Renal Fibromuscular Dysplasia Research Articles

Prevalence and clinical characteristics of renovascular hypertension associated with fibromuscular dysplasia in China.

The aim of this study was to investigate the clinical characteristics of renal artery fibromuscular dysplasia (FMD) in patients in China and identify the cure rate of hypertension after angioplasty. Consecutive hypertensive patients with renal artery stenosis caused by FMD who underwent catheter-based angiography, and were followed at two Chinese referral centres, were retrospectively analysed. All patients underwent a detailed investigation, including demographic characteristics, clinical characteristics, biochemical sampling, Doppler ultrasonography of carotid arteries, magnetic resonance angiography (MRA) of the intracranial artery, and CTA or MRA of the abdominal artery and catheter-based renal angiography. Patients were routinely followed up at 1 month, 6 months and every year after the procedure. Among 245 study participants, with a mean diagnosed age of 26.9 ± 9.9 years, 137 (55.9%) were women, and 38 (15.5%) were children. All patients were diagnosed with hypertension at a mean age of 23.4 ± 8.4 years. There were 73.5% focal and 15.2% multivessel cases. Aneurysms, arterial dissections and total occlusions were found in 21.6, 4.1 and 12.2% of patients, respectively. Patients with multifocal FMD were older (26.0 vs. 23.7 years, P = 0.021) and more often female (70.8 vs. 50.6%, P = 0.004). Among children with renal FMD, 55.2% were men, and 86.8% were focal. After a median follow-up of 7.0 years, multifocal FMD had a higher cure rate of hypertension than focal FMD after revascularization (71.7 vs. 55.8%, P = 0.032). In a cohort of mostly young Chinese patients, the prevalence of hypertension associated with renal FMD is similar in both sexes. Focal FMDs were more frequent than the multifocal ones and, after angioplasty, were associated with a worse blood pressure outcome.

Percutaneous Transluminal Renal Angioplasty for Fibromuscular Dysplasia and Prognostic Risk Factors: A Retrospective Chinese Cohort Study

Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disease involving small-to-medium-sized arteries. The characteristics of Chinese patients with FMD remain unclear. We retrospectively analyzed the data of patients with renal FMD who underwent percutaneous transluminal renal angioplasty (PTRA) for the first time at Fuwai Hospital between 2010 and 2021. The variables were selected through least absolute shrinkage and selection operator regression (LASSO), and logistic regression models were constructed to identify independent risk factors. A total of 116 patients (52 males, median age at diagnosis, 25.0 years) were enrolled. Elevated blood pressure was the leading complaint. After a median follow-up period of 18.0 months (interquartile range: 6.0-48.0 months), hypertension recurred in 34 patients and restenosis in nine patients, among whom four patients underwent secondary intervention and one patient underwent surgical revascularization. Bilateral renal artery involvement (odds ratio [OR]: 2.61, 95% confidence interval [CI]: 1.11-6.15; p = 0.028) and age at hypertension onset (OR: 0.93, 95% CI: 0.88-0.99; p = 0.018) were independent prognostic factors for adverse outcomes. The results indicate that patients with bilateral renal artery involvement and younger age at hypertension onset are more likely to have poorer clinical outcomes after PTRA, and should be more closely monitored.

INTRAVASCULAR ULTRASOUND SUBTYPES OF UNIFOCAL RENAL ARTERY FIBROMUSCULAR DYSPLASIA

Objective: A binary angiographic classification of renal artery FMD lesions into multifocal or unifocal is well established. The multifocal type, with the ‘string-of-beads’ appearance, is related to medial FMD. While the unifocal type (focal or tubular type), is not clearly related to specific histological lesions. IVUS is a useful tool, in addition to the angiography for evaluation of the anatomical changes of structures of the vessel wall. The purpose of this study was to reveal the different subtypes of unifocal renal artery fibromuscular dysplasia (FMD) based on intravascular ultrasound (IVUS) and assess the clinical characteristics. Design and method: Thirty-six hypertensive patients (mean age 24 ± 7 years, 13–39 years) with computer tomography angiography (CTA) scan suggestive of unifocal renal artery FMD underwent renal angiography, IVUS and angioplasty, with both immediate and long-term blood pressure (BP) response assessment. Results: Of the total 36 patients, renal angiography revealed focal type in 22 (66.1%) and tubular stenoses in 14 (38.9%) patients. On IVUS, two subtypes of unifocal renal artery FMD were identified include intima-media thickening (IMT, n = 21, 58.3%) and negative remodeling of the whole vessel (NR, n = 15, 41.7%). In the 22 patients with focal type on angiography, 18 (81.8%) showed IMT and 4 (18.2%) showed NR on IVUS. 14 patients with tubular type on angiography, IVUS showed IMT in 3 (21.4%) and NR in 11 (78.6%) patients. No difference in age of onset, gender, BMI, initial BP levels were found between two IVUS subtypes. However, the proportion of BP returning to normal in the immediate term (48 hours after angioplasty procedure) (76.2% vs 26.7%, p = 0.004) and the long-term (1–5years) cure rate of hypertension (90.5% vs 20.0%, p < 0.001) were much higher in the IMT subtype than those in the NR subtype. Conclusions: In this study, we describe a new classification into IMT or NR of unifocal renal artery FMD based on IVUS. Patients with IMT renal FMD were more likely to achieve cure of hypertension.

RENAL EXTRACTION EFFICIENCY FOR PAH IS COMPARABLE IN ESSENTIAL HYPERTENSION AND RENAL FIBROMUSCULAR DYSPLASIA

Objective: Previously, we demonstrated that intrarenal blood flow in most patients with renal fibromuscular dysplasia (FMD) does not differ from that of patients with essential hypertension and patent renal arteries. In the present study, we assessed whether the renal extraction of para-aminohippurate (PAH) as a marker of tubular function was affected by the presence of FMD. Design and method: We studied 136 hypertensive patients who were scheduled for diagnostic renal angiography with arterial and renal venous blood sampling. In all of them, a continuous infusion of PAH was started two hours before the procedure to obtain steady state plasma levels at the time of sampling. Blood was collected simultaneously from the arterial and venous catheter prior to the administration of contrast material. Mean renal blood flow (MRBF) was measured simultaneously using the 133Xenon washout technique. Results: Altogether, 21 patients were diagnosed with renal FMD, 45 with atherosclerotic renal artery stenosis (ARAS) and 70 with essential hypertension (EH). Extraction ratio of PAH (ER-PAH) in the patients with renal FMD averaged 72 + 4% which did not differ significantly from that in patients with EH (74 + 3%). MBRF did not differ between these two groups either. Per contra, in patients with ARAS both MRBF and ER-PAH were significantly lower as compared to the other groups. ER-PAH in FMD patients was not modified by age, sex, height of blood pressure or MBRF. Conclusions: The ER of PAH in patients with renal FMD does not differ from that in EH. Thus, both flow and tubular function are well preserved in FMD when is EH is taken as control. This suggests that the pathophysiological mechanism which contribute to hypertension in FMD are different from those which are active in ARAS.

PLASMA STEROID PROFILE IN WOMEN WITH FIBROMUSCULAR DYSPLASIA - THE ARCADIA-GYN STUDY

Objective: Fibromuscular dysplasia(FMD) mostly affects women in reproductive age. It has been suggested that FMD might be related to female hormone levels or contraceptive use. Therefore, the aim of the ARCADIA-POL substudy ARCADIA-GYN was to perform detailed, prospective obstetrics and gynaecological evaluation of women with FMD. Design and method: We enrolled in the ARCADIA-GYN study 42 women with FMD, ages between 18–46 years old and 45 age-matched healthy, normotensive women who have regular menstruation. By means of LC-MS/MS we analysed plasma steroid profile: estrogen, progesterone, testosterone, aldosterone, cortisol, 18-oxo-cortisol, 18-hydrocortisol, cortisone, 11-deoxycortisol, 21-deoxycortisol, corticosterone, 11-deoxycortisone, progesterone, 17-OH progesterone, pregnenolone, androstenedione, DHEA, DHEA-SO4. Blood samples in all subjects were collected in luteal phase while women stayed without any hormonal contraceptive. FMD was diagnosed based on ARCADIA-POL protocol as published previously. No signs of arterial lesions and/or disturbances in blood flow were found in Doppler duplex evaluations of carotid and renal arteries in control subjects. Results: 42 patients with FMD (34.2 ± 8.1years), predominantly with renal FMD, were compared to 45 healthy, normotensive women (34.0 ± 5.4years; p = 0.88). Hypertension was diagnosed in 88.1% (37) women with FMD. In women with FMD we found lower plasma levels of progesterone (5.1 inter quartile range [IQR] 2.0–8.2 vs 7.6 IQR 3.3–12.5ng/ml;p = 0.017),testosterone (0.20 IQR 0.15–0.28 vs 0.25 IQR 0.18–0.36ng/ml; p = 0.036), DHEA (3.6 IQR 2.6–4.3 vs 4.5 IQR 2.9–6.6ng/ml;p = 0.012), DHEAS04 (1167 IQR 819–1535 vs 1579 IQR 1197–1980ng/ml;p = 0.04) and 11-deoxycortisol (0.09 IQR 0.05–0.12 vs 0.14 IQR 0.09–0.18ng/ml;p = 0.002) as compared to control group. There were no significant differences in other analyzed hormones. There were no differences in steroid profile evaluations between patients with unifocal and multifocal FMD. Conclusions: Women with FMD as compared to healthy controls are characterized by hormonal alterations including lower plasma progesterone level. Taking into consideration previous findings of abnormal balance between estrogen and progesterone receptors in renal artery samples of FMD our results suggest that female hormonal disturbances may have a role in FMD development.

Simultaneous spontaneous dissection of right carotid and left vertebral artery followed by spontaneous coronary artery dissection in a patient with renal fibromuscular dysplasia and aneurysm.

Simultaneous spontaneous dissection of right carotid and left vertebral artery followed by spontaneous coronary artery dissection in a patient with renal fibromuscular dysplasia and aneurysm.

Increased Collagen Turnover Is a Feature of Fibromuscular Dysplasia and Associated With Hypertrophic Radial Remodeling: A Pilot, Urine Proteomic Study.

Fibromuscular dysplasia (FMD), a nonatherosclerotic, noninflammatory disease of medium-sized arteries, is an underdiagnosed disease. We investigated the urinary proteome and developed a classifier for discrimination of FMD from healthy controls and other diseases. We further hypothesized that urinary proteomics biomarkers may be associated with alterations in medium-sized, but not large artery geometry and mechanics. The study included 33 patients with mostly multifocal, renal FMD who underwent in depth arterial exploration using ultra-high frequency ultrasound. The cohort was separated in a training set of 23 patients with FMD from Belgium and an independent test set of 10 patients with FMD from Italy. For each set, controls matched 2:1 were selected from the Human Urinary Proteome Database. The specificity of the classifier was tested in 700 additional controls from general population studies, patients with chronic kidney disease (n=66) and coronary artery disease (n=31). Three hundred thirty-five urinary peptides, mostly related to collagen turnover, were identified in the training cohort and combined into a classifier. When applying in the test cohort, the area under the receiver operating characteristic curve was 1.00, 100% specificity at 100% sensitivity. The classifier maintained a high specificity in additional controls (98.3%), patients with chronic kidney (90.9%) and coronary artery (96.8%) diseases. Furthermore, in patients with FMD, the proteomic score was positively associated with radial wall thickness and wall cross-sectional area. In conclusion, a proteomic score has the potential to discriminate between patients with FMD and controls. If confirmed in a wider and more diverse cohort, these findings may pave the way for a noninvasive diagnostic test of FMD.

Challenges in managing a pregnancy with underlying renal fibromuscular dysplasia

Challenges in managing a pregnancy with underlying renal fibromuscular dysplasia

Resistant hypertension: drug-eluting balloon for revascularization of bilateral renal fibromuscular dysplasia.

Resistant hypertension: drug-eluting balloon for revascularization of bilateral renal fibromuscular dysplasia.

Pregnancy-Related Complications in Patients With Fibromuscular Dysplasia: A Report From the European/International Fibromuscular Dysplasia Registry.

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.

Renovascular Hypertension: Novel Insights.

Renovascular hypertension (RVH) remains among the most prevalent and important, but also potentially reversible, causes of secondary hypertension. The predominant causes of renal artery stenosis (RAS) are atherosclerotic renovascular arterial stenosis (ARAS) and renal fibromuscular dysplasia. This condition can lead to progressive renal injury, cardiovascular complications and 'flash pulmonary edema'. Duplex Doppler ultrasonography, computed tomographic angiography and magnetic resonance angiography are the most commonly used diagnostic methods. There are three therapeutic options available: medical therapy including renin-angiotensin-aldosterone system antagonists, lipid-lowering agents, and antiplatelet therapy, percutaneous angioplasty with or without stent placement and surgical revascularization. Three large trials failed to demonstrate the superiority of renal artery revascularization over pharmaceutical therapy in controlling blood pressure and preserving renal function. For this reason, today revascularization is only recommended for patients with progressive worsening of renal function, recurrent 'flash pulmonary edema' and rapid increase in antihypertensive requirement in patients with previously well-controlled hypertension. However, more properly designed trials are needed in order to identify which patient populations would probably benefit from renal revascularization.

HTA secondaires chez la femme

The two main non-iatrogenic causes of secondary hypertension in women of childbearing age are primary aldosteronism and renal fibromuscular dysplasia. It is recommended to look for a secondary hypertension in women who remain hypertensive three months after pregnancy, in patients under 40 years of age or in patients with a grade 3 HTN (BP≥180/110mm Hg) (Professional agreement). It is suggested that the initial assessment of a secondary HTN in women is performed by a HTN specialist; it will include an assessment of renin and aldosterone concentrations and an angio-CT of the renal arteries (or angio-MRI if contraindicated) (Grade C-Class 2).

Prevalence and characteristics of renal artery fibromuscular dysplasia in hypertensive women below 50 years old

Renal fibromuscular dysplasia (FMD) is typically diagnosed in young hypertensive women. The 2014 European FMD Consensus recommended screening in all hypertensive women <30 yo. However, the prevalence of renal FMD in young/middle-aged hypertensive women remains unclear. The aim of this work was to assess the prevalence and characteristics of renal FMD in hypertensive women ≤50 yo. We retrospectively included all consecutive women aged ≤50years referred to our Hypertension Unit from 2014 to 2017 and collected standardized information on patient characteristics and screening modalities. Of 1083 incident hypertensive patients, 157 patients fitted with inclusion criteria. The prevalence of renal FMD varied between 3.2% in the whole sample and 7.5% in patients explored by CTA and/or MRA (n=67). In the subgroup of patients ≤30 yo (n=32), the corresponding figures were 3.1% and 5.6%. The yearly prevalence of FMD tended to increase over time, in parallel with increased use of CTA/MRA as a first-line imaging modality. Out of 5 patients with renal FMD, 2 were revascularized and 1 had extra-renal FMD. The prevalence of renal FMD in young/middle-aged hypertensive women is probably one order of magnitude higher than previously assumed, in the range of 3%-8%, depending on imaging modalities. While the diagnosis of FMD does not influence short-term management in all patients, it may allow close monitoring and prevention of complications of the disease over time. This analysis provides the rationale for a prospective, multicentre study aiming at determining the cost-effectiveness of systematic screening for renal FMD.

Association of intrarenal blood flow with renal function and target organ damage in hypertensive patients with fibromuscular dysplasia: the ARCADIA-POL study.

INTRODUCTION Data on the assessment of intrarenal blood flow parameters in patients with renal fibromuscular dysplasia (FMD) are scarce. OBJECTIVES The aim of the study was to evaluate intrarenal blood flow parameters in patients with FMD and significant or nonsignificant renal artery stenosis (RAS). PATIENTS AND METHODS We evaluated intrarenal blood flow parameters by Doppler ultrasonography in 153 patients with renal FMD enrolled in the ARCADIA‑POL study: 32 and 121 patients with and without significant RAS, respectively, compared with 60 matched patients with essential hypertension and 60 healthy controls. RESULTS Patients with FMD and significant RAS had a lower renal resistive index (RRI) compared with patients with FMD without significant RAS, patients with essential hypertension, and normotensive controls (mean [SD], 0.51 [0.08] vs 0.60 [0.07], 0.62 [0.06], and 0.61 [0.06], respectively; P <0.001). In patients with nonsignificant RAS, RRI correlated significantly with carotid intima-media thickness, 24‑hour diastolic blood pressure, 24‑hour pulse pressure, left ventricular diastolic function, known duration time of hypertension, and age. In patients with significant RAS, there was a significant correlation between RRI and known duration time of hypertension, left ventricular diastolic function, and age. In a separate, "per‑kidney" analysis, renal arteries with FMD and significant RAS were characterized by lower RRI values, higher maximal blood flow velocity, higher renal aortic ratio, and longer acceleration time compared with renal arteries with FMD and nonsignificant RAS as well as renal arteries without FMD. CONCLUSIONS In contrast to atherosclerotic RAS, intrarenal blood flow in patients with FMD and RAS is preserved, confirming that renal vasculature is relatively intact in these patients.

Deep Vascular Phenotyping in Patients With Renal Multifocal Fibromuscular Dysplasia.

Arterial fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease, whose pathophysiology is still unknown. We performed deep image-based vascular phenotyping of nonaffected arteries to look for systemic vascular alterations in fibromuscular dysplasia. This single center cross-sectional study included 50 patients with multifocal renal fibromuscular dysplasia, 50 hypertensive patients, and 50 healthy controls, matched for age, sex, and ethnicity; hypertensive patients were matched also for blood pressure. Brachial artery endothelium-dependent and endothelium-independent dilation were studied by echotracking. Aortic stiffness was assessed by carotid-to-femoral pulse wave velocity. We quantified the presence of supernumerary acoustic interfaces within the common carotid wall by the triple signal (TS) score. We plotted the Young incremental elastic modulus/stress curves for common carotid artery, derived from echotracking and tonometry. Patients with fibromuscular dysplasia had impaired endothelium-independent dilation (adjusted P=0.002), smaller brachial artery diameter but comparable endothelium-dependent dilation and aortic stiffness. The prevalence of TS score >6 was 56%, 40%, 24% in patients with fibromuscular dysplasia, hypertensives, and controls, respectively ( P=0.005). Fibromuscular dysplasia remained significantly associated with TS in the multiple regression model ( P=0.022). Impaired endothelium-dependent dilation was present only in patients with fibromuscular dysplasia, TS score >6 ( P=0.047). Incremental elastic modulus was higher for a given wall stress (80 kPa) in the presence of a TS score >6, especially in fibromuscular dysplasia. In conclusion, nonclinically affected large- and medium-sized arteries in patients with multifocal renal fibromuscular dysplasia exhibit a cluster of diffuse alterations in smooth muscle cell function, arterial geometry, wall characteristics, and mechanical properties. Clinical Trial Registration URL: http://www.clinicaltrials.gov . Unique identifier: NCT01935752.

First International Consensus on the diagnosis and management of fibromuscular dysplasia.

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.

FREQUENCY OF CERVICAL AND INTRACRANIAL ARTERIES LESIONS AND ASSOCIATED CLINICAL SYMPTOMS IN PATIENTS WITH CONFIRMED RENAL FIBROMUSCULAR DYSPLASIA - ARCADIA-POL STUDY

Objective: To assess the frequency of cervical and intracranial arteries involvement and associated clinical symptoms in consecutive patients with renal fibromuscular dysplasia (FMD) enrolled into ARCADIA-POL study. Design and method: From 183 patients with FMD enrolled into ARCADIA-POL study since 2015 (Polish-French collaboration) all consecutive 157 patients (132F, 25 M, mean age:45.1 ± 15.1 years) with renal FMD were analyzed. All patients underwent clinical evaluation: ABPM, biochemical evaluation, biobanking, duplex Doppler of cervical and abdominal arteries and whole body angio-CT including cervical and intracranial arteries. Results: Among 157 patients with renal FMD, 38 (24.2%) had coexisting FMD lesions in cervical and/or intracranial arteries and/or intracranial aneurysms: 17 (10.8%) patients in carotid, 7 (4.5%) patients in vertebral and 24 (15.3%) patients had lesions in intracranial arteries. Dissections of carotid arteries were present in 3 (1.9%) patients and vertebral artery dissections in 3 (1.9%) patients. In 10 (6.4%) pts internal carotid artery aneurysm and in 2 (1.3%) patients vertebral artery aneurysm were found. 18 (11.5%) patients had intracranial aneurysms: 15 (9.6%) patients had one aneurysm, 2 (1.3%) pts 2 aneurysms and 1 (0.6%) patient had 3 aneurysms. Patients with and without cervical and/or intracranial FMD lesions didn’t differ in terms of age, gender, clinical and ambulatory blood pressure levels, hypertension prevalence and number of antihypertensive medications. There were no significant differences in the prevalence of symptoms such as headaches, tinnitus, dizziness and cervical bruits between the patients with and without cervical and/or intracranial FMD. There was only a significant difference in the presence of Horner's syndrome between patients with and without cervical and/or intracranial FMD (16.7% vs.1.9%; p = 0.001, respectively). There was no difference in the prevalence of stroke, transient ischemic attack or intracranial bleeding between the groups. Conclusions: There were no specific clinical features suggesting the presence of FMD lesions and vascular complications in cervical and/or intracranial arteries in patients with confirmed renal FMD included into ARCADIA-POL STUDY. Our study showed that systematic evaluation of cervical and intracranial arteries in patients with renal FMD results in revealing relatively high prevalence of FMD lesions and vascular complications in cervical and/or intracranial arteries.

ENDOTHELIAL FUNCTION IS ASSOCIATED WITH TRIPLE SIGNAL IN FIBROMUSCULAR DYSPLASIA

Objective: Triple signal (TS) is a subclinical lesion were found in the common carotid artery of individuals with renal fibromuscular dysplasia (FMD), However, the mechanisms underlying this alteration in FMD patients are still unknown and might involve endothelial and/or smooth muscle cell dysfunction. We aimed at investigated whether FMD or triple signal, is associated with systemic vascular dysfunction. Design and method: The MEDYA study is a case-control, cross-sectional study, enrolling 47 individuals with renal FMD,47 matched individuals with essential hypertension (HT) and 50 healthy controls (C). All individuals underwent an assessment of endothelium-dependent dilation (EDD) of the brachial artery by the flow-mediated dilation technique and endothelium-independent dilation (EID) by sublingual administration of glyceryl trinitrate 150 mcg. EDD and EID were allometrically corrected for baseline diameter. Triple signal score was also computed in the common carotid arteries bilaterally, based on B-mode and RF images. A cut-off of 8 was used to discriminate TS carriers. Results: FMD, but not HT, showed impaired EID in comparison to controls (p = 0.003). FMD (but not TS) was among the determinants of EID_corr in a multiple linear regression model (beta = −0.03, p = 0.005). TS prevalence (FMD 48.9%, HT 31.9%, C 16.0%) was higher in FMD than in C (p < 0.001), but tended to be increased in HT as well (p = 0.07). In a multiple logistic regression model adjusted for confounders, FMD (OR 4.54, CL95% 1.56–13.23) but not HT, was independently associated with TS. In the FMD subgroup, TS was associated with older age, higher peak shear stress and lower EDD. EDD was still reduced in FMD patients with TS after correction for confounders (1.14 ± 3.59 vs 3.55 ± 4.18, p = 0035). In the HT subgroup, TS was independently associated only with older age and hypercholesterolemia. Conclusions: FMD is characterized by impaired smooth muscle cell function and increased TS prevalence, subclinical alterations independent from each other. TS might have a different pathophysiological significance in FMD and HT. Indeed, among FMD patients TS is associated with impaired endothelial function. Conversely, in HT TS seems to be associated only with classical CV risk factors.

Echocardiographic assessment of left ventricular morphology and function in patients with fibromuscular dysplasia: the ARCADIA-POL study.

To provide a comprehensive assessment of left ventricle (LV) structure, and function and to detect alterations in cardiac properties in relationship to presence, subtypes and extent of fibromuscular dysplasia (FMD). We studied 144 patients with FMD. The control group consisted of 50 matched individuals. Office and ambulatory blood pressure levels were evaluated. Echocardiography was employed to assess: left ventricular mass index (LVMI), systolic function including speckle tracking echocardiography and diastolic function assessed by mitral flow and tissue Doppler imaging. There were no differences in LV morphology and function between patients with FMD and the control group. Among 128 patients with renal FMD, there were no differences in LVMI and LV systolic function between patients with unifocal and multifocal FMD. The patients with multifocal FMD were characterized by lower early diastolic velocity (e') as compared with unifocal FMD and control groups. However, in a multivariate regression model, e' was not independently correlated with FMD. There were no associations between echocardiographic indexes and vascular involvement of FMD. Also, there were no differences in LV morphology and function in patients with significant renal artery stenosis (RAS) compared with patients with history of significant RAS and patients with nonsignificant RAS. Our study in contrast to those with atherosclerotic RAS, did not show differences in LV morphology and function between FMD patients and matched controls. Although FMD can result in hypertension and serious vascular complications, there is no proof that it can alter LV regardless of FMD type and its extent.

A SHRINKING KIDNEY IN A FIBROMUSCULAR DYSPLASIA PATIENT: A CASE FOR HEMODYNAMIC ASSESSMENT!

Symptomatic renal fibromuscular dysplasia (RFMD) occurs in 0.4% of the general population and presents most frequently as renovascular hypertension. Revascularization is generally considered in patients with medically refractory hypertension. A 51-year-old woman with multifocal FMD of bilateral