Bilateral Renal Artery Stenosis Research Articles

Atherosclerotic Renal Arterial Stenosis with Multivascular Involvement in a Patient Presenting with Uncontrolled Hypertension and Cardiac Dysfunction

Renal artery stenosis (RAS) is the most common cause of secondary hypertension, with an incidence rate of 1–3% and atherosclerosis being the most common cause in 90% of the cases. The detection of atherosclerotic RAS may portend multivascular involvement; hence, a more comprehensive approach is essential. Clinical outcomes depend on renal condition and atherosclerotic burden with other coexisting cardiovascular diseases. A haemodynamically significant RAS can be considered with the onset of hypertensive crisis associated with worsening renal function and flash pulmonary oedema, known as Pickering syndrome. Despite the current controversy regarding the lack of randomised studies supporting the benefits of revascularisation over medical therapy, this may be considered in patients with RAS and Pickering syndrome. Reported here is the case of a 76-year-old woman with bilateral RAS who presented with persistent elevated blood pressure and heart failure symptoms. The patient underwent successful bilateral percutaneous transluminal renal angioplasty. Long-term monitoring requires maintaining adequate blood pressure control and renal function.

Prevalence and Risk Factors of Renal Artery Stenosis in Patients Undergoing Simultaneous Coronary and Renal Artery Angiography: A Systematic Review and Meta-Analysis of 31,689 Patients from 31 Studies.

Background/Objectives: Renal artery stenosis (RAS) is associated with coronary artery disease (CAD), exacerbation of arterial hypertension, and progression to heart failure, but remains frequently unrecognized in clinical practice. Methods: We conducted a systematic review and meta-analysis of studies by pooling data of patients undergoing CAG due to suspected or stable CAD that received a bilateral renal artery angiography. Results: A total of 31 studies with 31,689 patients were included (mean age 63.2 ± 8.7 years, 20.9% were female). Overall, 13.4% (95%CI 10.5-16.7%) of patients undergoing coronary angiography had significant RAS, with 6.5% (95% CI 4.5-8.9%) and 3.7% (95%CI 2.5-5.2%) having severe and bilateral RAS. The mean weighted proportion of patients with three-vessel coronary disease (3VD) was 25.1 (95%CI 19.6-30.9%) while 4.2% (95%CI 2.6-6.2%) had left main (LM) coronary disease. Patients with RAS compared to those without RAS were significantly older (mean difference, MD 4.2 years (95%CI 3.8-4.6)). The relative risk of RAS was greater for the female sex (risk ratio, 95%CI; RR 1.3, 1.03-1.57), presence of diabetes (RR 1.2, 1.10-1.36), arterial hypertension (RR 1.3, 1.21-1.46), dyslipidemia (RR 1.1, 1.06-1.14), peripheral artery disease (PAD) (RR 2.1, 1.40-3.16), chronic kidney disease (CKD) (RR 2.6, 2.04-3.37), 3VD (RR 1.6, 1.30-1.87), and LM disease (RR 1.8, 1.28-2.47). Smoking had a neutral effect on the risk of RAS occurrence (RR 1.0, 0.94-1.06). Conclusions: RAS is common in patients undergoing coronary angiography. CKD, PAD, older age, and severe CAD were among the strongest predictors for the presence of significant RAS.

Abstract P202: Excess of anything is detrimental - unraveling role of multiple accessory renal arteries in Pickering syndrome.

Flash pulmonary edema (FPE) appears to be more common in patients with bilateral renal artery stenosis (RAS) as compared to those with unilateral disease. The combination of bilateral RAS and recurrent FEP has been named the Pickering syndrome. Here, we present a case of Pickering syndrome caused by unilateral RAS in combination with multiple accessory renal arteries (ARA). To our knowledge, this case marks the inaugural report within the literature. A 68-year-old woman presented to ER with her third episode of FPE in two months, accompanied by hypertensive emergency, BP 232/127 mmHg. Initial evaluation, including laboratory tests and RA ultrasound, showed no abnormalities. However, a CTA of the abdomen revealed a single heavily calcified L main RA with severe atherosclerosis extending into the lumen of the aorta and the proximal L main RA. Three RAs supply R kidney with questionable stenosis of superior, smaller R RA. Atherosclerotic plaque is present at the origin of the inferior R RA, but no definitive significant stenosis is detected. Unilateral RAS and multiple ARAs were suspected to cause HTN and FPE. An aortogram with possible RA stenting was recommended by vascular surgery. It confirmed > 75% stenosis in the L RA, prompting stent deployment. However, no significant stenosis was observed in R ARAs. Post-procedure BP control was achieved with a single agent. FPE is a life-threatening syndrome. Cardiac systolic or diastolic dysfunctions usually accompany it. Recurrent FPE due to bilateral RAS was described by Pickering et al. in 1988. Although its exact mechanism is not completely understood, it is believed to result from excessive fluid volume due to decreased sodium excretion. A similar mechanism was proposed in RAS in a single functional kidney. Could the presence of an ARA mimic the pathophysiology of RAS? It has been theorized that an ARA may lead to HTN due to reduced blood flow to the kidney, caused by its longer and narrower caliber, which increases resistance and may lead to stenosis. The Poiseuille equation, rearranged to R=8μL/ΔPπR4 (where R=resistance, μ=viscosity, L=vessel's length, P=pressure, R=vessel's radius), suggests that both the radius and the length of ARAs may play a role in our patient's condition. In summary, ARAs are a common anatomical variation, and depending on their specific characteristics, they may contribute to resistant HTN and FPE through renin-dependent hyperaldosteronism.

Bilateral renal artery stenosis in pediatric patient with Post-Streptococcal Glomerulonephritis in children: A case report

The immune-complex mediated inflammation known as post-streptococcal glomerulonephritis (PSGN) was once thought to be one of the most common causes of acute nephritis in children. In this case, we present a 12-year-old girl experiencing symptoms of cough phlegm, congested breath, change of urine color, decreased urine production, as well swelling in the area face and legs after experiencing fever, pain swallowing, cough, and runny nose 3 weeks before admission. On physical examination, the pressure blood was 130/80 mmHg. In addition, the palpebral and extremity were edema bilateral, hyperemia of the tonsil and pharynx, enlarged tonsils T2/T3, and detritus were found. Laboratory and Imaging Tests: hematology; leukocytes 12,930, albumin 2.4 g/dl, on urinalysis, obtained urine cloudy, brownish, leukocyturia, hematuria, and proteinuria, there was also an increase in ASTO titers, abdominal ultrasound found bilateral renal artery stenosis and nephritis bilateral acute. The patient in this case presented with bilateral renal artery stenosis, which is a rare finding in PSGN cases. Management of PSGN include bed rest, low salt diet, fluid balance, as well supportive treatment with IVFD D5 ½ NS 15 drops/min (micro), Antibiotics injection of Ceftriaxone 1 gram/12 hours on the first day and continued with oral Erythromycin 4x500mg, injection prednisolone 3x1, injection Furosemide 1x1 amp, oral spironolactone and sublingual nifedipine, and hypoalbuminemia correction. Prognosis in patients with the given GNAPS governance optimally will give good results.

Renal denervation in secondary arterial hypertension therapy: clinical case

Introduction. Renovascular hypertension is one of the most severe types of hypertension in terms of the choice of treatment strategy. Recent studies have not provided a clear answer to the question of which treatment approach (conservative or surgical) is most appropriate for these patients.Brief description. The presented case describes a female patient with a longterm uncontrolled hypertension, whose examination revealed bilateral renal artery stenosis. Considering the debatable clinical effectiveness of revascularization of renal artery stenosis, the patient underwent distal renal artery denervation using multielectrode catheters. A decrease in blood pressure was shown. Follow-up of the patient demonstrated stable antihypertensive effect of the procedure, as well as its safety in case of impaired renal function.Discussion. This clinical demonstrates the third way in the treatment of patients with renal arteries stenosis and resistant hypertension — renal sympathetic denervation. This method requires a separate controlled study with individuals with renal dysfunction, including renovascular origin.

Diagnosis and Therapy of Atheroscerotic Renal Artery Stenosis - Update 2024

Abstract Atherosclerotic renal artery stenosis (ARAS), an increasingly recognized disorder, may cause ischemic nephropathy and renovascular hypertension, that may occur separately or together. Patients with chronic kidney disease (CKD) resulting from atherosclerotic renal artery stenosis usually have high-grade stenosis of both renal arteries or stenosis to a solitary functioning kidney. Patients at risk for bilateral renal artery stenosis are also at risk for other disorders that can present with similar clinical findings but cannot be corrected by surgery or angioplasty; these include hypertensive nephrosclerosis and atheroembolic kidney disease. In clinical decision-making about screening for and therapy of ARAS, we balance the chance of improving renal function and blood pressure with the risks of interventional procedures. In this article we provide an overview of the epidemiology, diagnosis, and treatment of ARVD based on consensus recommendations from a panel of physician experts who attended the recent KDIGO (Kidney Disease: Improving Global Outcomes) Controversies Conference on central and peripheral arterial diseases in chronic kidney disease. The rapid progress in this field necessitates an update. Most focus is provided for contentious issues, and we also outline aspects of investigation and management of ARVD that require further research.

Оценка чувствительности методов диагностики в выявлении гемодинамически значимых односторонних стенозов почечных артерий

Objective. Assessment of renal artery stenosis significance in patients with resistant arterial hypertension using non-invasive diagnostic methods and to compare them with the results of selective angiography and methods of physiological assessment of renal artery stenoses.Materials and methods. Prospectively, 156 patients with drug-resistant arterial hypertension and signs of renal artery stenosis detected by doppler ultrasonography of renal arteries were included in the study. Subsequently, 25 patients were excluded from the study due to multiple variant renal blood supply and bilateral renal artery stenosis. The remaining patients (n=131) underwent selective angiography of renal arteries, and 66 of them additionally underwent CTA of renal arteries with intravenous contrast. If the artery narrowing was 90% or more (n=27) in diameter, the stenosis was considered hemodynamically significant and further stenting of the affected artery was performed, and in case of 60-90% stenosis (n=52) additional assessment of functional significance of the stenosis was performed by measuring translesional pressure gradient, fractional blood flow reserve, instantaneous blood flow reserve (iFR) and Pd/ Pa ratio. Results. Among all patients (n=131), in whom the doppler ultrasonography of renal arteries showed signs of unilateral renal artery stenosis, after angiography combined with additional methods of functional significance assessment, hemodynamically significant renal artery stenosis was confirmed in 41% of cases (n=54). Thus, the sensitivity of doppler ultrasonography of renal arteries in detection of hemodynamically significant stenoses was 74%, prognostic value of positive 78% and negative result 64% (p<0,001). According to CTA (n=66) renal artery stenosis was confirmed in 56 patients. The results of CTA of renal arteries in 88% of cases coincided with the results of selective angiography, and using additional functional methods hemodynamically significant stenosis was confirmed in 32 (48%) patients. The sensitivity of CTA in detection of hemodynamically significant stenoses of renal arteries was 69%, specificity 91%, prognostic value of positive and negative results was 91 and 68% respectively. According to selective angiography, out of 131 patients, 24 patients had no renal artery stenosis, 28 patients had stenosis <60%, 27 patients had renal artery stenosis >90% and 52 patients had stenosis 60-90%. Patients with stenosis <60% were not considered candidates for renal artery stenting.

Radiation-induced Renal Artery Stenosis

We present a case of a 33 year old male presenting with uncontrolled hypertension, requiring four drugs and renal dysfunction. Investigations revealed calcific atherosclerosis of abdominal aorta and bilateral renal artery stenosis. He had received radiotherapy for abdominal Non-Hodgkin’s lymphoma 14 years ago. Transluminal angioplasty was done on the right side resulting in improved renal function and normalization of blood pressure. Radiotherapy for intra-abdominal malignancy induces atherosclerosis process in arteries within field of irradiation and they manifest after many years of the initial insult.

Mineralocorticoid Receptor Antagonists for Recurrent Hypertensive Emergency due to Bilateral Renal Artery Stenosis

Mineralocorticoid Receptor Antagonists for Recurrent Hypertensive Emergency due to Bilateral Renal Artery Stenosis

Hemodynamic analysis of unilateral and bilateral renal artery stenosis based on fluid-structure interaction

Renal artery stenosis (RAS) hypertension is a common type of secondary hypertension. This paper aimed to explore how unilateral renal artery stenosis (Uni-RAS) and bilateral renal artery stenosis (Bi-RAS) caused renovascular hypertension with the fluid-structure interaction (FSI) method. Based on a real RAS model, 20 ideal models with different stenosis degrees were established by modifying the stenosis segment. The hemodynamic parameters at different degrees of stenosis, mass flow rate (MFR), pressure drop (PD), fractional flow reserve (FFR), oscillatory shear index (OSI), and relative residence time (RRT), were numerically calculated by the computational fluid dynamics (CFD) method. The numerical results showed that RAS caused the decrease of MFR, and the increase of PD and the proportion of high OSI and high RRT. In the case of RAS, it could not be regarded as a reference indicator for causing renovascular hypertension that the value of FFR was greater than 0.9. In addition, the results of the statistical analysis indicated that Uni-RAS and Bi-RAS were statistically different for MFR, PD and the proportion of high RRT.

Lessons Learned in Determining Unilateral vs. Bilateral Intervention in Bilateral Renal Artery Stenosis

Lessons Learned in Determining Unilateral vs. Bilateral Intervention in Bilateral Renal Artery Stenosis

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.

#6465 MIR-451B AND CHRONIC KIDNEY DISEASE: A NOVEL TARGET TO MODULATE RENAL VEGF SIGNALING

Abstract Background and Aims Chronic kidney disease (CKD) is a progressive disorder that affects over 15% of adults worldwide. A major component of CKD pathophysiology is renal microvascular rarefaction and loss that pairs the progression of the disease and complications. We developed a translational swine model of CKD and showed that loss of renal function associates with a significant decrease in renal vascular endothelial growth factor (VEGF) signaling and progressive microvascular (MV) rarefaction. VEGF is a key endogenous cytokine for MV proliferation and repair, but underlying mechanisms of renal VEGF downregulation in CKD are unknown. We hypothesized that micro-RNA (miRNA)-mediated processes contribute to the downregulation of renal VEGF signaling in CKD. Method Unbiased renal micro-RNA (miRNA)-seq was performed in a swine model of CKD stage 3 (14 weeks of bilateral renal artery stenosis + atherogenic diet) and normal controls (n = 5/group). Micro-RNAs up- (>1.4 FC, p<0.05) and down-regulated (<0.7 FC, p<0.05) were identified, followed by target prediction analysis (miRwalk 3.0, Target Scan 7.2). Renal gene and protein expression of VEGF was measured by qPCR and western blot, respectively, and renal MV architecture was studied by 3D micro-CT. Results Unbiased miRNA-seq and target prediction analysis identified four miRs upregulated in the kidney of CKD pigs vs. controls, of which miR-451b (validated by qPCR), broadly conserved among species, was capable of targeting VEGFA (FC = 4.46, p = 0.04). Renal gene and protein expression of VEGF was downregulated in CKD vs. controls, associated with significant cortical and medullary microvascular rarefaction (Figure 1). Conclusion Our data suggest that post-transcriptional modulation of renal VEGF signaling may serve as a prominent mechanism of VEGF downregulation, which leads to MV rarefaction and disease progression in CKD. Furthermore, this work in progress identified a novel candidate (miR-451b) and may set the stage for its targeted modulation to preserve VEGF signaling as a potential strategy to protect the renal microvasculature in CKD.

Bilateral renal artery stenosis impacts postoperative complications after major vascular surgery

BackgroundIncidental atherosclerotic renal artery stenosis (RAS) is common in patients undergoing vascular surgery and has been shown to be associated with postoperative AKI among patients undergoing major non-vascular surgeries. We hypothesized that patients with RAS undergoing major vascular procedures would have a higher incidence of AKI and postoperative complications than those without RAS. MethodsA single-center retrospective cohort study of 200 patients who underwent elective open aortic or visceral bypass surgery (100 with postoperative AKI; 100 without AKI) were identified. RAS was then evaluated by review of pre-surgery CTAs with readers blinded to AKI status. RAS was defined as ≥50 % stenosis. Univariate and multivariable logistic regression was used to assess association of unilateral and bilateral RAS with postoperative outcomes. Results17.4 % (n = 28) of patients had unilateral RAS while 6.2 % (n = 10) of patients had bilateral RAS. Patients with bilateral RAS had similar preadmission creatinine and GFR as compared to unilateral RAS or no RAS. 100 % (n = 10) of patients with bilateral RAS had postoperative AKI compared with 45 % (n = 68) of patients with unilateral or no RAS (p < 0.05). In adjusted logistic regression models, bilateral RAS predicted severe AKI (OR 5.82; CI 1.33, 25.53; p = 0.02), in-hospital mortality (OR 5.71; CI 1.03, 31.53; p = 0.05), 30-day mortality (OR 10.56; CI 2.03, 54.05; p = 0.005) and 90-day mortality (OR 6.88; CI 1.40, 33.87; p = 0.02). ConclusionsBilateral RAS is associated with increased incidence of AKI as well as in-hospital, 30-day, and 90-day mortality suggesting it is a marker of poor outcomes and should be considered in preoperative risk stratification.

CORONARY ARTERY BYPASS SURGERY IN YOUNG ADULT A INSTITUTIONAL RETROSPECTIVE STUDY

Objective: To study the overall causes, perioperative mortality, post operative complications, prognosis, outcome and follow up in a young adult present with coronary artery disease under going coronary artery bypass in our institute. Methods: Asum of 56 patient who are 40 years and less then 40 years who underwent coronary artery bypass surgery between January 2017 to December 2022 who's clinical case history, coronary angiography, pre operative and post operative echo cardiography, operative notes, post operative notes, outpatient follow up are collected from our computer stored data and from medical record department in our college and from Tamilnadu chief minister comprehensive health insurance scheme institutional Web site data base Results: Out of 56 patient,8 Female(14.3%) and 48 Male(85.7%),mean age 37.1 years (range 32 – 40 years),Diabetic 39 Patient (62.5%),Hypertensive 21 patient (37.5%) 7 / 8 women(87.5%) diabetic ,3/8 women both diabetic and hypertensive. Out of 48 male 47(97.9%) chronic smoker, 45 (93.8%) chronic alcoholic,44 (91.6%) both alcoholic and smoke , no women in this study neither alcoholic or smoker, 3patient (5.35%) hyperlipidaemia, 2 patient (3.57%) hypothyroidism all 2/8 (25%) are female,2 patient on haemodialysis for kidney disease, 1patient with bilateral renal artery stenosis, 1 patient with right innominate, right subclavian, bilateral carotid stenosis. Left ventricular function was impaired with ejection fraction average 46% (range 30% - 55%). 1 patient with ventricular septal rupture.2 patient with post coronary stent, The indication for surgery was triple vessel disease in 30 patient(53.57%) ,double vessel disease in 20 patient(35.7%),single vessel disease in 1 patient, single vessel disease with ventricular septal rupture in 1 patient, left main coronary artery stenosis in 4 patient(7%). Surgical procedure Off pump coronary artery bypass surgery in 51 patient(91%), On pump beating heart coronary artery bypass surgery in 2 patient (3.57%),On pump arrested heart coronary artery bypass surgery in 3 patient(5.35%). A total of 132 grafts were constructed(mean 2.4 grafts per patient),35 (27%) of them being arterial only left internal mammary artery harvested and other grafts were great saphenous vein. Post operative average ventilation time18hrs (range 18hrs to 24hrs), There were 5(8.9%)hospital death and 5(8.9%) late death in follow up, total 10 (17.8%) death till now. Conclusion: Most common cause for young coronary artery disease from our clinical history is found to be early onset diabetic mellitus and from our personal history chronic smoking and chronic alcoholism, intra operative mortality and morbidity is high in patient present with severe left ventricular dysfunction, coronary artery diseases induced complication and additional comorbidity, however most of patient recovered from post operative cardiovascular lethal or morbid event and on regular clinical follow up

PS-BPR02-9: RECURRENCE OF RENAL ARTERY STENOSIS BY PONATINIB, A MULTI-TARGETED TYROSINE-KINASE INHIBITOR

Introduction: Cardiovascular disease and drug induced hypertension have been reported with tyrosine-kinase inhibitor including ponatinib treatment in chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ALL). We report bilateral renal artery stenosis (RAS) in a patient who had received ponatinib for relapsing Ph+ALL after 2nd allogeneic stem cell transplantation. Case: A patient was a 40-year-old woman with no history of cardiovascular disease and hypertension. She developed myocardial damage that appeared to be ponatinib -induced, and catheterization of the heart revealed multiple coronary artery lesions and started optical medical therapy. 16 months after ponatinib treatment, she was referred to the nephrology department for evaluation of hypertension and increase in serum creatinine from 1.47 to 2.02 mg/dL. Renal ultrasonography and MRI revealed significant stenosis of the right renal artery, and angiography and stenting were performed. 5 months after the stent treatment, progression of renal dysfunction due to exacerbated RAS was observed. Intravascular ultrasonography revealed diffuse intimal thickening occurred in-stent restenosis, and balloon dilation was performed. She required balloon dilatation a total of three times even after the discontinuation of ponatinib treatment. The patient was thought to have systemic vascular endothelial damage caused by ponatinib. Discussion: It should be considered when hypertension and renal dysfunction occurs after ponatinib use

A Novel Approach to Renovascular Hypertension in Takayasu Arteritis

Abstract Takayasu arteritis (TA) is an immune arteritis causing inflammation of the aorta, its major branches, and pulmonary arteries. It is predominantly a disease of young women, with onset typically between the ages of 20 and 30 years. Renal artery stenosis (RAS) is a significant cause of hypertension in patients with TA. An autologous conduit is preferred for renal revascularization in pediatric and young patients. We report a 26-year-old male patient, a case of TA with renovascular hypertension on four antihypertensive drugs presented to us with flash pulmonary edema. Computed tomography angiogram revealed bilateral RAS. The echocardiogram showed global hypokinesia with moderate left ventricular dysfunction. Surgical revascularization was done using a bifurcated common iliac artery (CIA) as a conduit with inflow from the aortic graft to both renal arteries. The purpose of this article was to highlight that bifurcated CIA can be used as an alternative conduit to revascularize both renal arteries.

169: SITOSTEROLEMIA PRESENTING WITH SEVERE HYPERTENSION AND CONGESTIVE HEART FAILURE IN A CHILD

Introduction: Sitosterolemia is an autosomal recessive condition caused by pathogenic variants in the ABCG5/ABCG8 genes that prohibit the body from excreting plant sterols, causing build-up in the soft tissues and systemic vasculature. Pediatric vasculopathy secondary to sitosterolemia is poorly described, and there is no clear guidance on the management of complications, including cardiac dysfunction. This case report describes the diagnosis and acute management of a child with severe systemic hypertension, multiple levels of arterial stenosis, and cardiomyopathy presenting in congestive heart failure and ultimately diagnosed with sitosterolemia. Description: An 8-year-old Amish male with no past medical history presented to the emergency department with emesis and associated fatigue and abdominal pain. Vital signs were significant for severe systemic hypertension (BP 220/110), a gallop rhythm, hepatomegaly, cardiomegaly, and mid-aortic narrowing on CT angiogram. Exam was also notable for bilateral knee xanthomas. Laboratory data after admission to the pediatric cardiac ICU showed elevated BNP (peak 4418 pg/mL) and total cholesterol (317 mg/dL) levels. Echocardiogram showed severe mitral and tricuspid valve regurgitation, severely dilated and concentrically hypertrophied and dysfunctional left ventricle, ejection fraction of 30%, and pulmonary hypertension. Consultations included genetics, cardiology, nephrology, and vascular surgery. Initial management included a continuous Milrinone infusion and loop diuretics with improved BP and congestive heart failure symptoms. Cardiac catheterization including coronary and renal angiograms demonstrated severe proximal right coronary artery stenosis and bilateral renal artery stenosis. Right coronary artery and left renal artery angioplasties were performed with good results, and showed significant improvement in parameters of ventricular function and pulmonary arterial hypertension. Genetic testing and positive serum plant sterols confirmed the diagnosis of sitosterolemia. Discussion: Sitosterolemia is a rare genetic disorder and a rare cause of pediatric arteriopathy. Early recognition can prevent the development of life-threatening systemic hypertension, arterial stenosis including coronary artery disease, and cardiomyopathy.

Malignant arterial hypertension in a 2-month-old girl: Etiological diagnosis and treatment

A 2-month-old girl presented with malignant arterial hypertension revealing bilateral renal artery stenosis secondary to neurofibromatosis type 1 (NF1). Life-supporting care was initiated immediately. High-dose peripheral vasodilator therapy induced life-threatening toxicity; vascular surgery was therefore performed. Technical difficulties due to the young age and low body weight of the patient resulted in fatal bleeding. Renovascular disease is an important cause of pediatric hypertension. NF1-associated renovascular hypertension in young pediatric patients is rare, and its highly specialized management is best delivered via a multidisciplinary approach. The long-term prognosis remains poor.

Flash pulmonary edema: Pickering syndrome due to bilateral renal artery stenosis

Flash pulmonary edema: Pickering syndrome due to bilateral renal artery stenosis