data from large-scale registries are required.
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Angioplastia de Balón , Displasia Fibromuscular , Obstrucción de la Arteria Renal , Humanos , Arteria Renal/diagnóstico por imagen , Arteria Renal/cirugía , Displasia Fibromuscular/complicaciones , Displasia Fibromuscular/diagnóstico por imagen , Displasia Fibromuscular/terapia , Angioplastia de Balón/efectos adversos , Estudios Retrospectivos , Resultado del Tratamiento , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/etiología , Obstrucción de la Arteria Renal/terapia , Medición de Riesgo , Stents/efectos adversosRESUMEN
Atherosclerotic renovascular disease (ARVD) is the most common type of renal artery stenosis. It represents a common health problem with clinical presentations relevant to many medical specialties and carries a high risk for future cardiovascular and renal events, as well as overall mortality. The available evidence regarding the management of ARVD is conflicting. Randomized controlled trials failed to demonstrate superiority of percutaneous transluminal renal artery angioplasty (PTRA) with or without stenting in addition to standard medical therapy compared with medical therapy alone in lowering blood pressure levels or preventing adverse renal and cardiovascular outcomes in patients with ARVD, but they carried several limitations and met important criticism. Observational studies showed that PTRA is associated with future cardiorenal benefits in patients presenting with high-risk ARVD phenotypes (i.e. flash pulmonary oedema, resistant hypertension or rapid loss of kidney function). This clinical practice document, prepared by experts from the European Renal Best Practice (ERBP) board of the European Renal Association (ERA) and from the Working Group on Hypertension and the Kidney of the European Society of Hypertension (ESH), summarizes current knowledge in epidemiology, pathophysiology and diagnostic assessment of ARVD and presents, following a systematic literature review, key evidence relevant to treatment, with an aim to support clinicians in decision making and everyday management of patients with this condition.
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Aterosclerosis , Hipertensión Renovascular , Hipertensión , Obstrucción de la Arteria Renal , Humanos , Angioplastia , Aterosclerosis/complicaciones , Hipertensión/diagnóstico , Hipertensión/etiología , Hipertensión/terapia , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/terapia , Riñón , Obstrucción de la Arteria Renal/complicaciones , Obstrucción de la Arteria Renal/diagnóstico , Obstrucción de la Arteria Renal/terapia , Guías de Práctica Clínica como AsuntoRESUMEN
Atherosclerotic renovascular disease is the most frequent cause of renovascular hypertension and its prevalence increases with age and in specific subset of patients, such as those with end-stage chronic kidney disease, heart failure, and coronary artery disease. Besides hypertension, atherosclerotic renovascular disease is responsible for several clinical manifestations, including life-threatening conditions, such as recurrent flash pulmonary edema, rapidly progressive chronic kidney disease, or acute kidney injury. Atherosclerotic renovascular disease is usually part of a more diffuse atherosclerotic process and requires a combination therapy including antihypertensive, antiplatelet and lipid-lowering agents, as well as optimization of antidiabetic treatment, if needed. Besides medical therapy, percutaneous renal angioplasty was supposed to be the most effective therapy for atherosclerotic renovascular disease, by leading to blood flow restoration. However, despite an apparently solid rationale, several randomized clinical trials failed to confirm the favorable effects of percutaneous renal angioplasty on blood pressure control, kidney function, cardiovascular and renal outcomes, previously reported in observational, retrospective and single-center cohorts, switching off the enthusiasm for this procedure. Several studies' limitations may partly account for this failure, including heterogeneity of diagnostic techniques, overestimation of the degree of renal artery stenosis, inappropriate timing of revascularization, multiple protocol revisions, frequent crossovers, and most importantly exclusion of patients at higher likelihood to respond to angioplasty. The purpose of this review is to summarize studies' potential weaknesses and provide guidance to the clinician for identification of patients who may benefit most from revascularization.
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Aterosclerosis , Hipertensión Renovascular , Fallo Renal Crónico , Obstrucción de la Arteria Renal , Humanos , Estudios Retrospectivos , Aterosclerosis/diagnóstico , Aterosclerosis/terapia , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/terapia , Obstrucción de la Arteria Renal/diagnóstico , Obstrucción de la Arteria Renal/terapia , Fallo Renal Crónico/diagnósticoRESUMEN
Several forms of secondary hypertension carry a high risk of cardiac morbidity and mortality. Evaluation of cardiac phenotypes in secondary hypertension provides a unique opportunity to study underlying hormonal and biochemical mechanisms affecting the heart. We review the characteristics of cardiac dysfunction in different forms of secondary hypertension and clarify the mechanisms behind the higher prevalence of heart damage in these patients than in those with primary hypertension. Attention to the specific clinical/biochemical phenotypes of these conditions may assist clinicians to screen for and confirm secondary forms of hypertension. Thereby, early signs of heart damage can be recognized and monitored, allowing individualized treatment to delay or prevent evolution toward more advanced disease.
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Insuficiencia Cardíaca , Lesiones Cardíacas , Hipertensión , Humanos , Hipertensión/epidemiología , FenotipoRESUMEN
Importance: Spontaneous coronary artery dissection (SCAD) has been associated with fibromuscular dysplasia (FMD) and other extracoronary arterial abnormalities. However, the prevalence, severity, and clinical relevance of these abnormalities remain unclear. Objective: To assess the prevalence and spectrum of FMD and other extracoronary arterial abnormalities in patients with SCAD vs controls. Design, Setting, and Participants: This case series included 173 patients with angiographically confirmed SCAD enrolled between January 1, 2015, and December 31, 2019. Imaging of extracoronary arterial beds was performed by magnetic resonance angiography (MRA). Forty-one healthy individuals were recruited to serve as controls for blinded interpretation of MRA findings. Patients were recruited from the UK national SCAD registry, which enrolls throughout the UK by referral from the primary care physician or patient self-referral through an online portal. Participants attended the national SCAD referral center for assessment and MRA. Exposures: Both patients with SCAD and healthy controls underwent head-to-pelvis MRA (median time between SCAD event and MRA, 1 [IQR, 1-3] year). Main Outcome and Measures: The diagnosis of FMD, arterial dissections, and aneurysms was established according to the International FMD Consensus. Arterial tortuosity was assessed both qualitatively (presence or absence of an S curve) and quantitatively (number of curves ≥45%; tortuosity index). Results: Of the 173 patients with SCAD, 167 were women (96.5%); mean (SD) age at diagnosis was 44.5 (7.9) years. The prevalence of FMD was 31.8% (55 patients); 16 patients (29.1% of patients with FMD) had involvement of multiple vascular beds. Thirteen patients (7.5%) had extracoronary aneurysms and 3 patients (1.7%) had dissections. The prevalence and degree of arterial tortuosity were similar in patients and controls. In 43 patients imaged with both computed tomographic angiography and MRA, the identification of clinically significant remote arteriopathies was similar. Over a median 5-year follow-up, there were 2 noncardiovascular-associated deaths and 35 recurrent myocardial infarctions, but there were no primary extracoronary vascular events. Conclusions and Relevance: In this case series with blinded analysis of patients with SCAD, severe multivessel FMD, aneurysms, and dissections were infrequent. The findings of this study suggest that, although brain-to-pelvis imaging allows detection of remote arteriopathies that may require follow-up, extracoronary vascular events appear to be rare.
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Aneurisma/epidemiología , Disección Aórtica/epidemiología , Anomalías de los Vasos Coronarios/epidemiología , Displasia Fibromuscular/epidemiología , Enfermedades Vasculares/congénito , Adulto , Aneurisma/diagnóstico por imagen , Disección Aórtica/diagnóstico por imagen , Estudios de Casos y Controles , Angiografía por Tomografía Computarizada , Anomalías de los Vasos Coronarios/genética , Femenino , Displasia Fibromuscular/diagnóstico por imagen , Humanos , Angiografía por Resonancia Magnética , Masculino , Proteínas de Microfilamentos/genética , Persona de Mediana Edad , Prevalencia , Reino Unido/epidemiología , Enfermedades Vasculares/epidemiología , Enfermedades Vasculares/genéticaRESUMEN
Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The majority of FMD patients are women. Although a variety of genetic, mechanical, and hormonal factors play a role in the pathogenesis of FMD, overall, its cause remains poorly understood. It is probable that the pathogenesis of FMD is linked to a combination of genetic and environmental factors. Extensive studies have correlated the arterial lesions of FMD to histopathological findings of arterial fibrosis, cellular hyperplasia, and distortion of the abnormal architecture of the arterial wall. More recently, the vascular phenotype of lesions associated with FMD has been expanded to include arterial aneurysms, dissections, and tortuosity. However, in the absence of a string-of-beads or focal stenosis, these lesions do not suffice to establish the diagnosis. While FMD most commonly involves renal and cerebrovascular arteries, involvement of most arteries throughout the body has been reported. Increasing evidence highlights that FMD is a systemic arterial disease and that subclinical alterations can be found in non-affected arterial segments. Recent significant progress in FMD-related research has led to improve our understanding of the disease's clinical manifestations, natural history, epidemiology, and genetics. Ongoing work continues to focus on FMD genetics and proteomics, physiological effects of FMD on cardiovascular structure and function, and novel imaging modalities and blood-based biomarkers that can be used to identify subclinical FMD. It is also hoped that the next decade will bring the development of multi-centred and potentially international clinical trials to provide comparative effectiveness data to inform the optimal management of patients with FMD.
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Arterias , Investigación Biomédica/tendencias , Displasia Fibromuscular , Técnicas de Diagnóstico Molecular/tendencias , Animales , Arterias/metabolismo , Arterias/patología , Arterias/fisiopatología , Displasia Fibromuscular/diagnóstico , Displasia Fibromuscular/genética , Displasia Fibromuscular/metabolismo , Displasia Fibromuscular/fisiopatología , Perfilación de la Expresión Génica/tendencias , Predisposición Genética a la Enfermedad , Hemodinámica , Humanos , Fenotipo , Valor Predictivo de las Pruebas , Pronóstico , Proteómica/tendencias , Medición de Riesgo , Factores de Riesgo , Remodelación VascularRESUMEN
Renovascular hypertension is one of the most common forms of secondary hypertension. Over 95% of cases of renovascular hypertension are due either to atherosclerosis of the main renal artery trunks or to fibromuscular dysplasia. These two causes of renal artery stenosis have been extensively discussed in recent reviews and consensus. The aim of the current article is to provide comprehensive and up-to-date information on the remaining causes. While these causes are rare or extremely rare, etiologic and differential diagnosis matters both for prognosis and management. Therefore, the clinician cannot ignore them. For didactic reasons, we have grouped these different entities into stenotic lesions (neurofibromatosis type 1 and other rare syndromes, dissection, arteritis, and segmental arterial mediolysis) often associated with aortic coarctation and other arterial abnormalities, and nonstenotic lesions, where hypertension is secondary to compression of adjacent arteries and changes in arterial pulsatility (aneurysm) or to the formation of a shunt, leading to kidney ischemia (arteriovenous fistula). Finally, thrombotic disorders of the renal artery may also be responsible for renovascular hypertension. Although thrombotic/embolic lesions do not represent primary vessel wall disease, they are characterized by frequent macrovascular involvement. In this review, we illustrate the most characteristic aspects of these different entities responsible for renovascular hypertension and discuss their prevalence, pathophysiology, clinical presentation, management, and prognosis.
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Aterosclerosis/complicaciones , Displasia Fibromuscular/complicaciones , Hipertensión Renovascular/etiología , Síndrome de Alagille/complicaciones , Disección Aórtica/complicaciones , Humanos , Neurofibromatosis 1/complicaciones , Obstrucción de la Arteria Renal/complicaciones , Arteritis de Takayasu/complicacionesRESUMEN
Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.
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Aneurisma , Displasia Fibromuscular , Hipertensión , Angiografía , Femenino , Displasia Fibromuscular/diagnóstico por imagen , Displasia Fibromuscular/epidemiología , Humanos , Masculino , FenotipoRESUMEN
AIMS: Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. METHODS AND RESULTS: All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. CONCLUSIONS: The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD.
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Disección Aórtica/epidemiología , Displasia Fibromuscular/epidemiología , Adulto , Factores de Edad , Anciano , Disección Aórtica/diagnóstico por imagen , Argentina/epidemiología , Asia/epidemiología , Angiografía por Tomografía Computarizada , Europa (Continente)/epidemiología , Femenino , Displasia Fibromuscular/diagnóstico por imagen , Humanos , Incidencia , Angiografía por Resonancia Magnética , Masculino , Persona de Mediana Edad , Fenotipo , Valor Predictivo de las Pruebas , Prevalencia , Pronóstico , Sistema de Registros , Medición de Riesgo , Factores de Riesgo , Factores Sexuales , Túnez/epidemiologíaRESUMEN
Current literature suggests a higher risk of pregnancy-related complications in patients with renal fibromuscular dysplasia (FMD). The aim of our study was to assess the nature and prevalence of pregnancy-related complications in patients subsequently diagnosed with FMD. A call for participation was sent to centers contributing to the European/International FMD Registry. Patients with at least 1 pregnancy were included. Data on pregnancy were collected through medical files and FMD characteristics through the European/International FMD Registry. Data from 534 pregnancies were obtained in 237 patients. Despite the fact that, in 96% of cases, FMD was not diagnosed before pregnancy, 40% of women (n=93) experienced pregnancy-related complications, mostly gestational hypertension (25%) and preterm birth (20%), while preeclampsia was reported in only 7.5%. Only 1 patient experienced arterial dissection and another patient an aneurysm rupture. When compared with patients without pregnancy-related complications, patients with complicated pregnancies were younger at FMD diagnosis (43 versus 51 years old; P<0.001) and had a lower prevalence of cerebrovascular FMD (30% versus 52%; P=0.003) but underwent more often renal revascularization (63% versus 40%, P<0.001). In conclusion, the prevalence of pregnancy-related complications such as gestational hypertension and preterm birth was high in patients with FMD, probably related to the severity of renal FMD. However, the prevalence of preeclampsia and arterial complications was low/moderate. These findings emphasize the need to screen hypertensive women for FMD to ensure revascularization before pregnancy if indicated and appropriate follow-up during pregnancy, without discouraging patients with FMD from considering pregnancy.
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Displasia Fibromuscular/epidemiología , Complicaciones del Embarazo/epidemiología , Nacimiento Prematuro/epidemiología , Adulto , Comorbilidad , Femenino , Displasia Fibromuscular/fisiopatología , Humanos , Persona de Mediana Edad , Embarazo , Complicaciones del Embarazo/fisiopatología , Nacimiento Prematuro/fisiopatología , Prevalencia , Sistema de Registros , Arteria Renal/fisiopatología , Adulto JovenRESUMEN
BACKGROUND: Haemodialysis patients have a high mortality rate. Part of this can be attributed to vascular access complications. Large retrospective studies have shown a higher mortality in patients dialysed with a catheter, which is mostly ascribed to infectious complications. Since we observe very little infectious complications in our haemodialysis patients, the aim of our study was to assess if we could still detect a difference in survival according to vascular access type. METHODS: Patients that started chronic haemodialysis treatment between 1/1/2007 and 31/12/2016 at the 'Universitair Ziekenhuis Brussel' were retrospectively studied. The time to death was studied as a function of the two main vascular access types using survival analysis, considering the type of vascular access at the initiation of dialysis or as time varying, and accounting for the available baseline characteristics. RESULTS: Of 374 patients 309 (82.6%) initiated haemodialysis with a catheter, while 65 patients initiated with an arteriovenous access. Vascular access type during follow-up did not change in 74% of all patients. A Kaplan Meier plot did not suggest a survival dependent on the vascular access type at start. An extended cox proportional hazard analysis showed that vascular access type was not independently correlated with mortality. However, age, history of congestive heart failure and active cancer at initiation of dialysis were independently associated with mortality. CONCLUSIONS: In this retrospective cohort study, haemodialysis vascular access type was not independently correlated with patient survival, even after taking into account change of vascular access over time.
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Derivación Arteriovenosa Quirúrgica/efectos adversos , Cateterismo Venoso Central/efectos adversos , Fallo Renal Crónico/terapia , Diálisis Renal/mortalidad , Adulto , Anciano , Anciano de 80 o más Años , Análisis de Varianza , Femenino , Humanos , Estimación de Kaplan-Meier , Fallo Renal Crónico/mortalidad , Masculino , Persona de Mediana Edad , Diálisis Renal/métodos , Estudios Retrospectivos , Dispositivos de Acceso Vascular/efectos adversosRESUMEN
OBJECTIVE: Visceral artery fibromuscular dysplasia (VA FMD) manifestations range from asymptomatic to life-threatening. The aim of the study is to evaluate the prevalence and clinical characteristics of VA FMD. METHODS: A total of 232 FMD patients enrolled into ongoing ARCADIA-POL study were included in this analysis. All patients underwent detailed clinical evaluation including ambulatory blood pressure monitoring, biobanking, duplex Doppler of carotid and abdominal arteries and whole body angio-computed tomography. Three control groups (patients with renal FMD without visceral involvement, healthy normotensive patients and resistant hypertensive patients) matched for age and sex were included. RESULTS: VA FMD was present in 32 patients (13.8%). Among these patients (women: 84.4%), FMD lesions were more frequent in celiac trunk (83.1%), 62.5% of patients showed at least one visceral aneurysm, and five patients presented with severe complications related to VA FMD. No demographic differences were found between patients with VA FMD and individuals from the three control groups, with the exception of lower weight (Pâ<â0.001) and BMI (Pâ<â0.001) in VA FMD patients. Patients with FMD (with or without visceral artery involvement) showed significantly smaller visceral arterial diameters compared with controls without FMD. CONCLUSION: Patients with FMD showed smaller visceral arterial diameters when compared with patients without FMD. This may reflect a new phenotype of FMD, as a generalized arteriopathy, what needs further investigation. Lower BMI in patients with VA FMD might be explained by chronic mesenteric ischemia resulting from FMD lesions. FMD visceral involvement and visceral arterial aneurysms in patients with renal FMD are far to be rare. This strengthens the need for a systematic evaluation of all vascular beds, including visceral arteries, regardless of initial FMD involvement.
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Arterias Carótidas/diagnóstico por imagen , Displasia Fibromuscular/epidemiología , Adulto , Anciano , Bancos de Muestras Biológicas , Monitoreo Ambulatorio de la Presión Arterial , Arterias Carótidas/fisiopatología , Femenino , Displasia Fibromuscular/diagnóstico por imagen , Displasia Fibromuscular/fisiopatología , Humanos , Hipertensión/diagnóstico por imagen , Hipertensión/fisiopatología , Riñón/fisiopatología , Masculino , Persona de Mediana Edad , Fenotipo , Prevalencia , Tomografía Computarizada por Rayos X , Ultrasonografía DopplerAsunto(s)
Aterosclerosis , Arteria Renal , Humanos , Riñón , Stents , Procedimientos Quirúrgicos VascularesRESUMEN
This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD), which was commissioned by the working group 'Hypertension and the Kidney' of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Finally, it provides insights on ongoing research programs and proposes future research directions for understanding this multifaceted arterial disease.
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Angiografía/normas , Angioplastia/normas , Fármacos Cardiovasculares/uso terapéutico , Displasia Fibromuscular/diagnóstico por imagen , Displasia Fibromuscular/terapia , Angioplastia/efectos adversos , Fármacos Cardiovasculares/efectos adversos , Toma de Decisiones Clínicas , Consenso , Displasia Fibromuscular/epidemiología , Predisposición Genética a la Enfermedad , Humanos , Valor Predictivo de las Pruebas , Factores de Riesgo , Resultado del TratamientoRESUMEN
This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD) which was commissioned by the Working Group 'Hypertension and the Kidney' of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Finally, it provides insights on ongoing research programs and proposes future research directions for understanding this multifaceted arterial disease.
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Displasia Fibromuscular/terapia , Sistema de Registros , Disección Aórtica/diagnóstico por imagen , Disección Aórtica/etiología , Disección Aórtica/terapia , Angiografía , Angioplastia , Diagnóstico Diferencial , Manejo de la Enfermedad , Displasia Fibromuscular/diagnóstico por imagen , Displasia Fibromuscular/etiología , Humanos , HipertensiónRESUMEN
Tissue hypoxia plays a key role in the development and progression of many kidney diseases. Blood oxygenation level-dependent magnetic resonance imaging (BOLD-MRI) is the most promising imaging technique to monitor renal tissue oxygenation in humans. BOLD-MRI measures renal tissue deoxyhaemoglobin levels voxel by voxel. Increases in its outcome measure R2* (transverse relaxation rate expressed as per second) correspond to higher deoxyhaemoglobin concentrations and suggest lower oxygenation, whereas decreases in R2* indicate higher oxygenation. BOLD-MRI has been validated against micropuncture techniques in animals. Its reproducibility has been demonstrated in humans, provided that physiological and technical conditions are standardized. BOLD-MRI has shown that patients suffering from chronic kidney disease (CKD) or kidneys with severe renal artery stenosis have lower tissue oxygenation than controls. Additionally, CKD patients with the lowest cortical oxygenation have the worst renal outcome. Finally, BOLD-MRI has been used to assess the influence of drugs on renal tissue oxygenation, and may offer the possibility to identify drugs with nephroprotective or nephrotoxic effects at an early stage. Unfortunately, different methods are used to prepare patients, acquire MRI data and analyse the BOLD images. International efforts such as the European Cooperation in Science and Technology (COST) action 'Magnetic Resonance Imaging Biomarkers for Chronic Kidney Disease' (PARENCHIMA) are aiming to harmonize this process, to facilitate the introduction of this technique in clinical practice in the near future. This article represents an extensive overview of the studies performed in this field, summarizes the strengths and weaknesses of the technique, provides recommendations about patient preparation, image acquisition and analysis, and suggests clinical applications and future developments.
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Biomarcadores/sangre , Riñón/fisiología , Oxígeno/metabolismo , Guías de Práctica Clínica como Asunto/normas , Insuficiencia Renal Crónica/fisiopatología , Humanos , Hipoxia , Riñón/irrigación sanguínea , Imagen por Resonancia Magnética/métodos , Consumo de OxígenoRESUMEN
Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. The clinical picture is very diverse, and diagnosis is based on CT-, MR- or conventional catheter-based angiography. Involvement of VAs generally occurs among patients with multi-vessel FMD. Therefore, screening for VA FMD is advised especially in renal artery (RA) FMD and in case of aneurysms and/or dissections. Treatment depends on the clinical picture. However, the level of evidence is low, and much of the common practice is extrapolated from visceral atherosclerotic disease.
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Enfermedades Asintomáticas , Displasia Fibromuscular/diagnóstico , Anciano , Anciano de 80 o más Años , Angiografía/métodos , Diagnóstico Precoz , Urgencias Médicas , Tratamiento de Urgencia/métodos , Femenino , Displasia Fibromuscular/etiología , Displasia Fibromuscular/terapia , Estilo de Vida Saludable , Humanos , Masculino , Inhibidores de Agregación Plaquetaria/uso terapéutico , Reperfusión , Vísceras/irrigación sanguíneaRESUMEN
Fibromuscular dysplasia (FMD) is a disease associated with abnormalities of the arterial wall of medium-sized arteries. These abnormalities can lead to stenosis or less frequently to dissections or aneurysms. FMD is probably more frequent than initially thought. Nowadays, it is often a chance finding during a radiologic exam. In symptomatic cases, poor organ perfusion due to stenosis, dissection or aneurysm rupture may lead to the diagnosis. The aim of this non-systematic review illustrated with a clinical case is to present our current knowledge of FMD and to highlight the necessity of a standardized and multidisciplinary work-up to improve management of affected patients and understanding of the disease.
La dysplasie fibromusculaire (DFM) est une maladie associée à des anomalies de la paroi des artères de moyen calibre pouvant entraîner des sténoses et plus rarement des anévrismes ou des dissections. Moins rare qu'on ne le pensait initialement, la DFM est parfois découverte fortuitement lors d'un bilan de santé lorsque la maladie est asymptomatique ou lors des manifestations secondaires à l'ischémie de l'organe atteint, conséquence d'une sténose, d'une dissection ou d'une rupture de la paroi artérielle. A partir d'un cas clinique, cette revue non systématique illustre nos connaissances actuelles de la DFM et souligne l'importance d'une prise en charge multidisciplinaire et standardisée pour de meilleurs soins aux patients et une meilleure compréhension de la maladie.
Asunto(s)
Displasia Fibromuscular , Hipertensión , Aneurisma/etiología , Displasia Fibromuscular/complicaciones , Humanos , Hipertensión/complicaciones , Hipertensión/fisiopatología , Arteria RenalRESUMEN
In patients with end-stage renal disease (ESRD) treated with haemodialysis or peritoneal dialysis, hypertension is common and often poorly controlled. Blood pressure (BP) recordings obtained before or after haemodialysis display a J- or U-shaped association with cardiovascular events and survival, but this most likely reflects the low accuracy of these measurements and the peculiar haemodynamic setting related to dialysis treatment. Elevated BP detected by home or ambulatory BP monitoring is clearly associated with shorter survival. Sodium and volume excess is the prominent mechanism of hypertension in dialysis patients, but other pathways, such as arterial stiffness, activation of the renin-angiotensin-aldosterone and sympathetic nervous systems, endothelial dysfunction, sleep apnoea and the use of erythropoietin-stimulating agents may also be involved. Non-pharmacologic interventions targeting sodium and volume excess are fundamental for hypertension control in this population. If BP remains elevated after appropriate treatment of sodium and volume excess, the use of antihypertensive agents is necessary. Drug treatment in the dialysis population should take into consideration the patient's comorbidities and specific characteristics of each agent, such as dialysability. This document is an overview of the diagnosis, epidemiology, pathogenesis and treatment of hypertension in patients on dialysis, aiming to offer the renal physician practical recommendations based on current knowledge and expert opinion and to highlight areas for future research.