Fibromuscular dysplasia of the renal arteries: what do we know in 2024? / Dysplasie fibromusculaire des artères rénales : que savons-nous en 2024 ?

Fibromuscular dysplasia (FMD) is a rare nonatherosclerotic, noninflammatory vascular disease affecting mostly renal and carotid arteries and is the second most frequent cause of renal artery stenosis. The symptomatology is dominated by arterial hypertension due to the frequent involvement of the renal arteries and depends on the location of the lesions. Most of the cases are middle-aged women of Caucasian origin. There are two subtypes based on angiographic aspect: multifocal FMD (80% of the cases) and focal FMD (rarer with a more balanced sex ratio). Angioplasty of the renal arteries is generally disappointing with less than 50% cure of hypertension. It appears necessary to improve our knowledge of the FMD and to optimize the selection of eligible patients for revascularization with transdisciplinary collegial therapeutic decision.

La dysplasie fibromusculaire (DFM) est une maladie rare caractérisée par des sténoses segmentaires non artérioscléreuses, non inflammatoires, des artères de moyens calibres, touchant surtout les artères rénales et les carotides. Elle constitue la seconde cause de sténoses des artères rénales. La symptomatologie dépend de la localisation des lésions et est dominée par l'hypertension artérielle (HTA) en raison de l'atteinte fréquente des artères rénales. Cette pathologie touche majoritairement les femmes caucasiennes d'âge moyen. Il en existe deux sous-types, basés sur l'aspect angiographique : la DFM multifocale (80 % des cas) et la DFM focale (plus rare, sex ratio plus équilibré). Les résultats des prises en charge interventionnelles s'avèrent globalement décevants avec moins de 50 % de guérison de l'HTA. Il est nécessaire d'améliorer nos connaissances sur la physiopathologie de la DFM et d'optimiser la sélection des patients éligibles à une revascularisation par une prise de décision thérapeutique collégiale, en réunion de concertation pluridisciplinaire.

Open bypass revascularisation for endovascular-resistant transplant renal artery stenosis secondary to mechanical renal artery kinking.

Transplant renal artery stenosis (TRAS) represents a significant vascular complication subsequent to renal transplantation. This pathology is associated with grave implications including graft dysfunction and mortality. Early identification and therapeutical intervention are imperative for preserving graft longevity and achieving optimal clinical outcomes. We detail the case of a male in his 20s, following renal transplantation, who encountered recurrent TRAS, aetiologically linked to mechanical arterial kinking. Initial management using endovascular techniques yielded insufficient resolution. Consequently, the persistence of endovascular-resistant stenosis necessitated a surgical bypass intervention using the great saphenous vein, granting a 2-year period devoid of restenosis. The existing literature emphasises the indispensability of discerning the appropriate juncture for transitioning from endovascular to surgical management in TRAS cases. The robustness and durability of bypass grafts present an efficacious therapeutical strategy in contemporaneous practice.

Deep-learning-based renal artery stenosis diagnosis via multimodal fusion.

PURPOSE: Diagnosing Renal artery stenosis (RAS) presents challenges. This research aimed to develop a deep learning model for the computer-aided diagnosis of RAS, utilizing multimodal fusion technology based on ultrasound scanning images, spectral waveforms, and clinical information. METHODS: A total of 1485 patients received renal artery ultrasonography from Peking Union Medical College Hospital were included and their color doppler sonography (CDS) images were classified according to anatomical site and left-right orientation. The RAS diagnosis was modeled as a process involving feature extraction and multimodal fusion. Three deep learning (DL) models (ResNeSt, ResNet, and XCiT) were trained on a multimodal dataset consisted of CDS images, spectrum waveform images, and individual basic information. Predicted performance of different models were compared with senior physician and evaluated on a test dataset (N = 117 patients) with renal artery angiography results. RESULTS: Sample sizes of training and validation datasets were 3292 and 169 respectively. On test data (N = 676 samples), predicted accuracies of three DL models were more than 80% and the ResNeSt achieved the accuracy 83.49% ± 0.45%, precision 81.89% ± 3.00%, and recall 76.97% ± 3.7%. There was no significant difference between the accuracy of ResNeSt and ResNet (82.84% ± 1.52%), and the ResNeSt was higher than the XCiT (80.71% ± 2.23%, p < 0.05). Compared to the gold standard, renal artery angiography, the accuracy of ResNest model was 78.25% ± 1.62%, which was inferior to the senior physician (90.09%). Besides, compared to the multimodal fusion model, the performance of single-modal model on spectrum waveform images was relatively lower. CONCLUSION: The DL multimodal fusion model shows promising results in assisting RAS diagnosis.

Assessment of Angiography-Based Renal Quantitative Flow Ratio Measurement in Patients with Atherosclerotic Renal Artery Stenosis.

Background: Quantitative flow ratio (QFR) is an angiography-based fractional flow reserve measurement without pressure wire or induction of hyperemia. A recent innovation that uses combined geometrical data and hemodynamic boundary conditions to measure QFR from a single angiographic view has shown the potential to measure QFR of the renal artery-renal QFR (rQFR). Objective: The aim of this pilot study was to assess the feasibility of rQFR measurement and the contribution of rQFR in selecting patients with atherosclerotic renal artery stenosis (ARAS) undergoing revascularization. Methods: This retrospective trial enrolled patients who had ARAS (50-90%) and hypertension. The enrolled patients were treated by optimal antihypertensive medication or revascularization, respectively, and the therapeutic strategies were based on rFFR measurement and/or clinical feature. Results: A total of 55 patients underwent rQFR measurement. Among the enrolled patients, 18 underwent optimal antihypertensive medication and 37 underwent revascularization, 19 patients in whom rQFR and rFFR were both assessed. During the 180-day follow-up, 25 patients saw an improvement in their blood pressure among the 37 patients that underwent revascularization. ROC analysis revealed that rQFR had a high diagnostic accuracy for predicting blood pressure improvement (AUCrQFR = 0.932, 95% CI 0.798-0.998). The ideal cut-off value of rQFR for predicting blood pressure improvement after revascularization is ≤0.72 (sensitivity: 72.00%, specificity: 100%). The paired t test and Bland-Altman analyses demonstrated good agreement between rQFR and rFFR (t = 1.887, 95% CI -0.021 to 0.001, 95% limits of agreement: -0.035 to 0.055, p = 0.075). The Spearman correlation test reveals that there was a significant positive correlation between rQFR and rFFR (r = 0.952, 95% CI 0.874 to 0.982, p < 0.001). Conclusion: The rQFR has the potential to enhance the ability of angiography to detect functionally significant renal artery stenosis during angiography and to produce results that are comparable to invasive hemodynamic assessment.

Risks and benefits of renal artery stenting in fibromuscular dysplasia: Lessons from the ARCADIA-POL study.

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.

A Rare Case of Focal Renal Fibromuscular Dysplasia Treated With Angioplasty: A Case Report.

BACKGROUND: Fibromuscular dysplasia is an idiopathic, segmental, nonatherosclerotic, noninflammatory vascular disease that can lead to arterial stenosis, tortuosity, occlusion, aneurysms, and dissection. Fibromuscular dysplasia is a rare cause of hypertension that can easily be missed. To date, there has been no definitive treatment for fibromuscular dysplasia. CASE REPORT: In this report, we present an uncommon case of renovascular hypertension in a 21-year-old non-white female with a 3-year history of hypertension secondary to fibromuscular dysplasia involving bilateral renal arteries. Computed tomography angiography during the arterial phase revealed distal focal narrowing of the right main renal artery, distal focal narrowing of the left main renal artery, and proximal focal narrowing of the left accessory lower renal artery. Percutaneous balloon dilatation of the stenotic lesion was performed successfully up to 1 year After the procedure, the arterial blood pressure was within the normal range (110/70 to 125/75 mmHg) without medication. After 1 year of follow-up, CTA revealed re-stenosis in left main renal artery without clinical symptoms and normal blood pressure. Repeated procedure was done successfully. CONCLUSIONS: This case report highlights the difficulty in the diagnosis and treatment of focal fibromuscular dysplasia in young non-white female patients. Computerized tomographic angiography is a useful tool for identifying the cause and showing the benefit of percutaneous transluminal renal angioplasty treatment for this rare entity, as an early percutaneous angioplasty intervention may have a clinical cure for hypertension.

Transplant renal artery stenosis: Study of incidence using doppler ultrasound, risk factors and analysis is effect in graft outcomes.

INTRODUCTION AND OBJECTIVES: Transplant renal artery stenosis (TRAS) is a vascular complication after kidney transplantation which estimated incidence is 13%. It could cause refractory arterial hypertension, kidney dysfunction and premature death in transplant recipients. METHODS: We carried out a retrospective study including every patient who underwent renal transplantation between 2014 and 2020. They were evaluated with a systematic post-transplant renal Doppler ultrasound. To identify independent risk factors for transplant renal artery stenosis we performed a multivariate analysis. RESULTS: Seven hundred twenty-four kidney transplants were included, 12% ​​were living donors and 88% were deceased donors. The mean age was 54.8 in recipients and 53 in donors. Transplant renal artery stenosis was diagnosed in 70 (10%) recipients, the majority in the first 6 months after surgery. 51% of patients with transplant renal artery stenosis were managed conservatively. The multivariate analysis showed diabetes mellitus, graft rejection, arterial resuture and donor body mass index as independent risk factors for transplant renal artery stenosis. Survival of the grafts with transplant renal artery stenosis was 98% at 6 months and 95% at two years. CONCLUSIONS: The systematic performance of Doppler ultrasound in the immediate post-transplant period diagnosed 10% of transplant renal artery stenosis in our cohort. Despite the above risk factors, an adequate monitoring and treatment could avoid the increased risk of graft loss in patients with transplant renal artery stenosis.

Endovascular treatment of renal artery stenosis in Germany: a retrospective analysis of the DEGIR registry 2018-2021.

PURPOSE: To provide an overview of endovascular treatment of renal artery stenosis (RAS) using the data of the Deutsche Gesellschaft für interventionelle Radiologie (DeGIR) quality management system. MATERIALS AND METHODS: A retrospective analysis was performed. Pre-, peri- and postprocedural data, technical success rates, complication rates, and clinical success rates at dismissal were examined. RESULTS: Between 2018 and 2021, 2134 angiography examinations of the renal arteries were performed: diagnostic angiography in 70 patients (3 %), balloon angioplasty in 795 (37 %), stent implantation in 1166 (55 %) and miscellaneous procedures in 103 (5 %). The lesion length was less than or equal to 5 mm in 1837 patients (87 %), between 5 and 10 mm in 197 (9 %), and between 10 and 20 mm in 62 (3 %). The degree of stenosis was less than 50 % in 156 patients (7 %), greater than 50 % in 239 (11 %), and greater than 70 % in 1472 (70 %). Occlusion was treated in 235 patients (11 %). Symptoms at discharge resolved in 600 patients (29 %), improved in 1012 (49 %), were unchanged in 77 (4 %), and worsened in 5 (0.2 %). Complications were reported in 51 patients (2.5 %) and the mortality rate was 0.15 %. CONCLUSION: A substantial number of patients with RAS and occlusions were treated by radiologists in Germany, with high technical success rates and low complication rates. The indication should be determined carefully as the current European guidelines for the treatment of RAS suggest that only carefully selected groups of patients will benefit from recanalizing treatment. KEY POINTS: · Carefully selected patient groups may benefit from endovascular treatment of renal artery stenosis.. · Analysis of the DEGIR quality management database shows that treatment of renal artery stenosis was performed by radiologists in Germany with high technical success rates and low complication rates.. · Recanalization even led to symptom improvement in a large proportion of patients with occlusions..

Clinical Practice Report of Contrast-Enhanced Ultrasound in Renal Artery Disease.

OBJECTIVES: To evaluate the diagnostic performance of renal artery contrast-enhanced ultrasound (CEUS) with modified inspection section and summarize subsequent changes in imaging assessment of renal artery disease. METHODS: A total of 1015 patients underwent renal artery CEUS were included in the study. Among them, 79 patients (156 renal arteries) suspected with renal artery stenosis (RAS) underwent digital subtraction angiography (DSA) subsequently. DSA was used as the gold standard to evaluate the diagnostic performance of CEUS in detecting RAS (≥30%) and severe stenosis (≥70%), as well as the diagnostic accuracy of classification of stenosis degree. Besides, 127 of the 1015 patients underwent other imaging examinations such as computed tomography angiography (CTA) or magnetic resonance angiography (MRA) after CEUS and annual proportion of these imaging examinations was assessed. RESULTS: The sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) of CEUS for detecting RAS (≥30%) was 96.4%, 88.6%, 94.2%, 95.6% and 90.7%, respectively and the kappa value was .857 (P < .01). CEUS had a good performance in distinguishing severe stenosis (≥70%) with a sensitivity of 91.1%, specificity of 95.5%, accuracy of 92.9%, PPV of 96.5%, NPV of 88.7% and the kappa value was 0.857(P < .01). There was no significant difference between CEUS and DSA in detecting stenosis (P = 1.0) and severe stenosis (P = .227). The diagnostic accuracy of CEUS in grading RAS was 85.3% and the kappa value was 0.753 (P < .01). Besides, the annual proportion of other imaging examinations decreased for 4 consecutive years. CONCLUSIONS: CEUS is a non-invasive, safe and valuable technique for the assessment of renal artery disease and worthy of promotion.

A rare cause and an even rarer treatment of hypertension in a 5-year-old boy: Mid-aortic syndrome.

Renal artery stenosis is one of the common vascular diseases that cause hypertension in children. However, renal artery aneurysms and abdominal aortic aneurysms, which may be components of mid-aortic syndrome, are rarely associated with renal artery stenosis. Despite its rarity, early diagnosis and treatment are critical to prevent fatal complications. Currently, non-surgical invasive techniques are considered the first choice for treatment, but in some cases, surgery is inevitable. Here, we present a 5-year-old boy with a mid-aortic syndrome. The patient presented with a history of severe headache and epistaxis 5-6 times a day and was diagnosed with hypertension. A 9 × 9 mm saccular aneurysm on the anterior surface of the abdominal aorta at the level of the left renal artery ostium, and a 12 mm aneurysm in the left renal artery after a stenotic segment at the hilum level was detected in the doppler USG and contrast-enhanced imaging techniques. The patient was operated on electively. We used a PTFE patch to repair the abdominal aorta and, saphenous vein which was taken from his father to repair the renal artery. The patient recovered well and was discharged on the 18th day.

Renal artery stenosis stenting with unmasking and embolisation of a renal arteriovenous fistula.

A man in his 70s was presented to the emergency department with uncontrollable hypertension and worsening renal function on a background of atherosclerosis-related bilateral renal artery stenosis. Following progressive deterioration in renal function and refractory hypertension, the patient was referred to interventional radiology for consideration of renal artery stenting. Following stenting of the right renal artery, a large renal arteriovenous fistula became apparent, which required emergent embolisation. Both procedures were successful, with excellent clinical and radiological responses.

Drug-coated balloon for treatment of non-atherosclerotic renal artery stenosis-a multi-center study.

INTRODUCTION: Renal artery stenosis (RAS) is a significant reason for secondary hypertension. Impaired renal function and subsequent cardiopulmonary dysfunction could also occur. Patients of non-atherosclerotic RAS has a relatively young age and long life expectancy. Revascularization with percutaneous transluminal angioplasty (PTA) is a viable treatment option. However, restenosis is unavoidable which limits its use. Drug-coated balloon (DCB) has been proven to be effective in restenosis prevention in femoropopliteal arterial diseases and in patients with renal artery stenosis. And PTA for Renal artery fibromuscular dysplasia is safe and clinically successful. Therefore, we could speculate that DCB might have potential efficacy in non-atherosclerotic RAS treatment. METHODS AND ANALYSIS: This will be a randomized multi-center-controlled trial. Eighty-four eligible participants will be assigned randomly in a 1:1 ratio to the control group (plain old balloon, POB) and the experimental group (DCB). Subjects in the former group will receive balloon dilatation alone, and in the latter group will undergo the DCB angioplasty. The DCB used in this study will be a paclitaxel-coated balloon (Orchid, Acotec Scientific Holdings Limited, Beijing, China). Follow-up visits will be scheduled 1, 3, 6, 9, and 12 months after the intervention. Primary outcomes will include controlled blood pressure and primary patency in the 9-month follow-up. Secondary outcomes will include technical success rate, complication rate, and bail-out stenting rate. TRIAL REGISTRATION: ClinicalTrials.gov (number NCT05858190). Protocol version V.4 (3 May 2023).

Recurrent flash pulmonary edema in unilateral renal artery stenosis with contralateral kidney shrinkage: A case report.

RATIONALE: Flash pulmonary edema is a critical medical condition characterized by sudden and severe fluid accumulation in the lungs, which poses an immediate and life-threatening emergency. This can arise from a variety of underlying causes. This manuscript presents a case of recurrent pulmonary edema that was successfully managed through the insertion of a renal artery stent. PATIENT CONCERNS: A 78-year-old woman visited the emergency room with recurrent acute dyspnea. Computed tomography renal angiography revealed renal artery stenosis of a single-functioning kidney. DIAGNOSES: Flash pulmonary edema caused by renal artery stenosis of a functioning single kidney. INTERVENTIONS: Percutaneous transluminal angioplasty and stenting were performed for the renal artery stenosis. OUTCOMES: The patient's kidney function rapidly improved, and she has been free of flash pulmonary edema for 2 years. LESSONS: Flash pulmonary edema can have various causes and can immediately be a life-threatening emergency. However, it can be treated with percutaneous revascularization if it is caused by renal artery stenosis. This case report reinforces the importance of accurate and immediate diagnosis when dealing with flash pulmonary edema. This case emphasizes the potential therapeutic benefit of renal artery stenting in the management of flash pulmonary edema caused by renal artery stenosis in patients with a single-functioning kidney.

Pickering Syndrome: An Overlooked Renovascular Cause of Recurrent Heart Failure.

ABSTRACTRecurring and rapidly developing (flash) pulmonary edema is the hallmark of Pickering syndrome, affecting patients with hypertension and atherosclerotic renal artery stenosis (either bilateral or unilateral) in a solitary functioning kidney, and impaired renal function. We herein report on a series of consecutive patients with recurrent hospital admissions for pulmonary edema, impaired renal function (chronic kidney disease class 4-5), and atherosclerotic bilateral renal artery stenosis, in whom Pickering syndrome had been long neglected. We also describe a streamlined diagnostic strategy entailing little or no need for contrast medium, thus carrying no risks of further worsening of renal function. This allowed us to make the correct diagnosis and opened the way to revascularization by percutaneous transluminal renal angioplasty with stent, which provided swift recovery of kidney function with resolution of pulmonary congestion and long-term pulmonary edema- and dialysis-free survival in all cases. In summary, these findings support the following key messages: (1) considering the diagnosis of Pickering syndrome, followed by searching atherosclerotic renal artery stenosis, is an essential step toward a life-saving revascularization that avoids dialysis and an otherwise poor outcome; and (2) a simplified strategy entailing little or no need for contrast medium, carrying no associated risks of deteriorating renal function, permits the diagnosis of Pickering syndrome.

Using Ultrahigh b -Value Diffusion-Weighted Imaging to Noninvasively Assess Renal Fibrosis in a Rabbit Model of Renal Artery Stenosis.

OBJECTIVE: This study aimed to investigate the feasibility of diffusion-weighted imaging with ultrahigh b values ( ub DWI) for the evaluation of renal fibrosis (RF) induced by renal artery stenosis (RAS) in a rabbit model. METHODS: Thirty-two rabbits underwent left RAS operation, whereas 8 rabbits received sham surgery. All rabbits underwent ub DWI ( b = 0-4500 s/mm 2 ). The standard apparent diffusion coefficient (ADC st ), molecular diffusion coefficient ( D ), perfusion fraction ( f ), perfusion-related diffusion coefficient ( D *) and ultrahigh apparent diffusion coefficient (ADC uh ) were longitudinally assessed before operation and at weeks 2, 4, and 6 after operation. The degree of interstitial fibrosis and the expression of aquaporin (AQP) 1 and AQP2 were determined through pathological examination. RESULTS: In the stenotic kidney, the ADC st , D , f , and ADC uh values of the renal parenchyma significantly decreased compared with those at baseline (all P < 0.05), whereas the D * values significantly increased after RAS induction ( P < 0.05). The ADC st , D , D *, and f were weakly to moderately correlated with interstitial fibrosis as well as with the expression of AQP1 and AQP2. Furthermore, the ADC uh negatively correlated with interstitial fibrosis ( ρ = -0.782, P < 0.001) and positively correlated with AQP1 and AQP2 expression ( ρ = 0.794, P < 0.001, and ρ = 0.789, P < 0.001, respectively). CONCLUSIONS: Diffusion-weighted imaging with ultrahigh b values shows the potential for noninvasive assessment of the progression of RF in rabbits with unilateral RAS. The ADC uh derived from ub DWI could reflect the expression of AQPs in RF.

The performance of duplex ultrasonography for the assessment of renal artery stenosis in Takayasu's arteritis patients.

BACKGROUND: This study intends to analyze the hemodynamic parameters of the renal artery in patients with Takayasu's arteritis (TAK) to explore the diagnostic efficacy of duplex ultrasonography in assessing the involved renal artery in TAK patients. METHODS: One hundred fifteen TAK patients with 314 renal arteries were retrospectively analyzed, who were admitted to Peking Union Medical College Hospital between 2017 and 2022. These patients underwent both renal artery ultrasonography and angiography within a 4-week period. Specifically, the study compared seven ultrasonic parameters across groups categorized by the severity of renal artery stenosis (RAS), including noninvolvement, < 50% stenosis, 50-69% stenosis, and 70-99% stenosis. Receiver operating characteristic (ROC) curves were employed to determine the optimal threshold values for renal artery peak systolic velocity (RPSV), renal-aortic PSV ratio (RAR), and renal-interlobar PSV ratio (RIR) in order to diagnose various degrees of RAS in TAK patients. RESULTS: Statistically significant differences were observed in RAR and RIR among the four groups (all P < 0.05). However, no statistically significant differences were found in RPSV and AT between the moderate stenosis group (50-69% stenosis) and the severe stenosis group (70-99% stenosis). The discrimination of interlobar PSV (IPSV) and interlobar RI (IRI) was not significant, and IEDV did not show statistical significance among the four groups. For TAK patients, the recommended thresholds of RPSV for the diagnosis of renal artery involvement (RAI), ≥ 50% RAS, and ≥ 70% RAS were determined to be 143 cm/s, 152 cm/s, and 183 cm/s, respectively. The sensitivities, specificities, and accuracies of these thresholds were all found to be greater than 80%. Additionally, the optimal thresholds of RIR for detecting RAI, ≥ 50% RAS, and ≥ 70% RAS were determined to be 4.6, 5.6, and 6.4, respectively, with satisfactory diagnostic efficiencies. The areas under the curve (AUCs) for RPSV and RIR were calculated to be 0.908 and 0.910, respectively, for the diagnosis of ≥ 50% RAS, and 0.876 and 0.882 for the diagnosis of ≥ 70% RAS. When the aortic PSV is greater than or equal to 140 cm/s, the RAR exhibits inadequate diagnostic efficacy. Conversely, when the aortic PSV is less than 140 cm/s, a RAR value of 2.2 or higher can be employed as the diagnostic threshold for identifying RAS of 70% or greater, with a sensitivity of 84.00%, specificity of 89.93%, and an overall accuracy of 89.08%. CONCLUSION: In the present study, it has been demonstrated that RPSV and RIR possess substantial diagnostic value as ultrasonic parameters for diagnosing RAS in TAK patients. Furthermore, when assessing the diagnostic efficacy of RAR, it is crucial to consider the severity of aortic stenosis as a determining factor.

Updated trends in percutaneous renal arteriography among radiologists and other specialties.

PURPOSE: Prior studies have demonstrated an overall decline in percutaneous renal artery angioplasty with and without stenting from 1988 to 2009. We evaluated the recent utilization trends in percutaneous renal arteriography (PTRA) among radiologists and non-radiologist providers from 2010 to 2018. METHODS: Data from the 2010-2018 nationwide Medicare Part B fee-for-service database were used to tabulate case volumes for PTRA. Annual utilization rates per 10,000 Medicare beneficiaries were calculated and aggregated based on physician specialty: radiologists, cardiologists, vascular surgeons, general surgeons, or others. RESULTS: From 2010 to 2018, the overall utilization rate of PTRA markedly declined (-72% change; from 15.5 to 4.3 cases per 10,000 Medicare beneficiaries). Proportionally, the cardiologist share of PTRA saw the greatest decline, falling from 74% market share in 2010 (11.4/15.5 cases) to only 36% market share in 2018 (1.6/4.3 cases). The market share of PTRA performed by radiologists grew from 12% market share in 2010 (1.9/15.5 cases) to 28% in 2018 (1.2/4.3 cases); despite this, the absolute number of PTRA performed by radiologists saw a smaller decline over this period (-34%; 1.9 to 1.2 cases). CONCLUSION: The total utilization rates of PTRA in the Medicare population has continued to decline from 2010 to 2018, likely due to clinical trials suggesting limited efficacy of angioplasty and stenting in the treatment of renovascular hypertension and other factors such as declining reimbursement. The overall and per-specialty rates continue to decline, reflecting an overarching trend away from procedural management of renovascular hypertension.

The Effect of Endovascular Treatment of Renal Artery Stenoses on Coexistent Aneurysms Associated with Fibromuscular Dysplasia.

PURPOSE: Endovascular data on patients with coexistent renal artery stenosis (RAS) and renal artery aneurysm (RAA) caused by fibromuscular dysplasia (FMD) are scarce, and the outcomes from RAS-specific treatment on RAA remain unclear. This study aimed to evaluate the safety and effectiveness of RAS-specific endovascular management in patients with coexisting RAA caused by FMD. MATERIALS AND METHODS: Clinical and endovascular data on 19 patients with coexistent RAS and RAA caused by FMD who underwent RAS-specific endovascular therapy were analyzed prospectively. An RAA located within 10 mm of the RAS was defined as a stenosis-related RAA (SRAA), and long-term outcomes were evaluated. RESULTS: Nineteen patients (24 RASs and 30 RAAs) underwent endovascular therapy. Twenty-one RASs were treated with balloon angioplasty alone, whereas 3 RASs were treated with stent implantation. None of the RAAs were treated directly. During an average of 4.2 years ± 3.2 of follow-up, systolic and diastolic blood pressures decreased from 183.0 mm Hg ± 19.5 and 120.2 mm Hg ± 19.0 to 127.9 mm Hg ± 10.3 and 80.9 mm Hg ± 6.9, respectively; the number of antihypertensive medications reduced from 1.7 ± 1.0 to 0.8 ± 0.3 (for all, P < .001). The serum creatinine level remained stable. The maximum diameter of all RAAs decreased from 14.6 mm ± 9.7 to 11.3 mm ± 8.4 (P < .001). There was a significant difference in the improvement rate of the maximum diameter between SRAAs (65.0%, 13 of 20) and non-SRAAs (20.0%, 2 of 10) (P = .019). CONCLUSIONS: RAS-specific endovascular therapy is safe and effective and possibly aids in preventing RAA progression in patients with FMD with coexistent RAS and RAA.

Moyamoya disease presenting with tubular dysfunction in a child: pitfalls in diagnosing an atypical hyponatremic-hypertensive syndrome.

BACKGROUND: Moyamoya disease, a cause of pediatric stroke, has been shown to affect furthermore extra-cranial districts, mostly the kidney arterial site, resulting in steno-occlusive changes. Unilateral renal artery stenosis accounts for 8%-10% out of cases of renovascular hypertension in childhood, however it rarely underlies a hyponatremic-hypertensive syndrome (HHS). CASE PRESENTATION: We describe an 18-month-old boy with a recent history of polyuria and polydipsia, who presented an acute febrile gastroenteritis with neurological impairment, severe dehydration, hyponatremia, hypokalemia, kidney tubular dysfunction, and elevated aldosterone and renin even with a normal blood pressure. Fluid and electrolytes correction was performed, with complete recovery. An abdominal ultrasound displayed a smaller right kidney. A brain magnetic resonance and an electroencephalogram did not show any relevant abnormalities. Five months later, the child experienced a left-side hemiparesis after a traumatic concussion, and a severe hypertension. A brain tomography documented a cerebral ischemia. Brain and kidney angiographic studies displayed puff of smoke findings of internal right carotid artery branches and a steno-occlusive pattern of right renal artery, respectively. Hence, moyamoya disease with HHS secondary to unilateral renal artery stenosis was diagnosed. After an unsuccessful antiplatelet and antihypertensive pharmacological treatment, the boy underwent a renal angioplasty and a cerebral STA-MCA bypass (direct superficial temporal artery-to-middle cerebral artery bypass), resulting in a significant improvement of both neurological and kidney disease. CONCLUSIONS: Although the association between unilateral renal artery stenosis and HHS has been previously shown, this is the first report of atypical HHS, with hypertension preceded by tubular dysfunction, recognized in the framework of moyamoya disease.