Renal Artery Fibromuscular Dysplasia Research Articles

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

IntroductionRenal 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 analysisThis 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 registrationClinicalTrials.gov (number NCT 05858190).Protocol version V.4 (3 May 2023).

Abstract 211: Fibromuscular Dysplasia Causing Upper Extremity Acute Limb Ischemia

Fibromuscular dysplasia (FMD) is a non-atherosclerotic arterial disease that affects mainly the renal and cervical arteries. It can also affect peripheral arteries leading to acute or chronic ischemia of the limbs. We present a rare case of FMD causing acute limb ischemia. Case Presentation: A 45-year-old male presented with acute onset pain and coldness in his left upper extremity, associated with numbness and tingling sensation in his left hand. He denied any history of trauma or recent surgery. His medical history was significant for hypertension. He was a non-smoker and denied any use of illicit drugs. On exam, his left hand was pale, cold, with absent distal pulses. The capillary refill time was prolonged, and the skin was mottled. There was tenderness in the hand muscles with no signs of compartment syndrome. Rest of the exam was normal. A diagnosis of acute limb ischemia was made, and the patient was urgently taken to the cath lab. Angiogram showed string and beads pattern of the radial artery with distal occlusion suggestive of FMD. Balloon angioplasty of the radial artery failed to improve blood flow. He subsequently underwent radial artery bypass using autologous vein graft with restoration of blood flow. Renal and carotid arteries were unaffected on CTA. Discussion: Fibromuscular dysplasia is a non-atherosclerotic, non-inflammatory disease that affects medium to small sized arteries. It is characterized by hyperplasia and fibrosis within the arterial wall. The exact etiology of FMD is unknown, and it is believed to be multifactorial. Renal artery FMD may present with renovascular hypertension, cervical artery involvement may present with headache, tinnitus, and vertigo. Patients with peripheral artery involvement may present with intermittent claudication, acute limb ischemia, or limb loss. CTA, MRA, or conventional angiography are diagnostic. In patients with acute limb ischemia, emergent intervention with thrombectomy, embolectomy, or bypass surgery may be necessary. Anti platelets and anticoagulants are also used. Conclusion: FMD is a rare disease that can present with a variety of clinical manifestations, including acute limb ischemia. Prompt diagnosis and treatment are essential to prevent limb loss and other serious complications.

Renovascular Hypertension with Progressive Atherosclerotic Renal Artery Stenosis in an Elderly Patient with Known Fibromuscular Dysplasia: A Case Report.

BACKGROUND Atherosclerotic renal artery stenosis (ARAS) and renovascular fibromuscular dysplasia (FMD) are 2 of the most common etiologies of renovascular hypertension. They have different pathophysiologies, risk factors, presentations, and treatment options. However, as our population ages, it can become increasingly common to see patients who previously had FMD who develop ARAS at an advanced age, indicated by recurrent renovascular hypertension. CASE REPORT We present a case of a 66-year-old female patient who, in 2007, had presented with uncontrolled hypertension. She underwent magnetic resonance angiography and was found to have bilateral FMD, for which she received balloon angioplasty to a severe lesion on the mid-right renal artery and subsequently had normalization of blood pressures and resolution of symptoms. In 2021 she returned with uncontrolled hypertension while being treated with 3 antihypertensives. Bilateral renal arteriography revealed new severe ostial stenosis of the left renal artery and a patent right renal artery in which balloon angioplasty was performed 14 years ago. Based on the angiographic pattern of this new left RAS, we concluded this lesion was caused by atherosclerosis. The left ostial lesion was treated with a bare-metal stent and the patient was continued on antihypertensive medication and statin; at follow-up 4 months later, her blood pressures had normalized. CONCLUSIONS This case features a patient who developed severe ARAS with underlying history of bilateral renal artery FMD. Clinicians need to be aware that in patients with FMD, worsening renovascular hypertension at an advanced age could indicate the development of new hemodynamically significant ARAS. These patients need repeat diagnostic testing and treatment with medial optimization with or without endovascular revascularization in the appropriate clinical setting.

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.

JS-NG-3: UNIQUE IMAGING FINDINGS IN RENAL ARTERY FIBROMUSCULAR DYSPLASIA

A 35-year-old female patient with fluctuating blood pressure was referred to hypertension department for investigation of possible secondary hypertension. Computed tomographic angiography (CTA) of the renal arteries was performed, demonstrating abnormal right renal artery at the medial portion of the vessel with complete tubular narrowing and right kidney atrophy (right 79*35 mm, left 109*45 mm) (Panel A). Subsequent renal arteriography confirmed the long tubular narrowed right renal artery (Panel C) and the established severe tortuosity collateral vessel from the abdominal aorta (Panel B). The etiological diagnosis of the renal artery stenosis in this young woman was challenging and was supposed to be congenital renal dysplasia. Catheter-based angioplasty was not recommended. However, intravascular ultrasound (IVUS) revealed that it was not congenital narrowed artery, but concentric intimal-medial thickening caused stenosis of the vessel (Panel E). Therefore, renal artery fibromuscular dysplasia (FMD) was diagnosed and considered to be intimal type. Balloon angioplasty was performed, and IVUS showed that the lesion lumen was dilated successfully (Panel F). However, a bailout stent had to be placed because of intimal dissection during angioplasty, and renal blood flow was restored after procedure (Panel D). One month after the procedure, the patient was normotensive without any antihypertensive medication. At 1 year follow-up, the 99mTc-glomerular filtration rate of the right kidney increased from 19.7 to 40.4 ml/min. FMD is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects renal arteries and is recommended to be classified according to angiographic finding as multifocal FMD or focal FMD. Compared with multifocal FMD, focal FMD is less often seen, and the diagnosis of some cases is challenging. In the present case, such a long tubular stenosis of renal artery is rare, and the diagnosis was confirmed by IVUS. IVUS may be a useful tool for the diagnosis of focal FMD.

PS-C34-12: MAJOR CLINICAL CHARACTERISTICS OF RENAL ARTERY FIBROMUSCULAR DYSPLASIA IN CHINESE HYPERTENSIVE PATIENTS

Background: Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects renal arteries. There is growing awareness of this disease entity. However, most of the previous studies have been from US or European populations. Methods: Consecutive hypertensive patients with renal artery stenosis caused by FMD underwent catheter-based angiography, followed at two Chinese referral centers in China between January 2000 and December 2021. Results: Of 245 study participants, with a mean diagnosed age of 26.9 ± 9.9 years, 137 (55.9%) were female, 38 (15.5%) were children. All patients had diagnosed hypertension at a mean age of 23.4 ± 8.4 years. 73.5% were focal and 15.2% were multivessel. Aneurysms, arterial dissections, and total occlusions were found in 21.6%, 4.1% and 12.2% of patients, respectively. Patients with multifocal FMD, compared to those with focal FMD, were older (26.0 vs.23.7 years, P = 0.021) and more often female (70.8% vs. 50.6%, P = 0.004), had a higher proportion of renal artery dissection (9.2% vs. 2.2%, P = 0.014), lower proportion of kidney atrophy (18.5% vs.31.1%, P = 0.013), and had fewer antihypertensive drugs (1.7 vs. 2.1, P = 0.002). After a median of 6.9 years follow-up, multifocal FMD had a higher cure rate of hypertension than focal FMD after revascularization (71.2% vs.55.2%, P = 0.033). Conclusions: Chinese patients with renal artery FMD had different characteristics from Caucasians, especially in adults. In Chinese, renal artery FMD occurred primarily in the young hypertensive patients with little sex predilection, and was frequently focal, which showed a worse blood pressure outcome than multifocal.

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.

Abstract MP72: Renal Artery Stenosis Screening Among Women With Hypertensive Disorders Of Pregnancy And Associations With Pregnancy Outcomes

Introduction: Patients with hypertensive disorders of pregnancy (HDoP) have increased prevalence of renal artery stenosis due to fibromuscular dysplasia (FMD) that is usually diagnosed years post-partum. Because renal artery FMD is a treatable cause of hypertension, earlier diagnosis has potential to decrease maternal risk and improve pregnancy outcomes. We sought to characterize utilization of renal artery duplex screening among women with HDoP and associations with pregnancy outcomes. Hypothesis: We hypothesize that renal artery duplex screening for renal artery stenosis among women with HDoP will be low. Methods: Women diagnosed with HDoP treated at a single academic medical center from 2016-2021 were identified using ICD-9&10 codes for hypertension of pregnancy, pre-eclampsia, eclampsia, and HELLP syndrome. Screening for renal artery FMD was categorized based on performance of at least one renal duplex ultrasounds during the study period. Associations with maternal demographic factors and pregnancy outcomes based on screening status were explored using descriptive statistics and categorical tests. Results: 11,564 women diagnosed with HDoP were identified. Mean age was 37.1±8.5 years. Race was categorized as 17% African American, 5% Asian, and 73% white; 4% of women had Hispanic or Latino ethnicity. 33% of patients were current or former smokers. Renal artery stenosis screening was performed on 72 women (0.6%). No significant differences in age, race, or ethnicity were identified between screened versus unscreened patients. Renal artery duplex results were abnormal in 14/72 (19%) of women screened, and an additional 3 women were diagnosed with FMD at a non-renal anatomic location based on imaging studies other than renal artery duplex. Women screened for renal artery stenosis for 44/8386 pregnancies (0.5%) associated with HDoP during the study period and had higher rates of preterm delivery (53% versus 20%; P<0.001). Conclusions: Renal artery stenosis screening is uncommon among women with HDoP, but identified abnormal findings in a significant proportion of studies. Associations between screening and preterm delivery may reflect selection bias based on severity of HDoP symptoms and related risks. Renal artery stenosis screening may be under-utilized among women with HDoP, and expanded screening has potential to reduce both short- and long-term maternal risk and improve pregnancy outcomes.

Acute Renal Infarcts from Spontaneous Intra-Renal Dissection: Case Series with a Review of Literature

Introduction: To explore the causes and management of renal infarction from pathologies such as renal artery fibromuscular dysplasia and spontaneous dissection. It is a rare occurrence and often misdiagnosed in clinical practice. Methods: We present four patients, between 30-50 years of age, who have no underlying cardiac conditions, hypertension or diabetes mellitus. They presented with abdominal/loin/flank pain due to spontaneous renal artery dissection and were treated with endovascular stents. Two patients had renal artery fibromuscular dysplasia, confirmed by CT angiogram. Results: All four patients recovered fully from the operation, with no post-operative complications noted. These patients were post-operatively managed medically with anti- platelet therapy for two years and have not experience any post-procedural complications at their 24-month follow up

Renal Artery Variations in Patients With Mild-to-Moderate Hypertension From the RADIANCE-HTN SOLO Trial

PurposeTo assess the variability of renal artery (RA) anatomy and presence of RA-pathology in patients with mild-to-moderate hypertension enrolled in the RADIANCE-HTN SOLO trial. BackgroundRADIANCE-HTN SOLO was a multicenter, international, blinded, randomized, sham-controlled trial evaluating ultrasound-based endovascular renal denervation (RDN) in patients with mild-to-moderate hypertension while off antihypertensive medications. MethodsEligible subjects had pre-randomization renal CT- or MR- angiography (CTA, MRA) to confirm anatomic suitability and to define RA ablation sites. All images were sent for independent review for evaluation of RA anatomy and other vascular pathology. ResultsA total of 324 patients underwent RA imaging (282 CTA and 42 MRA). Of those, 178 had simple anatomy with a single left and single right RA with mean diameters of 5.4 ± 0.9 and 5.1 ± 0.8 mm and mean lengths of 40.0 ± 12.9 and 52.0 ± 13.1 mm, respectively. Twenty-seven patients (8.3%) had unilateral or bilateral dual RAs with mean diameters of 4.0 ± 0.9 mm on the left and 3.9 ± 0.9 mm on the right. Forty percent (129/324) of patients had at least 1 accessory RA, with mean accessory diameters of 2.4 ± 0.8 mm on the left and 2.3 ± 0.8 mm on the right. Twenty-eight patients (8.6%) had at least 1 short (<25 mm) main RA. Incidental findings included: 9 patients (2.8%) with atherosclerotic RA stenosis ≥30%, 9 patients (2.8%) with fibromuscular dysplasia of RA and 2 patients (0.6%) with kidney and adrenal gland tumors. ConclusionsPre-procedure CTA or MRA imaging is a valuable aid in assessing RA anatomy prior to RDN because of variable RA anatomy. CTA or MRA may detect RA lesions, and renal or adrenal tumors which may need additional workup prior to consideration of RDN. Clinical trial registrationClinicalTrials.gov NCT02649426.

Renal Artery Fibromuscular Dysplasia Observed on Nonobstructive Angioscopy.

Renal Artery Fibromuscular Dysplasia Observed on Nonobstructive Angioscopy.

Clinical Characteristics and Long-Term Outcomes of Endovascular Treatment of Renal Artery Fibromuscular Dysplasia With Branch Lesions

Renal artery fibromuscular dysplasia (FMD) can cause arterial stenosis, dissection, and aneurysm of renal arteries. This study aimed to analyze the clinical characteristics and evaluate the long-term outcomes of renal branch artery FMD in children and adults. Sixty-one patients with renal artery FMD underwent endovascular treatment, including 23 children and 38 adults. They were divided into two groups, the main artery FMD group (n = 40, with severe stenosis located in the main renal artery) and the branch artery FMD group (n = 21, with only the branch lesions in unilateral or bilateral branch artery). The clinical characteristics and long-term outcomes of these pediatric and adult patients were evaluated. The incidence of branch FMD was higher in children than in adults (P = 0.005). Thirteen children showed one or more branch artery involvements. Hypertension and headache were the most common symptoms. The branch aneurysm with coexisting stenosis was more common in patients with branch artery FMD. During the follow-up, blood pressure was normal in 8 patients and lowered in 11 patients in the branch FMD group. The patient’s glomerular filtration was increased in 61 patients (P < 0.001). Four-year freedom from reintervention in 21 branch artery FMD patients was lower than that in 40 main artery FMD patients (P < 0.05). A higher incidence of renal branch artery FMD was observed in children than in adults. Endovascular treatment with balloon angioplasty can be used for treating renal branch artery FMD.

MO960RENAL TRANSPLANTATION FROM A LIVING DONOR WITH RENAL ARTERY FIBROMUSCULAR DYSPLASIA

Abstract Background and Aims Renal transplantation is considered the most effective and less costly modality of renal replacement therapy in patients with end stage renal disease. The disparity between kidney allografts and recipients has led to a global effort to increase the pool of kidney donors. Accordingly, fibromuscular dysplasia (FMD) is no longer considered an absolute contraindication for kidney donation. The incidence of FMD is about 2.3%-5.8% in potential kidney donors. There are few cases in the literature where renal artery stenosis in allografts with known pre-transplantation FMD became worse after transplantation, indicating the importance of a proper follow up in the recipients. This is a case of a living kidney donor with no history of hypertension, proteinuria or elevated serum creatinine, whose intra-arterial digital subtraction angiography revealed FMD lesions in the left renal artery. Method Case report Results A 54-year-old Caucasian female with medical history of hypothyroidism took the decision to offer her kidney to her 37-year-old son who was diagnosed with end-stage renal disease five years ago secondary to diabetes mellitus type I. She had no history for diabetes, hypertension and renal disease. Her vital signs on admission were heart rate of 78 beats/min and blood pressure of 130/70 mmHg. Urinalysis, biochemical profile and serological evaluations were all within normal ranges. Blood urea was 36 mg/dL and serum creatinine was 0.6 mg/dL (eGFR 97ml/min/1.73m2). The abdominal ultrasound and renogram with Tc-99m DTPA showed no remarkable findings. On intra-arterial digital subtraction angiography an abnormal succession of dilatations and multifocal stenoses of the left renal artery, characteristic of medial FMD, was found. The right renal artery was normal. Apart from a dysfunctional permanent left femoral catheter, the patient had no other vascular access for hemodialysis because of Superior Vena Cava syndrome, so he needed urgent transplantation. Taking all of these into consideration, the patient was offered renal transplantation as the best option. A left open donor nephrectomy was performed; the renal artery was divided distal to the stenotic dysplastic area. The allograft was placed at the right iliac fossa of the recipient with arterial and venous anastomosis to the extrarenal iliac vessels. Post-operatively, the recipient had a delayed graft function lasted 13 days. On renal artery Doppler in the allograft we found increased resistance index (RI) that gradually normalized without any intervention. An immunosuppressive regiment of tacrolimus, mycophenolate and prednisone was administered according to our center protocol. At discharge serum creatinine was 1.7 mg/dL (eGFR: 50ml/min/1.73m2). At the year follow-up, the donor was normotensive and had near normal renal function (Cr:1.3mg/dL, eGFR: 70ml/min/1.73m2). The recipient has a well-controlled blood pressure receiving two antihypertensive drugs and maintains a satisfactory renal function. Conclusion Few cases with FMD in renal allografts from living and deceased donors have been described. In a review of 4 studies the authors concluded that the outcome of transplantation with allografts from living donors with medial FMD was satisfactory and these allografts could be used to increase the donor pool. Furthermore, it is strongly recommended to have a thorough pre-transplantation check of the donor as well as a close monitoring of both the donor and recipient after transplantation. This case shows that allografts harvested from carefully selected donors with renal arterial FMD can be successfully used, particularly in urgent conditions. Detailed pre-tranplantation imaging of donor’s renal arteries, selection of the appropriate screening method, as well as close monitoring of both donor and recipient for early interventions after transplantation is of paramount importance.

Male Sex Is Associated With Cervical Artery Dissection in Patients With Fibromuscular Dysplasia.

BackgroundCervical artery dissection (CeAD) is a frequent manifestation of fibromuscular dysplasia (FMD). However, risk factors for CeAD are unknown. We investigated factors associated with CeAD in the ARCADIA (Assessment of Renal and Cervical Artery Dysplasia) registry.Methods and ResultsThe ARCADIA registry includes women or men aged ≥18 years, with a diagnosis of renal, cervical, or intracranial artery FMD, who were prospectively recruited at 16 university hospitals in France and Belgium. Diagnosis of acute or past CeAD at inclusion was established on imaging according to standard diagnostic criteria. Associations between potential determinants and CeAD were assessed by logistic regression analyses. Among 469 patients (75 men) with FMD, 65 (13.9%) had CeAD. Patients with CeAD were younger, more likely to be men, have a history of migraine, and less likely to have a history of hypertension than patients without CeAD. In the multivariable analysis, male sex (odds ratio [OR], 2.66; 95% CI, 1.34–5.25), history of migraine (OR, 1.90; 95% CI, 1.06–3.39), age ≥50 years (OR, 0.41; 95% CI, 0.23–0.73), history of hypertension (OR, 0.35; 95% CI, 0.20–0.64), and involvement of ≥3 vascular beds (OR, 2.49; 95% CI, 1.15–5.40) were significantly associated with CeAD. To validate the association between CeAD and sex, we performed a systematic review. We collected additional data on sex from 2 published studies and unpublished data from the US Registry for Fibromuscular Dysplasia and the European/International FMD Registry. In the pooled analysis (289 CeAD, 1933 patients), male sex was significantly associated with CeAD (OR, 2.04; 95% CI, 1.41–2.95; I 2=0%).ConclusionsIn patients with FMD, male sex and multisite involvement are associated with CeAD, in addition to other previously known risk factors.RegistrationURL: https://www.clinicaltrials.gov; Unique identifier: NCT02884141.

148 Acute Renal Infarcts from Spontaneous Intra-Renal Dissection

Abstract Spontaneous renal artery dissection and acute renal infarcts are rare occurrences and often misdiagnosed in clinical practice. We present four male patients, between 30-50 years of age, who have no underlying cardiac conditions, hypertension, or diabetes mellitus. They presented with abdominal/loin/flank pain due to spontaneous renal artery dissection and were all treated with endovascular stenting. Two patients had renal artery fibromuscular dysplasia, confirmed by CT angiogram, a rare pathology which is reported in literature to mostly affect women. All four patients recovered fully from the operation, with no post-operative complications noted. These patients were post-operatively managed medically with anti-platelet therapy for two years and did not experience any post-procedural complications at their 24-month follow up. Classically visceral artery dissection can be managed by anti-platelet therapy alone, however fibromuscular dysplasia can cause spontaneous dissection with renal infarcts and this requires urgent treatment with endovascular stents. Further research is needed on the post-procedural medical management guidelines.

Clinical characteristics and long-term outcomes of endovascular treatment of renal artery fibromuscular dysplasia with branch lesions.

Renal artery fibromuscular dysplasia (FMD) can cause arterial stenosis, dissection, and aneurysm of renal arteries. This study aimed to analyze the clinical characteristics and evaluate the long-term outcomes of renal branch artery FMD in children and adults. Sixty-one patients with renal artery FMD underwent endovascular treatment, including 23 children and 38 adults. They were divided into two groups, the main artery FMD group (n = 40, with severe stenosis located in the main renal artery) and the branch artery FMD group (n = 21, with only the branch lesions in unilateral or bilateral branch artery). The clinical characteristics and long-term outcomes of these pediatric and adult patients were evaluated. The incidence of branch FMD was higher in children than in adults (P = 0.005). Thirteen children showed one or more branch artery involvements. Hypertension and headache were the most common symptoms. The branch aneurysm with coexisting stenosis was more common in patients with branch artery FMD. During the follow-up, blood pressure was normal in 8 patients and lowered in 11 patients in the branch FMD group. The patient's glomerular filtration was increased in 61 patients (P < 0.001). Four-year freedom from reintervention in 21 branch artery FMD patients was lower than that in 40 main artery FMD patients (P < 0.05). A higher incidence of renal branch artery FMD was observed in children than in adults. Endovascular treatment with balloon angioplasty can be used for treating renal branch artery FMD.

Abstract 14194: Triple Vessel Spontaneous Coronary Artery Dissection With Coronary Artery Fibromuscular Dysplasia

Introduction: Spontaneous coronary artery dissection (SCAD) is a rare albeit well-established etiology of myocardial infarction and most commonly involves a single coronary vessel. We present a unique case of a patient presenting with chest pain and found to have triple vessel SCAD with associated findings suggestive of coronary artery fibromuscular dysplasia (FMD). Case Presentation: A 53 year-old woman with a past medical history of hypertension and chronic headaches presented with intermittent exertional substernal chest pain for two days. Labs were significant for an elevated Troponin-I of 0.12 ng/mL (normal &lt; 0.04 ng/mL). Coronary angiogram revealed tortuous vessels with evidence of SCAD in multiple coronary arteries including the left anterior descending artery (LAD), posterior descending artery (PDA), and posterior left ventricular artery (PLV) (Figure A, B). Intracoronary nitroglycerin was administered during the procedure to ensure the findings were not due to coronary vasospasm. Due to the known association of SCAD and FMD, a renal angiogram was performed, which demonstrated a “beading” appearance of the right renal artery consistent with renal artery FMD. She was conservatively managed with medical therapy.A head computed tomography angiogram (CTA) was performed, which showed evidence of FMD of the bilateral vertebral arteries. A repeat coronary angiogram was performed six weeks after discharge. She was found to have complete resolution of SCAD in the LAD and PLV (Figure C, D). Interestingly, the PDA displayed a “string of beads” appearance concerning for intracoronary artery FMD (Figure D). Conclusion: Our case demonstrates evidence that coronary artery FMD may contribute to the underlying etiology of the coronary artery dissection. A conservative management approach resulted in a favorable outcome and the patient was able to avoid unnecessary intervention and potential related complications.

Kidney transplantation from living donor with monolateral renal artery fibromuscular dysplasia using a cryopreserved iliac graft for arterial reconstruction: a case report and review of the literature

BackgroundAging and mortality of patients on waiting lists for kidney transplantation have increased, as a result of the shortage of organs available all over the world. Living donor grafts represent a significant source to maintain the donor pool, and resorting successfully to allografts with arterial disease has become a necessity. The incidence of renal artery fibromuscular dysplasia (FMD) in potential living renal donors is reported to be 2–6%, and up to 4% of them present concurrent extra-renal involvement.Case presentationWe present a case of renal transplantation using a kidney from a living donor with monolateral FMD. Resection of the affected arterial segment and its subsequent replacement with a cryopreserved iliac artery graft from a deceased donor were performed. No intraoperative nor post-operative complications were reported. The allograft function promptly resumed, with satisfying creatinine clearance, and adequate patency of the vascular anastomoses was detected by Doppler ultrasounds.ConclusionLiterature lacks clear guidelines on the eligibility of potential living renal donors with asymptomatic FMD. Preliminary assessment of the FMD living donor should always rule out any extra-renal involvement. Whenever possible, resection and reconstruction of the affected arterial segment should be taken into consideration as this condition may progress after implantation.

Case report: secondary hypertension in a 6-year old child with bilateral renal artery stenosis

Background. Renovascular hypertension (RVH) is an elevated blood pressure caused by partial or complete occlusion of one or both renal arteries or their branches. According to different studies, renal artery stenosis accounts for 5–10% of all cases of hypertension in children. The most common causes of renal artery stenosis are deemed to be atherosclerosis and fibromuscular dysplasia.Case description. The article describes clinical case of a newly diagnosed renovascular hypertension amid background of fibromuscular dysplasia of renal arteries in a 6-year old child, particulars of the clinical course, laboratory and instrumental methods of investigation and treatment including aortography and balloon angioplasty of renal arteries.Conclusion. This case report illustrates that in the absence of complaints and pronounced clinical symptoms the diagnosis of renovascular hypertension in a 6-year old child could not be established for a long time. Timely detection of elevated BP in children, including the young ones, not only during visiting specialist physicians but also during prophylactic examination by pediatrician, is required for early diagnosis of the disease and development of the examination and treatment strategy.

Case report: secondary hypertension in a 6-year old child with bilateral renal artery stenosis

Background. Renovascular hypertension (RVH) is an elevated blood pressure caused by partial or complete occlusion of one or both renal arteries or their branches. According to different studies, renal artery stenosis accounts for 5–10% of all cases of hypertension in children. The most common causes of renal artery stenosis are deemed to be atherosclerosis and fibromuscular dysplasia.Case description. The article describes clinical case of a newly diagnosed renovascular hypertension amid background of fibromuscular dysplasia of renal arteries in a 6-year old child, particulars of the clinical course, laboratory and instrumental methods of investigation and treatment including aortography and balloon angioplasty of renal arteries.Conclusion. This case report illustrates that in the absence of complaints and pronounced clinical symptoms the diagnosis of renovascular hypertension in a 6-year old child could not be established for a long time. Timely detection of elevated BP in children, including the young ones, not only during visiting specialist physicians but also during prophylactic examination by pediatrician, is required for early diagnosis of the disease and development of the examination and treatment strategy.