Improving outcomes in atherosclerotic renovascular disease: importance of clinical presentation and multi-disciplinary review.

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.

A Nine-year-old Boy with Renovascular Hypertension: Knowing Where to Look.

Renal artery entrapment (RAE) by hypertrophic diaphragmatic crura is an extremely rare cause of renovascular hypertension (RVH). Here, we report the case of a 9-year-old boy diagnosed with RVH caused by right RAE by a hypertrophic diaphragmatic crus and successfully managed with close medical monitoring. Diagnosis of this entity is easily overlooked if the optimal views are not obtained during imaging, which depends on a high index of suspicion. We would like to remind clinicians to keep this rare condition in mind when evaluating children with RVH.

Coarctação de aorta abdominal justarrenal associada à hipertensão renovascular e claudicação incapacitante: tratamento utilizando ponte ilíaco-birrenal e técnica "paving and cracking" / Coarctation of the abdominal aorta associated with renovascular hypertension and incapacitating claudication: repair with iliac-birenal bypass and paving and cracking technique

Resumo Coarctação da aorta abdominal é uma causa rara de claudicação de membros inferiores e hipertensão refratária. O tratamento é complexo e exige conhecimento de diversas técnicas de reconstrução vascular. Apresentamos um caso de coarctação ao nível das artérias renais, seu tratamento e revisão da literatura. Paciente feminina, 65 anos, com hipertensão refratária desde os 35 anos, utilizando cinco medicações anti-hipertensivas em dose máxima. Pressão arterial média de 260/180mmHg e claudicação incapacitante (menos de 20 metros) bilateral. Angiotomografia computadorizada demonstrou coarctação de aorta justarrenal de 4 mm de maior diâmetro, calcificação circunferencial no local da estenose e tortuosidade da aorta infrarrenal. Foi submetida a tratamento híbrido, com ponte ilíaco-birrenal e implante de stent Advanta V12 no local da estenose. A paciente evoluiu satisfatoriamente e, 60 dias depois da cirurgia, apresentava-se com uma pressão arterial de 140/80mmHg, em uso de apenas duas medicações anti-hipertensivas e sem claudicação.

Abstract Coarctation of the abdominal aorta is a rare etiology of intermittent claudication and refractory hypertension. Treatment is complex and requires knowledge of several vascular reconstruction techniques. We report a case of aortic coarctation at the level of the renal arteries, describing its treatment and presenting a literature review. Female patient, 65 years old, with refractory hypertension since the age of 35, using five antihypertensive medications at maximum doses. Blood pressure was 260/180mmHg and she had disabling claudication (less than 20 meters). Computed tomography angiography showed a 4mm coarctation in the juxtarenal aorta, with circumferential calcification at the stenosis site, and tortuous infrarenal aorta. Hybrid repair was performed with an iliac-birenal bypass and implantation of an Advanta V12 stent at the stenosis site. The patient's postoperative course was satisfactory, she was free from claudication, and her blood pressure 60 days after surgery was 140/80mmHg, taking two antihypertensive medications.

Hipertensión Arterial Secundaria ¿por dónde empezar? / Secondary Arterial Hypertension, where to start?

30 to 40% of the adult population worldwide has been diagnosed with hypertension, among these patients 5 to 10% of them could have a possibly curable condition. In order to recognize this special population, the clinician must perform a complete work up and be aware of the main underlying causes of secondary hypertension. Often this could be a goal difficult to accomplish. The purpose of this article is to discuss the most frequent causes of secondary hypertension and offer a diagnostic approach for these patients. Clinicians should never forget that drug-related hypertension is a common cause that is discovered only with the help of a good medical history.

Infarto renal bilateral: una rara complicación de la displasia fibromuscular / Bilateral renal infarction: A rare complication of fibromuscular dysplasia

El infarto renal bilateral como complicación de la displasia fibromuscular (DFM) es extremadamente raro, y hasta el momento se han informado pocos casos. Reportamos el caso de un hombre de 44 años que presentó dolor abdominal e hipertensión severa. La tomografía computarizada de abdomen reveló infarto renal bilateral, y una angiografía renal confirmó la estenosis bilateral e irregular debida a DFM, en ausencia de vasculitis sistémica y otros factores de riesgo para eventos tromboembólicos. Por lo tanto, se realizó una angioplastia con balón para obtener una buena permeabilidad del vaso y mejorar la permeabilidad en el seguimiento a largo plazo. Después del tratamiento endovascular la presión arterial mejoró notablemente, manteniendo este resultado a los 3 meses de seguimiento. Nuestro caso destaca la importancia de reconocer el infarto renal como una presentación inicial de la DFM

Bilateral renal infarction complicating Fibromuscular Dysplasia (FMD) is extremely rare and has so far been reported only in a handful of cases. We report a 44-year-old man who presented with complaints of abdominal pain and severe hypertension. Computed tomography of the abdomen revealed bilateral renal infarction and a renal angiography confirmed the bilateral and irregular stenosis due to FMD, in the absence of systemic vasculitis and other risk factors for thromboembolic events. Therefore, angioplasty with balloon was performed in order to obtain a good vessel patency and to improve patency in the long term follow-up. After the endovascular treatment the blood pressure improved markedly, maintaining this result at 3-months follow-up. Our case highlight the importance of recognizing renal infarction as an initial presentation of FMD

Obliteración de la arteria ilíaca como causa de hipertensión renovascular en el paciente trasplantado renal, un diagnóstico difícil y poco frecuente / Iliac artery obliteration as a cause of renovascular hypertension in kidney graft recipients: A difficult and uncommon diagnosis

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Hipertensión arterial secundaria a displasia fibromuscular de la arteria renal. A propósito de un caso / Hypertension secondary to fibromuscular dysplasia of the renal artery. Report of a case

Se presenta el caso clínico de una paciente que debutó con hipertensión arterial (HTA) grado II realizándose los estudios sistematizados correspondientes para descartar patologías secundarias. Dentro de los estudios de primera línea en la valoración merece destacarse la ecografía Doppler renal por la información que ella aporta, orientado al diagnóstico de estenosis de la arteria renal. La identificación de una HTA de origen renovascular supone el hallazgo de una de las causas reversibles de hipertensión. Dentro de esta etiología la más frecuente es la displasia fibromuscular (DFM) que mejora con la corrección de la estenosis mediante técnicas quirúrgicas o percutáneas con angioplastia con balón o stent. El objetivo es presentar un caso clínico para mostrar la complejidad del proceso diagnóstico y la importancia que tiene la sospecha clínica para la corrección oportuna de este tipo de HTA reversible...

¿Es la edad un factor predictor en el desarrollo de hipertensión en pacientes con oclusión unilateral de la arteria renal secundario a trauma abdominal contuso que fueron tratados conservadoramente? / Is age a predictor for the development of hypertension in conservatively managed unilateral renal artery occlusion secondary to blunt abdominal trauma?

Objetivo: Presentar 2 casos de oclusión de la arteria renal unilateral secundario a trauma abdominal contuso manejados conservadoramente y revisar, en la literatura, el rol de la edad como factor predictor en el desarrollo de hipertensión. Material y métodos: Masculino y femenina de 14 y 12 años, respectivamente, participaron en accidentes automovilísticos diferentes sin portar cinturón de seguridad permaneciendo en el asiento. Ambos arribaron a urgencias pediatría con signos vitales normales. Ambas tomografías revelaron riñón sin captación de contraste. El manejo fue conservador. Resultados: Posterior a 38 y 36 meses de seguimiento, respectivamente, ninguno presenta hipertensión o daño en la función renal. Encontramos solo 7 casos, en la literatura, con edad ≤16 manejados conservadoramente. En total 9, existió hipertensión en 2(22%). Conclusiones: No se puede llegar a una conclusión contundente con una muestra tan pequeña aunque existe evidencia suficiente que soporta el manejo conservador del padecimiento a cualquier edad (AU)

Objective: To present 2 cases with unilateral renal artery occlusion after blunt trauma conservatively treated and review, in the literature, the role of age as a prognostic factor for the development of hypertension. Material and methods: A 14 and 12 y/o male and female passengers, who were unrestrained in the back seat of a car, were involved in different motor vehicle accidents. Both remained in the seat and arrived at the Pediatric Emergency Department with normal vital signs. Computerized tomography scans showed unenhancing kidneys. Conservative management was advocated. Results: After 38 and 36 months follow-up, respectively, normal renal function and arterial pressure. We found only 7 cases in the literature where age ≤16 was reported and treatment was conservative. Nine in total, hypertension developed in 2(22%). Conclusions: No conclusion could be made with such a small sample. There is enough evidence in the literature supporting conservative management at all ages (AU)

Aneurisma de aorta abdominal y enfermedad vascular renal / Abdominal aortic aneurysm and renovascular disease

Los recientes avances tecnológicos aplicados al diagnóstico y tratamiento del aneurisma de aorta abdominal (AAA) y la enfermedad vascular renal (EAR) están ocasionando un cambio en el abordaje terapéutico de los pacientes que experimentan estas enfermedades. La prevalencia de ambas enfermedades tiene tendencia a aumentar. Ello se debe a la prolongación de la esperanza de vida en los pacientes que presentan arteriosclerosis, factor etiopatogénico fundamental de ambas enfermedades. La aplicación de métodos de diagnóstico por la imagen en enfermedades no vasculares permite el diagnóstico precoz de AAA. La sospecha de EAR se puede confirmar fácilmente con el uso de elementos diagnósticos de alta definición, como la angiotomografía o la angiorresonancia. El tratamiento intervencionista endovascular está sustituyendo con éxito a los tratamientos de reparación quirúrgica convencionales. Todo ello abre la posibilidad de mejorar el pronóstico de ambas enfermedades con actuaciones efectivas y poco cruentas. Las futuras innovaciones tecnológicas permitirán la aplicación de técnicas endovasculares en la amplia mayoría de los pacientes con AAA o EAR (AU)

Recent technological advances in the diagnosis and therapy of abdominal aortic aneurysm and renovascular disease are continuing to bring about changes in the way patients suffering from these conditions are treated. The prevalence of both these conditions is increasing. This is due to greater life-expectancy in patients with arteriosclerosis, a pathogenetic factor underlying both conditions. The application of diagnostic imaging techniques to non-vascular conditions has led to the early diagnosis of abdominal aortic aneurysm. Clinical suspicion of reno-vascular disease can be confirmed easily using high-resolution diagnostic imaging modalities such as CT angiography and magnetic resonance angiography. Endovascular intervention is successfully replacing conventional surgical repair techniques, with the result that it may be possible to improve outcome in both conditions using effective and minimally invasive approaches. Future technological developments will enable these endovascular techniques to be applied in the large majority of patients with abdominal aortic aneurysm or renovascular disease (AU)

Avanços na avaliação e no tratamento da hipertensão renovascular / Advances on evaluation and treatment of renovascular hypertension

A hipertensão renovascular é secundária à isquemia renal. É causada principalmente por doença aterosclerótica da artéria renal e displasia fibromuscular. A ativação do eixo renina-angiotensina-aldosterona e suas conseqüências hemodinâmicas é o principal fator fisiopatológico. A investigação diagnóstica está indicada em pacientes com características clínicas sugestivas da doença. A avaliação é feita através de métodos não-invasivos, como a ultra-sonografia com Doppler, a ressonância nuclear magnética, a tomografia computadorizada espiral e a cintilografia renal com captopril. A avaliação invasiva com a arteriografia permanece o padrão-ouro no diagnóstico da hipertensão renovascular. Há duas escolhas possíveis de tratamento: o medicamentoso e a revascularização cirúrgica ou percutânea. As indicações mais claras de angioplastia em estenoses hemodinamicamente significativas são a displasia fibromuscular e, no caso de doença aterosclerótica, hipertensão não-controlada, perda de função renal progressiva e edema pulmonar recorrente sem causa cardíaca identificada.

Revascularización eficaz de obstrucción total de arterial renal mediante angioplastia y colocación de Stent / Revascularization of total renal artery occlusion by angioplasty and stent placement

La revascularización de la estenosis de la arteria renal para el tratamiento de la hipertensión es un procedimiento ya admitido. En escenarios clínicos seleccionados, el éxito del procedimiento de revascularización puede mantener o restaurar la función renal. Presentamos el caso clínico de un paciente varón de 66 años con hipertensión arterial secundaria y deterioro de función renal causada por arteriosclerosis bilateral de las arterias renal (obstrucción completa de la arteria renal izquierda y estenosis suboclusiva de la derecha) en el cual la presión arterial fue controlada con éxito y la función renal mejoró después de una angioplastia transluminal percutánea bilateral y colocación de sendos stents de arterias renales

Revascularization of renal artery stenosis for the treatment of hypertension is an established procedure. In selected clinical scenarios, successful revascularization procedures may preserve or restore renal function. We present a 66-year-old man with secundary hypertension and deteriorating renal function caused by bilateral atherosclerotic renal artery disease (complete obstruction of the left renal artery and subocclusive stenosis of the right) in which blood pressure was successfully controlled and renal function improved and maintained steady after bilateral percutaneus transluminal angioplasty and renal artery stenting

Increased arterial distensibility and renovascular hypertension in Goldenhar syndrome

Relatamos a associação de hipertensão renovascular por estenose de artéria renal e a Sindrome de Goldenhar (variante da displasia oculoauriculovertebral) em uma paciente do sexo feminino de 13 anos de idade. Este é o primeiro relato de tratamento por angioplastia. Além disso, detectamos por métodos não invasivos um aumento da distensibilidade arterial, a despeito da hipertensão arterial. A similaridade destes achados com outras doenças genéticas sugere que as alterações vasculares presentes podem estar relacionadas à Síndrome de Goldenhar.

Hipertensión renovascular en la infancia / Renovascular hypertension in children

El objetivo de este trabajo es conocer los datos epidemiológicos, etiología, clínica al diagnóstico, tratamiento y evolución de la hipertensión arterial renovascular en nuestro medio. Se realizó una revisión retrospectiva de los niños con esta enfermedad diagnosticados en el Hospital Universitario Materno-Infantil de Canarias, de enero de 1993 a diciembre de 2001.Seis pacientes, con edades comprendidas entre los cuatro meses y los siete años, fueron diagnosticados de hipertensión renovascular. La clínica fue inespecífica en cinco pacientes (83,3%). Se demostraron complicaciones cardiacas en todos los pacientes. Los diagnósticos establecidos fueron: 4 pacientes con displasia fibromuscular, 1 con neurofibromatosis, y 1 con síndrome de aorta abdominal. Se efectuaron seis angioplastias percutáneas trasluminales (APT): cuatro como primera elección y dos en segunda alternativa. Tres pacientes recibieron tratamiento médico. Dos niños precisaron nefrectomía para control de la hipertensión arterial. El tiempo medio de seguimiento en consulta fue de 5,8 años (2,5-8 años). Se perdió el seguimiento de un paciente. Dos pacientes (20%) precisan tratamiento farmacológico para control de la presión arterial. Se recomienda valorar la APT como tratamiento de elección de la hipertensión renovascular. Se produjo una alta incidencia de complicaciones neurológicas y cardiacas. Una tercera parte de los pacientes quedaron monorrenos, aunque todos mantienen una función renal dentro de la normalidad

The aim of this study was to assess the epidemiological data, etiology, diagnosis, treatment and course of arterial renovascular hypertension in our pediatric population. For this purpose, we carried out a retrospective review of the children diagnosed as having renovascular hypertension in our hospital between January 1993 and December 2001.Six patients, ranging in age between 4 months and 7 years old, were diagnosed as having renovascular hypertension. The clinical findings were nonspecific in 5 patients (83.3%). All six patients had cardiac complications. The diagnosis was fibromuscular dysplasia in four cases, neurofibromatosis in one and abdominal aorta syndrome in one. All the patients underwent percutaneous transluminal angioplasty (PTA), as the treatment of first choice in four and as a second option in the other two. Three patients received medical treatment. Two of the children required nephrectomy to control arterial hypertension. The mean length of outpatient follow-up is 5.8 years (range: from 2.5 to 8 years). One patient was lost to follow-up. Two patients required pharmacological treatment in order to control their blood pressure. We recommend that PTA be considered as the possible treatment of choice for renovascular hypertension. We report a high incidence of neurological and cardiac complications. One third (n= 2) of our patients have only one kidney, although all of them present normal renal function

Síndrome antifosfolipídico primario e HTA maligna / Antiphospholipid síndrome associated to malignat hypertension

El síndrome antifosfolipídico viene definido por la presencia de anticuerpos antifosfolípidos y trombosis de repetición, siendo más prevalentes las trombosis venosas que las arteriales. La afectación renal en el síndrome antifosfolipídico primario es poco frecuente, oscilando entre un 20-25% de los casos y siendo la trombosis de la arteria renal principal un acontecimiento raramente descrito. Presentamos el caso de una mujer de 39 años, con historia previa de trombosis de repetición y diagnosticada de síndrome antifosfolipídico primario, que desarrolló hipertensión arterial maligna en el contexto de una oclusión de la arteria renal. Presentaba anticuerpos anticardiolipina IgG positivos a títulos elevados, anticoagulante lúpico positivo. El renograma isotópico objetivó una asimetría en la captación renal (60% a nivel del riñón derecho y un 40% a nivel del riñón izquierdo). La arteriografía renal detectó una trombosis pre-oclusiva de la arteria renal izquierda. Se consiguió un control óptimo de la presión arterial mediante el tratamiento con inhibidores de la ECA y alfa-bloqueantes (AU)

The antiphospholipid syndrome is defined by the presence of antiphospholipid antibodies and recurrent thrombosis, affecting the venous system more frequently than the arterial one. Renal involvement is only observed in approximatelly 20- 25% of cases, main renal artery thrombosis has been exceptionally described. We report a 39 year old woman with previous history of recurrent thrombosis diagnosed as primary antiphospholipid syndrome, who presented malignant hypertension in the context of a renal artery thrombosis. She had a high IgG anticardiolipin antibodies title and positive lupic anticoagulant. An isotopic renogram demonstrated assymetrical activity (60% right vs 40% left kidney). Renal arteriography demonstrated preoclusive thrombosis in the left renal artery. Blood pressure was well controlled by the use of ACE-inhibitor and alfablockers (AU)

Fracaso renal en paciente VIH / HI and Renal Failure

Los avances en los últimos años en el tratamiento de la infección por VIH, han logrado incrementar la supervivencia de estos pacientes. Esta mejoría en el pronóstico se ha seguido de un aumento de la prevalencia de la patología renal asociada a la infección por VIH. Presentamos el caso de un paciente, con infección por VIH conocida, que desarrolló un cuadro de fracaso renal. En la biopsia renal se objetivaron lesiones secundarias a hipertensión arterial maligna y microangiopatía trombótica en varios estadios evolutivos. El estudio analítico permitió el diagnóstico de síndrome antifosfolípido. Revisamos la importancia de un diagnóstico precoz del síndrome antifosfolípido por sus importantes implicaciones pronósticas y su posible relación con la infección por VIH (AU)

Advances in the HIV infection’s treatment in the last year have improved the survival of these patients. This change in the better prognosis has been followed by a higher prevalence of kidney disease with these patients. We present a patient with a known HIV infection that developed renal insufficiency. The renal biopsy showed lesions of thrombotic microangiopathy and malignant hypertension with different of vascular and glomerular damage stages. The laboratory studies allowed us the diagnosis of anti phospholipid syndrome. Early diagnosis of the antiphospholipid syndrome in patients with HIV infection is important, because it can have serious implications in the prognosis (AU)