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1.
J Nephrol ; 37(4): 1093-1105, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38594599

RESUMEN

BACKGROUND AND OBJECTIVES: Atherosclerotic renal artery stenosis may cause hypertension, chronic kidney disease and heart failure, but large randomized control trials to date have shown no major additional benefit of renal revascularization over optimal medical management. However, these trials did not consider outcomes specifically in relation to clinical presentations. Given that atherosclerotic renal artery stenosis is a heterogenous condition, measures of success likely differ according to the clinical presentation. Our retrospective study objectives were to determine the effects of revascularization when applied to specific clinical presentations and after careful multi-disciplinary team review. METHODS: All patients presenting to our centre and its referring hospitals with radiological findings of at least one renal artery stenosis > 50% between January 2015 and January 2020 were reviewed at the renovascular multi-disciplinary team meeting with revascularization considered in accordance with international guidelines, notably for patients with anatomically significant renal artery stenosis, adequately sized kidney and presentations with any of; deteriorating kidney function, heart failure syndrome, or uncontrollable hypertension. Optimal medical management was recommended for all patients which included lipid lowering agents, anti-platelets and anti-hypertensives targeting blood pressure ≤ 130/80 mmHg. The effect of revascularization was assessed according to the clinical presentation; blood pressure and number of agents in those with renovascular hypertension, delta glomerular filtration rate in those with ischaemic nephropathy and heart failure re-admissions in those with heart failure syndromes. RESULTS: During this 5-year period, 127 patients with stenosis ≥ 50% were considered by the multidisciplinary team, with 57 undergoing revascularization (17 primarily for severe hypertension, 25 deteriorating kidney function, 6 heart failure syndrome and 9 for very severe anatomical stenosis). Seventy-nine percent of all revascularized patients had a positive outcome specific to their clinical presentation, with 82% of those with severe hypertension improving blood pressure control, 72% with progressive ischaemic nephropathy having attenuated GFR decline, and no further heart failure admissions in those with heart failure. Seventy-eight percent of patients revascularized for high grade stenosis alone had better blood pressure control with 55% also manifesting renal functional benefits. CONCLUSIONS: Multi-disciplinary team discussion successfully identified a group of patients more likely to benefit from revascularization based on 3 key factors: clinical presentation, severity of the renal artery lesion and the state of the kidney beyond the stenotic lesion. In this way, a large proportion of patients can clinically improve after revascularization if their outcomes are considered according to the nature of their clinical presentation.


Asunto(s)
Hipertensión Renovascular , Obstrucción de la Arteria Renal , Humanos , Obstrucción de la Arteria Renal/terapia , Obstrucción de la Arteria Renal/complicaciones , Obstrucción de la Arteria Renal/fisiopatología , Estudios Retrospectivos , Anciano , Femenino , Masculino , Hipertensión Renovascular/terapia , Hipertensión Renovascular/etiología , Hipertensión Renovascular/fisiopatología , Hipertensión Renovascular/diagnóstico , Resultado del Tratamiento , Persona de Mediana Edad , Aterosclerosis/complicaciones , Tasa de Filtración Glomerular , Grupo de Atención al Paciente , Antihipertensivos/uso terapéutico
2.
Catheter Cardiovasc Interv ; 104(1): 92-96, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38686525

RESUMEN

The double-kiss mini-crush (DKMC) technique has been successfully deployed in the past for the treatment of complex coronary lesions even for left main lesions. Our case report consists of a proof-of-principle that the DKMC technique can be successfully translated as well to the field of complex renal artery lesions. Insightful thinking out-of-the "coronary" box in concert with skillful off-label application of coronary stenting procedures may open the gate for unprecedented opportunities for the treatment of difficult-to-tackle in-stent restenosis in the renal circulation.


Asunto(s)
Recurrencia , Obstrucción de la Arteria Renal , Stents , Humanos , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/terapia , Obstrucción de la Arteria Renal/fisiopatología , Obstrucción de la Arteria Renal/etiología , Resultado del Tratamiento , Masculino , Angioplastia de Balón/instrumentación , Grado de Desobstrucción Vascular , Anciano , Arteria Renal/diagnóstico por imagen , Arteria Renal/fisiopatología
3.
J Cardiovasc Magn Reson ; 26(1): 100993, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38218433

RESUMEN

BACKGROUND: Percutaneous-transluminal renal angioplasty (PTRA) and stenting aim to halt the progression of kidney disease in patients with renal artery stenosis (RAS), but its outcome is often suboptimal. We hypothesized that a model incorporating markers of renal function and oxygenation extracted using radiomics analysis of blood oxygenation-level dependent (BOLD)-MRI images may predict renal response to PTRA in swine RAS. MATERIALS AND METHODS: Twenty domestic pigs with RAS were scanned with CT and BOLD MRI before and 4 weeks after PTRA. Stenotic (STK) and contralateral (CLK) kidney volume, blood flow (RBF), and glomerular filtration rate (GFR) were determined, and BOLD-MRI R2 * maps were generated before and after administration of furosemide, a tubular reabsorption inhibitor. Radiomics features were extracted from pre-PTRA BOLD maps and Robust features were determined by Intraclass correlation coefficients (ICC). Prognostic models were developed to predict post-PTRA renal function based on the baseline functional and BOLD-radiomics features, using Lasso-regression for training, and testing with resampling. RESULTS: Twenty-six radiomics features passed the robustness test. STK oxygenation distribution pattern did not respond to furosemide, whereas in the CLK radiomics features sensitive to oxygenation heterogeneity declined. Radiomics-based model predictions of post-PTRA GFR (r = 0.58, p = 0.007) and RBF (r = 0.68; p = 0.001) correlated with actual measurements with sensitivity and specificity of 92% and 67%, respectively. Models were unsuccessful in predicting post-PTRA systemic measures of renal function. CONCLUSIONS: Several radiomics features are sensitive to cortical oxygenation patterns and permit estimation of post-PTRA renal function, thereby distinguishing subjects likely to respond to PTRA and stenting.


Asunto(s)
Modelos Animales de Enfermedad , Tasa de Filtración Glomerular , Imagen por Resonancia Magnética , Valor Predictivo de las Pruebas , Obstrucción de la Arteria Renal , Circulación Renal , Stents , Sus scrofa , Obstrucción de la Arteria Renal/fisiopatología , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/terapia , Animales , Oxígeno/sangre , Factores de Tiempo , Corteza Renal/diagnóstico por imagen , Corteza Renal/irrigación sanguínea , Corteza Renal/fisiopatología , Corteza Renal/metabolismo , Furosemida/administración & dosificación , Angioplastia de Balón/instrumentación , Arteria Renal/diagnóstico por imagen , Arteria Renal/fisiopatología , Femenino , Masculino , Diuréticos , Interpretación de Imagen Asistida por Computador , Resultado del Tratamiento , Radiómica
4.
Ann Vasc Surg ; 78: 362-372, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34543714

RESUMEN

OBJECTIVE: Renal artery revascularization has been performed to improve blood pressure control and to cure hypertension in patients with renal artery fibromuscular dysplasia (RAFMD). We herein conducted a systematic review and meta-analysis of studies assessing outcomes associated with the treatment of hypertensive RAFMD patients via endovascular angioplasty in order to offer an up-to-date overview of the relative costs and benefits of this approach to revascularization in RAFMD patients. METHODS: We systematically searched the PubMed and Embase databases and the Cochrane Central Register for Controlled Trials to identify relevant studies published as of January 15, 2020. Key outcomes of interest in these studies included technical success, the incidence of perioperative complications, cure rates, and overall improvement rates. RESULTS: In total, we identified 36 relevant studies of 1916 total repairs conducted in 1191 patients. Of these included studies, 33 were retrospective, while 3 were prospective. The overall technical success rate across these studies was 94.3%. Rates of total, major, and minor complications in these pooled studies were 12.9%, 4.6%, and 7.4%, respectively. Pooled rates of cured hypertension and improved hypertension following angioplasty, defined according to study-specific criteria, were 37.0% [95% CI: 27.0%-47.0%] and 80.0% [95% CI: 75.0% to 84.0%], respectively, although these rates varied highly among studies. Cure rates for studies used current clinical definitions for substantial variations across studies. Cure rates in studies using current definitions of cured hypertension (blood pressure <140/90 mm Hg without treatment) were just 18.1% following angioplasty. Cure rates fell markedly with increasing mean patient age (OR associated with an increase in mean age of 10 years: -0.24 [95% CI: -0.44 to -0.04, P = 0.019] and with mean known duration of hypertension (OR associated with an increase in mean hypertension duration of 5 years: -0.09 [95% CI: -0.12 to -0.05, P = 0.001]). CONCLUSIONS: These findings suggest that endovascular treatment yielded moderate benefits to RAFMD patients, with substantial variation across studies. The blood pressure outcome was strongly influenced by patient age.


Asunto(s)
Angioplastia , Displasia Fibromuscular/complicaciones , Hipertensión Renovascular/terapia , Obstrucción de la Arteria Renal/terapia , Adulto , Factores de Edad , Angioplastia/efectos adversos , Presión Sanguínea , Femenino , Displasia Fibromuscular/diagnóstico , Displasia Fibromuscular/fisiopatología , Humanos , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/fisiopatología , Masculino , Obstrucción de la Arteria Renal/diagnóstico , Obstrucción de la Arteria Renal/etiología , Obstrucción de la Arteria Renal/fisiopatología , Resultado del Tratamiento
5.
J Vasc Surg ; 75(3): 939-949.e1, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-34601043

RESUMEN

OBJECTIVE: The indication of percutaneous renal transluminal angioplasty (PTRA) in fibromuscular dysplasia (FMD) is mainly based on renal artery stenosis (RAS) due to atherosclerosis criteria, which are not specific to FMD. Consequently, the selection of patients who could benefit from this treatment and its effectiveness remain uncertain. The aims of this study were to: (1) report the effects of PTRA guided by trans-stenotic pressure measurements on hypertension 7 months after treatment; (2) assess the impact of pressure measurement to guide treatment efficacy in comparison to visual angiographic parameters; and (3) evaluate the reproducibility and accuracy of the stenosis measurement using a 4F catheter in comparison to a pressure guidewire. METHODS: This prospective multi-centric study analyzed 24 patients with hypertension with RAS due to FMD that required PTRA. Clinical, duplex ultrasound, and angiographic indices were collected, and patients were followed up for 7 months (±1 month). Angiographic indices were measured twice both by a pressure guidewire and a 4F catheter. Assessment of procedural and clinical success of angioplasty was performed for all patients. RESULTS: Twenty-three patients (96%) had procedural success (considered as a post-PTRA translesional systolic gradient ≤10 mmHg or reduced by at least 80%) with a significant decrease in the systolic gradient after angioplasty (26.50 mmHg; [interquartile range, 16.75-38.75] vs 0.00 [interquartile range, 0.00-2.00]; P < .01). Three patients (12%) had complications, including two renal artery dissections and one partial renal infarction. Twenty-one patients (88%) were clinical responders to angioplasty at follow-up. Visual stenosis assessment showed a poor correlation with systolic gradient measurement before and after PTRA (R from -0.05 to 0.41; P = 0.06-0.82). High correlations were found between pressure measurements made by a 4F catheter and guidewire (R from 0.64 to 0.89; P ≤ .003). CONCLUSIONS: In patients selected by clinical indicators and duplex ultrasound, reaching a translesional systolic gradient ≤10 mmHg or reduced by at least 80% after angioplasty, promotes a high success rate for PTRA in hypertension due to FMD RAS.


Asunto(s)
Angioplastia de Balón , Presión Arterial , Displasia Fibromuscular/terapia , Hipertensión Renovascular/terapia , Obstrucción de la Arteria Renal/terapia , Arteria Renal/fisiopatología , Adulto , Angioplastia de Balón/efectos adversos , Determinación de la Presión Sanguínea/instrumentación , Angiografía por Tomografía Computarizada , Femenino , Displasia Fibromuscular/complicaciones , Displasia Fibromuscular/diagnóstico , Displasia Fibromuscular/fisiopatología , Francia , Humanos , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/fisiopatología , Masculino , Persona de Mediana Edad , Tomografía Computarizada Multidetector , Estudios Prospectivos , Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/diagnóstico , Obstrucción de la Arteria Renal/etiología , Obstrucción de la Arteria Renal/fisiopatología , Factores de Tiempo , Transductores de Presión , Resultado del Tratamiento , Ultrasonografía Doppler en Color , Dispositivos de Acceso Vascular
6.
Ann Vasc Surg ; 78: 379.e1-379.e5, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34481882

RESUMEN

BACKGROUND: To describe a retrograde recanalization for the proximal occluded lesion in right renal artery (RRA) in young patient with fibromuscular dysplasia (FMD). METHODS: A 10-year-old girl presented to our hospital with proximal RRA occlusion and refractory hypertension though she took anti-hypertension medicines. Her renin and aldosterone were beyond the normal level in both base state and excited state. Her glomerular filtration rate at right kidney was only 18.4 ml/min. Angiography revealed proximal RRA occlusion and a compensated collateral artery (CCA) from the infrarenal aorta to the RRA. She was thus diagnosed with focal FMD. A retrograde recanalization was performed through this CCA. RESULTS: Angioplasty and stenting were successfully performed to treat the proximal RRA occlusion. Postoperatively, the glomerular filtration rate in the right kidney improved. One-year follow-up revealed that, the blood pressure maintained at normal range without any antihypertensive agents. No other discomfort was complained. CONCLUSIONS: It is feasible to establish a working pathway with patient's compensated collateral artery to treat the renal artery occlusion.


Asunto(s)
Angioplastia de Balón , Circulación Colateral , Displasia Fibromuscular/complicaciones , Hipertensión Renovascular/terapia , Obstrucción de la Arteria Renal/terapia , Circulación Renal , Angioplastia de Balón/instrumentación , Presión Sanguínea , Niño , Femenino , Displasia Fibromuscular/diagnóstico , Displasia Fibromuscular/fisiopatología , Humanos , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/fisiopatología , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/etiología , Obstrucción de la Arteria Renal/fisiopatología , Stents , Resultado del Tratamiento
7.
BMC Cardiovasc Disord ; 21(1): 606, 2021 12 20.
Artículo en Inglés | MEDLINE | ID: mdl-34930129

RESUMEN

BACKGROUND: A honeycomb-like structure (HLS) is a rare abnormality characterized by a braid-like appearance. Angiograph and intravascular examination, including coherence tomography and intravascular ultrasound (IVUS), can further confirm the multiple intraluminal channels or honeycomb structure, which can also be described as looking like 'swiss cheese', a 'spider web' or a 'lotus root'. Previous studies have mostly reported this abnormality in coronary arteries, with a few cases in renal arteries. More information about the characteristics and development of HLS is needed. CASE PRESENTATION: A 69-year-old Han man with resistant hypertension received abdominal enhanced computerised tomography and was revealed to have left renal artery stenosis with the possibility of left renal infarction. Renal artery angiography confirmed a 95% stenosis located in the proximal segment of the left renal artery, and the middle segment was blurred with multi-channel-like blood flow. Further IVUS was performed and identified multiple channels surrounded by fibrous tissue. It was a rare case of HLS in the renal artery secondary to the thrombus, with organisation and recanalisation. Balloon dilatation and stent implantation at the proximal segment of the left renal artery were performed successfully. Blood pressure was well controlled after the procedure. CONCLUSIONS: The IVUS findings are helpful for forming interventional therapeutic strategies for HLS lesions in the renal artery.


Asunto(s)
Angioplastia de Balón , Hipertensión Renovascular/terapia , Obstrucción de la Arteria Renal/terapia , Arteria Renal/anomalías , Anciano , Angiografía , Angioplastia de Balón/instrumentación , Humanos , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/fisiopatología , Masculino , Arteria Renal/diagnóstico por imagen , Arteria Renal/fisiopatología , Obstrucción de la Arteria Renal/complicaciones , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/fisiopatología , Stents , Resultado del Tratamiento , Ultrasonografía Intervencional
8.
Eur Rev Med Pharmacol Sci ; 25(20): 6333-6338, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34730214

RESUMEN

OBJECTIVE: Arterial hypertension (AH) represents a major risk factor for cardiovascular disease and is associated to several complications, such as prolonged corrected QT (QTc) interval and impaired heart rate variability (HRV). Secondary causes of AH include autosomal dominant polycystic kidney disease (ADPKD) and atherosclerotic renal artery stenosis (ARAS), both known to be related to arrhythmic risk and autonomic imbalance. The aim of the study is to evaluate whether global autonomic activity and QTc interval differently affect ADPKD and ARAS hypertensive patients. PATIENTS AND METHODS: An observational study was performed on 59 patients: 16 ADPKD patients and 19 diagnosed with ARAS, compared to 24 healthy controls (HC). All patients underwent clinical evaluation, biochemical lab tests, 24-hour electrocardiogram (ECG) and renal Doppler ultrasound. HRV was assessed through the analysis of 24-hour ECG to detect standard deviation of normal-to-normal RR intervals (SDNN). QTc interval was defined as prolonged when > 440 msec. RESULTS: SDNN was significantly lower in ADPKD and ARAS patients than HC (p < 0.0001) and no significant differences were found between ADPKD and ARAS patients (p > 0.05). QTc was found significantly higher in ARAS patients than HC (p = 0.001) and in ARAS patients than ADPKD patients (p = 0.004). CONCLUSIONS: The pathogenesis of hypertension in ADPKD and ARAS patients is related to the activation of the renin angiotensin aldosterone system (RAAS). In ADPKD, cyst enlargement leads to kidney ischemia and renin release, associated to endothelial dysfunction, low nitric oxide and sympathetic tone activation. Differently, reduction in renal perfusion pressure activates RAAS and renal adrenergic nerves in ARAS patients. We can speculate that prolonged QTc interval is more present in ARAS vs. ADPKD hypertensive patients due to a greater activation of RAAS. We suggest adding 24-hour HRV evaluation in association with traditional risk factors in course of ADPKD and ARAS hypertension to better stratify cardiovascular risk in these groups of patients.


Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Hipertensión/fisiopatología , Riñón Poliquístico Autosómico Dominante/fisiopatología , Obstrucción de la Arteria Renal/fisiopatología , Adulto , Anciano , Aterosclerosis/patología , Estudios de Casos y Controles , Electrocardiografía , Femenino , Humanos , Hipertensión/etiología , Masculino , Persona de Mediana Edad , Óxido Nítrico/metabolismo , Riñón Poliquístico Autosómico Dominante/complicaciones , Obstrucción de la Arteria Renal/complicaciones , Renina/metabolismo , Sistema Renina-Angiotensina/fisiología , Factores de Riesgo , Ultrasonografía Doppler
10.
Prog Cardiovasc Dis ; 65: 60-70, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33745915

RESUMEN

Renal artery stenosis is the most common secondary cause of hypertension and predominantly caused by atherosclerosis. In suspected patients, a non-invasive diagnosis with ultrasound is preferred. Asymptomatic, incidentally found RAS does not require revascularization. In symptomatic patients requiring revascularization, renal artery stenting is the preferred therapy. Selecting appropriate patients for revascularization requires careful consideration of lesion severity and is optimized with a multidisciplinary team. All patients with atherosclerotic RAS should be treated with guideline-directed medical therapy, including hypertension control, diabetes control, statins, antiplatelet therapy, smoking cessation and encouraging activity.


Asunto(s)
Procedimientos Endovasculares , Obstrucción de la Arteria Renal/terapia , Arteria Renal/cirugía , Injerto Vascular , Fármacos Cardiovasculares/uso terapéutico , Procedimientos Endovasculares/efectos adversos , Procedimientos Endovasculares/instrumentación , Ejercicio Físico , Humanos , Prevalencia , Arteria Renal/diagnóstico por imagen , Arteria Renal/fisiopatología , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/epidemiología , Obstrucción de la Arteria Renal/fisiopatología , Medición de Riesgo , Factores de Riesgo , Conducta de Reducción del Riesgo , Cese del Hábito de Fumar , Stents , Resultado del Tratamiento
12.
BMC Med Imaging ; 21(1): 21, 2021 02 10.
Artículo en Inglés | MEDLINE | ID: mdl-33568089

RESUMEN

BACKGROUND: Endovascular therapy is the gold standard in patients with hemodynamic relevant renal artery stenosis (RAS) resistant to medical therapy. The severity grading of the stenosis as well as the result assessment after endovascular approach is predominantly based on visible estimations of the anatomic appearance. We aim to investigate the application of color-coded DSA parameters to gain hemodynamic information during endovascular renal artery interventions and for the assessment of the procedures´ technical success. METHODS: We retrospectively evaluated 32 patients who underwent endovascular renal artery revascularization and applied color-coded summation imaging on selected monochromatic DSA images. The differences in time to peak (dTTP) of contrast enhancement in predefined anatomical measuring points were analyzed. Furthermore, differences in systolic blood pressure values (SBP) and serum creatinine were obtained. The value of underlying diabetes mellitus as a predictor for clinical outcome was assessed. Correlation analysis between the patients´ gender as well as the presence of diabetes mellitus and dTTP was performed. RESULTS: Endovascular revascularization resulted in statistically significant improvement in 4/7 regions of interest. Highly significant improvement of perfusion in terms of shortened TTP values could be found at the segmental artery level and in the intrastenotical segment (p < 0.001), significant improvement prestenotical and in the apical renal parenchyma (p < 0.05). In the other anatomic regions, differences revealed not to be significant. Differences between SBP and serum creatinine levels before and after the procedure were significant (p = 0.004 and 0.0004). Patients´ gender as well as the presence of diabetes mellitus did not reveal to be predictors for the clinical success of the procedure. Furthermore, diabetes and gender did not show relevant correlation with dTTP in the parenchymal measuring points. CONCLUSIONS: The supplementary use of color-coding DSA and the data gained from parametric images may provide helpful information in the evaluation of the procedures´ technical success. The segmental artery might be a particularly suitable vascular territory for analyzing differences in blood flow characteristics. Further studies with larger cohorts are needed to further confirm the diagnostic value of this technique.


Asunto(s)
Angiografía de Substracción Digital/métodos , Angioplastia , Interpretación de Imagen Radiográfica Asistida por Computador/métodos , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Algoritmos , Color , Femenino , Hemodinámica , Humanos , Masculino , Persona de Mediana Edad , Obstrucción de la Arteria Renal/fisiopatología , Estudios Retrospectivos , Adulto Joven
13.
Neurology ; 96(13): e1783-e1791, 2021 03 30.
Artículo en Inglés | MEDLINE | ID: mdl-33568546

RESUMEN

OBJECTIVE: To test the hypothesis that de novo genetic variants are responsible for moyamoya disease (MMD) in children with unaffected relatives, we performed exome sequencing of 28 affected children and their unaffected parents. METHODS: Exome sequencing was performed on 28 trios of affected patients with MMD and unaffected parents. RESULTS: We identified 3 novel rare de novo RNF213 variants, 1 in the RING domain and 2 in a highly conserved region distal to the RING domain (4,114-4,120). These de novo cases of MMD present at a young age with aggressive MMD and uniquely have additional occlusive vascular lesions, including renal artery stenosis. Two previously reported cases had de novo variants in the same limited region and presented young with aggressive MMD, and 1 case had narrowing of the inferior abdominal aorta. CONCLUSIONS: These results indicate a novel syndrome associated with RNF213 rare variants defined by de novo mutations disrupting highly conserved amino acids in the RING domain and a discrete region distal to the RING domain delimited by amino acids 4,114 to 4,120 leading to onset of severe MMD before 3 years of age and occlusion of other arteries, including the abdominal aorta, renal, iliac, and femoral arteries.


Asunto(s)
Adenosina Trifosfatasas/genética , Enfermedad de Moyamoya/genética , Ubiquitina-Proteína Ligasas/genética , Adulto , Edad de Inicio , Enfermedades de la Aorta/genética , Enfermedades de la Aorta/fisiopatología , Arteriopatías Oclusivas/genética , Arteriopatías Oclusivas/fisiopatología , Preescolar , Femenino , Arteria Femoral , Humanos , Arteria Ilíaca , Masculino , Enfermedad de Moyamoya/fisiopatología , Mutación , Obstrucción de la Arteria Renal/genética , Obstrucción de la Arteria Renal/fisiopatología
14.
Ann Vasc Surg ; 74: 287-293, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33548401

RESUMEN

BACKGROUND: The purpose of this study was to analyze our initial experience in renal artery reconstruction with heparin-bonded expanded polytetrafluoroethylene (ePTFE) grafts. METHODS: The authors retrospectively reviewed the data of consecutive patients who received open renal reconstruction with a heparin-bonded ePTFE graft at our institution between January 2014 and December 2019. RESULTS: A total of 22 renal reconstructions with a heparin-bonded ePTFE graft were performed in 17 consecutive patients. In all cases, renal reconstruction was a concomitant procedure during surgical or endovascular aortic procedures. Postoperative complications within 30 days were observed in 9 (53%) patients, including acute kidney injury (n = 6), pneumonia (n = 1), retrograde type B aortic dissection (n = 1), and lower limb ischemia (n = 1). The 30-day mortality rate was 0%. In a median follow-up period of 32 (19-39) months, all grafts were patent without re-intervention. Six patients with preoperative stage 2 chronic kidney disease progressed to stage 3 during follow-up. No patient required temporary or permanent hemodialysis. One patient died from intestinal ischemia at 23 months after surgery. CONCLUSIONS: This study showed that the patency after open renal reconstruction with a heparin-bonded ePTFE graft was excellent, with acceptable renal outcomes, and demonstrates its safety as a concomitant procedure during an aortic procedure. Heparin-bonded ePTFE grafts are a feasible and effective choice for open renal reconstruction in contemporary practice.


Asunto(s)
Anticoagulantes/administración & dosificación , Aneurisma de la Aorta Abdominal/cirugía , Aneurisma de la Aorta Torácica/cirugía , Implantación de Prótesis Vascular/instrumentación , Prótesis Vascular , Materiales Biocompatibles Revestidos , Heparina/administración & dosificación , Procedimientos de Cirugía Plástica/instrumentación , Politetrafluoroetileno , Obstrucción de la Arteria Renal/cirugía , Arteria Renal/cirugía , Anciano , Anticoagulantes/efectos adversos , Aneurisma de la Aorta Abdominal/diagnóstico por imagen , Aneurisma de la Aorta Abdominal/fisiopatología , Aneurisma de la Aorta Torácica/diagnóstico por imagen , Aneurisma de la Aorta Torácica/fisiopatología , Implantación de Prótesis Vascular/efectos adversos , Femenino , Heparina/efectos adversos , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Diseño de Prótesis , Procedimientos de Cirugía Plástica/efectos adversos , Arteria Renal/diagnóstico por imagen , Arteria Renal/fisiopatología , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/fisiopatología , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Grado de Desobstrucción Vascular
15.
J Vasc Surg ; 74(1): 79-89.e2, 2021 07.
Artículo en Inglés | MEDLINE | ID: mdl-33340698

RESUMEN

OBJECTIVE: To evaluate the outcomes of various surgical approaches in the treatment of renovascular hypertension and midaortic syndrome (MAS) in children. METHODS: We performed a retrospective medical record review of patients who had undergone surgery for renovascular hypertension from 2010 to 2018 at our center under the care of a multidisciplinary team. The operative interventions included mesenteric artery growth improves circulation (MAGIC), tissue expander-stimulated lengthening of arteries (TESLA), aortic bypass using polytetrafluorethylene, renal artery reimplantation, and autotransplantation. The MAGIC procedure uses the meandering mesenteric artery as a free conduit for aortic bypass. The TESLA procedure is based on lengthening the normal distal aorta and iliac arteries by gradual filling of a retroaortic tissue expander for several weeks, followed by resection of the stenotic aorta and subsequent primary reconstruction. RESULTS: A total of 39 patients were identified, 10 with isolated renal artery stenosis, 26 with MAS, and 3 with systemic inflammatory vasculitis. The median age at presentation and surgery was 6.4 years (range, 0-16.3 years) and 9.3 years (range, 0-9.2 years), respectively. The MAS-associated syndromes included neurofibromatosis type 1 (15.4%) and Williams syndrome (5.1%), although most cases were idiopathic. At surgery, 33.3% had had stage 1 hypertension (HTN), 53.8% stage 2 HTN, and 12.8% normal blood pressure with a median of three antihypertensive medications. Follow-up of 37 patients at a median of 2.5 years demonstrated normal blood pressure in 86.1%, stage 1 HTN in 8.3%, and stage 2 HTN in 5.6%, with a median of one antihypertensive medication for the entire cohort. CONCLUSIONS: The patterns of vascular involvement leading to renovascular hypertension in children are variable and complex, requiring thoughtful multidisciplinary planning and surgical decision-making. The MAGIC and TESLA procedures provide feasible approaches for aortic bypass and reconstruction using autologous tissues and will result in normalization of blood pressure in 85% of children 2.5 years after surgery.


Asunto(s)
Aorta/cirugía , Enfermedades de la Aorta/cirugía , Arteriopatías Oclusivas/cirugía , Hipertensión Renovascular/cirugía , Obstrucción de la Arteria Renal/cirugía , Procedimientos Quirúrgicos Vasculares , Adolescente , Factores de Edad , Aorta/diagnóstico por imagen , Aorta/fisiopatología , Enfermedades de la Aorta/complicaciones , Enfermedades de la Aorta/diagnóstico por imagen , Enfermedades de la Aorta/fisiopatología , Arteriopatías Oclusivas/complicaciones , Arteriopatías Oclusivas/diagnóstico por imagen , Arteriopatías Oclusivas/fisiopatología , Presión Sanguínea , Prótesis Vascular , Implantación de Prótesis Vascular/instrumentación , Niño , Preescolar , Femenino , Humanos , Hipertensión Renovascular/diagnóstico , Hipertensión Renovascular/etiología , Hipertensión Renovascular/fisiopatología , Arteria Ilíaca/fisiopatología , Arteria Ilíaca/cirugía , Lactante , Masculino , Arterias Mesentéricas/crecimiento & desarrollo , Arterias Mesentéricas/fisiopatología , Arterias Mesentéricas/trasplante , Arteria Renal/fisiopatología , Arteria Renal/cirugía , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/etiología , Obstrucción de la Arteria Renal/fisiopatología , Reimplantación , Estudios Retrospectivos , Síndrome , Factores de Tiempo , Expansión de Tejido/instrumentación , Dispositivos de Expansión Tisular , Trasplante Autólogo , Resultado del Tratamiento , Procedimientos Quirúrgicos Vasculares/efectos adversos , Procedimientos Quirúrgicos Vasculares/instrumentación
16.
J Cell Physiol ; 236(5): 4036-4049, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33151557

RESUMEN

Percutaneous transluminal renal angioplasty (PTRA) has been used to treat renovascular disease (RVD), a chronic condition characterized by renal ischemia and metabolic abnormalities. Mitochondrial injury has been implicated as a central pathogenic mechanism in RVD, but whether it can be reversed by PTRA remains uncertain. We hypothesized that PTRA attenuates mitochondrial damage, renal injury, and dysfunction in pigs with coexisting renal artery stenosis (RAS) and metabolic syndrome (MetS). Four groups of pigs (n = 6 each) were studied after 16 weeks of diet-induced MetS and RAS (MetS + RAS), MetS + RAS treated 4 weeks earlier with PTRA, and Lean and MetS Sham controls. Single-kidney renal blood flow (RBF) and glomerular filtration rate (GFR) were assessed in vivo with multidetector computed tomography, and renal tubular mitochondrial structure and function and renal injury ex vivo. PTRA successfully restored renal artery patency, but mean arterial pressure remained unchanged. Stenotic kidney RBF and GFR, which fell in MetS + RAS compared to MetS, rose after PTRA. PTRA attenuated MetS + RAS-induced mitochondrial structural abnormalities in tubular cells and peritubular capillary endothelial cells, decreased mitochondrial H2 02 production, and increased renal cytochrome-c oxidase-IV activity and ATP production. PTRA also improved cortical microvascular and peritubular capillary density and ameliorated tubular injury and tubulointerstitial fibrosis in the poststenotic kidney. Importantly, renal mitochondrial damage correlated with poststenotic injury and dysfunction. Renal revascularization attenuated mitochondrial injury and improved renal hemodynamics and function in swine poststenotic kidneys. This study suggests a novel mechanism by which PTRA might be relatively effective in ameliorating mitochondrial damage and improving renal function in coexisting MetS and RAS.


Asunto(s)
Angioplastia , Riñón/cirugía , Síndrome Metabólico/complicaciones , Síndrome Metabólico/cirugía , Mitocondrias/patología , Obstrucción de la Arteria Renal/complicaciones , Obstrucción de la Arteria Renal/cirugía , Animales , Células Endoteliales/patología , Células Endoteliales/ultraestructura , Fibrosis , Hemodinámica , Hipertensión/complicaciones , Hipertensión/fisiopatología , Riñón/irrigación sanguínea , Riñón/patología , Riñón/fisiopatología , Síndrome Metabólico/fisiopatología , Mitocondrias/ultraestructura , Estrés Oxidativo , Obstrucción de la Arteria Renal/fisiopatología , Porcinos
17.
Clin Exp Hypertens ; 43(1): 7-12, 2021 Jan 02.
Artículo en Inglés | MEDLINE | ID: mdl-32635757

RESUMEN

Background: Rare cases of concurrent primary aldosteronism (PA) and renal artery stenosis (RAS) have been reported. Methods: In this retrospective case-control study, we selected a cohort of 10 PA with RAS patients and a control group of 20 PA without RAS patients from January 1, 2006, to January 1, 2016.  Results: All patients presented with refractory hypertension, and a nonstatistically significant trend toward lower mean serum potassium was seen in the PA with RAS group (p =.07). PA with RAS patients had lower mean orthostatic aldosterone-to-renin ratios (38.4 ± 41.4 ng dL-1/ng mL-1 h-1 vs. 87.4.4 ± 38.4 ng dL-1/ng mL-1 h-1, respectively; p < .01) and a higher false-negative rate (50% vs. 15%, respectively; p < .05) compared with controls. All misdiagnosed patients had the diagnosis of PA confirmed when we revaluated the repeated screening and confirmative tests because of residual hypertension or hypokalemia after successful revascularization of renal artery stenosis.  Conclusions: PA is easily missed in patients with RAS because of the high false-negative rate for screening tests. RAS patients with residual hypertension after successful renal angioplasty should be monitored for coexisting PA. Reevaluation of screening and confirmatory tests is helpful in establishing the correct diagnoses.


Asunto(s)
Hiperaldosteronismo/fisiopatología , Hipertensión/fisiopatología , Hipopotasemia/sangre , Obstrucción de la Arteria Renal/fisiopatología , Adulto , Aldosterona/sangre , Estudios de Casos y Controles , Estudios de Cohortes , Errores Diagnósticos , Femenino , Humanos , Hiperaldosteronismo/sangre , Hiperaldosteronismo/complicaciones , Hiperaldosteronismo/diagnóstico , Hipertensión/etiología , Hipopotasemia/etiología , Masculino , Tamizaje Masivo , Persona de Mediana Edad , Obstrucción de la Arteria Renal/sangre , Obstrucción de la Arteria Renal/complicaciones , Obstrucción de la Arteria Renal/diagnóstico , Renina/sangre , Estudios Retrospectivos
18.
J Vis Exp ; (164)2020 10 26.
Artículo en Inglés | MEDLINE | ID: mdl-33165324

RESUMEN

Renal artery stenosis is a common condition in patients with coronary or peripheral vascular disease where the renin angiotensin aldosterone system (RAAS) is overactivated. In this context, there is a narrowing of the renal arteries that stimulate an increase in the expression and release of renin, the rate-limiting protease in RAAS. The resulting rise in renin expression is a known driver of renovascular hypertension, frequently associated with kidney injury and end organ damage. Thus, there is a great interest in developing novel treatments for this condition. The molecular and cellular mechanism of renin control in renal artery stenosis is not fully understood and warrants further investigation. To induce renal artery stenosis in mice, a modified 2 kidney 1 clip (2K1C) Goldblatt mouse model was developed. The right kidney was stenosed in wild type mice and sham operated mice were used as control. After renal artery stenosis, we determined renin expression and kidney injury. Kidneys were harvested, and fresh cortices were used to determine protein and mRNA expression of renin. This animal model is reproducible and can be used to study pathophysiological responses, molecular and cellular pathways involved in renovascular hypertension and kidney injury.


Asunto(s)
Lesión Renal Aguda/diagnóstico , Modelos Animales de Enfermedad , Riñón/cirugía , Lipocalina 2/metabolismo , Obstrucción de la Arteria Renal/fisiopatología , Arteria Renal/fisiopatología , Renina/metabolismo , Lesión Renal Aguda/etiología , Lesión Renal Aguda/metabolismo , Animales , Presión Sanguínea , Femenino , Riñón/metabolismo , Masculino , Ratones , Ratones Endogámicos C57BL , Obstrucción de la Arteria Renal/complicaciones , Obstrucción de la Arteria Renal/metabolismo
19.
J Med Vasc ; 45(5): 294-297, 2020 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-32862988

RESUMEN

The transplant renal artery stenosis occurred up to 23% after kidney transplantation. Endovascular treatment has become the treatment of choice of this complication, but could be challenging in immediate postoperative anastomotic narrowing. We report the case of a adolescent with early renal graft artery stenosis located in the anastomosis and diagnosed 3 days after renal transplantation, with necessity of haemodialysis. This complication was treated by angioplasty on the 25th day, using a polymer free drug eluting stent with a good result. We discussed the precautions and technical features of this procedure.


Asunto(s)
Angioplastia/instrumentación , Stents Liberadores de Fármacos , Trasplante de Riñón/efectos adversos , Obstrucción de la Arteria Renal/terapia , Adolescente , Humanos , Masculino , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/etiología , Obstrucción de la Arteria Renal/fisiopatología , Resultado del Tratamiento , Grado de Desobstrucción Vascular
20.
Hypertension ; 76(2): 497-505, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32507040

RESUMEN

The significance of peristenotic collateral circulation (PCC) development around a stenotic renal artery is unknown. We tested the hypothesis that PCC is linked to loss of kidney function and recovery potential in patients with atherosclerotic renovascular disease (ARVD). Thirty-four patients with ARVD were assigned to medical-therapy with or without revascularization based on clinical indications. The PCC was visualized using multidetector computed tomography and defined relative to segmental arteries in patients with essential hypertension. PCC number before and 3 months after treatment was correlated with various renal parameters. Thirty-four stenotic kidneys from 30 patients were analyzed. PCC number correlated inversely with kidney volume. ARVD-stenotic kidneys with baseline PCC (collateral ARVD [C-ARVD], n=13) associated with elevated 24-hour urine protein and stenotic kidney vein level of tumor necrosis factor-α, lower single-kidney volume and blood flow, and greater hypoxia than in stenotic kidneys with no PCC (no collateral ARVD [NC-ARVD], n=17). Revascularization (but not medical-therapy alone) improved stenotic kidney function and reduced inflammation in both NC-ARVD and C-ARVD. In C-ARVD, revascularization also increased stenotic kidney volume, blood flow, and oxygenation to levels comparable to NC-ARVD, and induced PCC regression. However, revascularization improved systolic blood pressure, plasma renin activity, and filtration fraction only in NC-ARVD. Therefore, patients with C-ARVD have greater kidney dysfunction, atrophy, hypoxia, and inflammation compared with patients with NC-ARVD, suggesting that PCC does not effectively protect the stenotic kidney in ARVD. Renal artery revascularization improved in C-ARVD stenotic kidney function, but not hypertension or renin-angiotensin system activation. These observations may help direct management of patients with ARVD.


Asunto(s)
Aterosclerosis/fisiopatología , Circulación Colateral/fisiología , Enfermedades Renales/fisiopatología , Riñón/fisiopatología , Obstrucción de la Arteria Renal/fisiopatología , Circulación Renal/fisiología , Anciano , Aterosclerosis/diagnóstico por imagen , Presión Sanguínea/fisiología , Femenino , Tasa de Filtración Glomerular/fisiología , Humanos , Riñón/diagnóstico por imagen , Enfermedades Renales/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Tomografía Computarizada Multidetector , Obstrucción de la Arteria Renal/diagnóstico por imagen
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