Impact of interventional therapy on top of standard drug therapy on left ventricular structure and function in HFrEF patients complicating with middle aortic syndrome caused by Takayasu arteritis / 中华心血管病杂志

Objective: To evaluate the impact of interventional therapy on top of drug therapy on cardiac function and structure in heart failure with reduced ejection fraction (HFrEF) patients complicating with middle aortic syndrome caused by Takayasu arteritis (TA-MAS). Methods: It was a retrospective longitudinal study. The data of patients with TA-MAS and HFrEF, who received interventional therapy on top of drug therapy in Fuwai Hospital from January 2010 to September 2020, were collected and analyzed. Baseline clinical data (including demographic data, basic treatment, etc.) were collected through the electronic medical record system. Changes of indexes such as New York Heart Association (NYHA) classification, N-terminal pro-brain natriuretic peptide (NT-proBNP), left ventricular end diastolic diameter (LVEDD), left ventricular ejection fraction (LVEF), left ventricular mass index (LVMI) before and after therapy were analyzed. Results: A total of 10 patients were collected. There were 8 females in this patient cohort, age was (18.4±5.0) years and onset age was (15.3±5.0) years. All 10 patients received standard heart failure medication therapy in addition to hormone and/or immunosuppressive anti-inflammatory therapy, but cardiac function was not improved, so aortic balloon dilatation and/or aortic stenting were performed in these patients. The median follow-up was 3.3(1.3, 5.6) years. On the third day after interventional therapy, the clinical symptoms of the 10 patients were significantly improved, NYHA classfication was restored from preoperative Ⅲ/Ⅳ to Ⅱ at 6 months post intervention(P<0.05). Compared with preoperation, NT-proBNP (P=0.028), LVEDD (P=0.011) and LVMI (P=0.019) were significantly decreased, LVEF was significantly increased (P<0.001) at 6 months after operation. Compared with preoperation, NT-proBNP (P=0.016), LVEDD (P=0.023) and LVMI (P=0.043) remained decreased, LVEF remained increased (P<0.001) at 1 year after operation. Conclusion: Results from short and medium term follow-up show that interventional therapy on top of heart failure drug therpay can effectively improve left cardiac function and attenuate cardiac remodeling in patients with TA-MAS comorbid with HFrEF.

Annuloaortic ectasia and arteritis: clinical features, treatments of choice, and causative relations

Abstract The roles that aortitis plays in the development of annuloaortic ectasia (AAE) remain uncertain, while clinical features of AAE in arteritis are largely unknown. This study was designed to highlight the clinical features of AAE, the treatments of choice, and the causative relations between aortitis and AAE. The morphology of the aortic valve leaflets was normal in half of the patients, while the valves were thin and overstretched in the other half. Most patients had an aortic aneurysm. Half of the patients had severe aortic valve insufficiency, and one-quarter of them had dilation of the sinuses of Valsalva. Takayasu arteritis was prone to develop coronary artery lesions, whereas giant cell arteritis were not. Aortic branch lesions in Takayasu arteritis were stenotic or occlusive in 92.9% of the patients, while in giant cell arteritis, they were all dilated lesions. Most patients (94.7%) required surgical treatment with steroid therapy. However, long-term follow-up results showed a higher anastomotic dehiscence rate, particularly in patients with Takayasu arteritis. Further morphometric and pathological research on AAE in arteritis should be undertaken, and more feasible measures should be warranted for preventing postoperative anastomotic dehiscence.

Taquicardia persistente en mujer joven deportista / A young woman with persistent tachycardia

Resumen: Una mujer joven, deportista, evoluciona con compromiso del estado general y síntomas constitucionales, asociado a taquicardia persistente y baja de peso de casi un año de evolución, con laboratorio, electrocardiograma y ecocardiograma inicialmente anodinos.

Abstracts: An athletic young woman presented with almost one year of general symptoms, weight loss, and persistent tachycardia. The initial laboratory, electrocardiogram and echocardiogram findings were unremarkable.

Tratamiento endovascular en arteritis de Takayasu: Presentación de un caso clínico / Endovascular intervention in Takayasu Arteritis: Case report

Una paciente de cuatro años concurrió a la Guardia por edemas e hipertensión arterial. Al momento del examen físico, se detectó disminución del pulso en el brazo izquierdo y ausente en los miembros inferiores, con diferencia de tensión arterial mayor de 10 mmHg entre los miembros superiores. Se realizó un ecocardiograma, en el que se observó coartación grave de la aorta a nivel abdominal y disfunción ventricular. Con la sospecha de arteritis de Takayasu, se completaron estudios con angiotomografía cardíaca, que confirmó una desconexión de la arteria subclavia izquierda y estrechez grave en la aorta abdominal. El diagnóstico definitivo fue de arteritis de Takayasu. Se realizó una angioplastía con estent por Hemodinamia en la aorta abdominal, con buenos resultados posteriores. Mejoraron los pulsos de los miembros inferiores, y se obtuvieron valores normales de la tensión arterial. En el ecocardiograma, mejoró la función ventricular, y el gradiente en la zona de coartación se redujo significativamente.

A four year old patient with no medical history was admitted to our hospital's Emergency Department, suffering from edema and hypertension. During physical examination a low pulse was detected in the left arm and a lack of pulses in lower limbs, with a blood pressure difference greater than 10 mm Hg between both arms. An echocardiogram demonstrated severe aortic abdominal coarctation and ventricular dysfunction. Takayasu arteritis was diagnosed and cardiac angiography was performed. Disconnection of left subclavian artery and severe tightness at the abdominal aorta were confirmed. The definitive diagnosis was Takayasu arteritis. Angioplasty with stent was performed in abdominal aorta, with good subsequent results. The pulses improved in the lower limbs, and normal blood pressure values without gradient between all members were registered. The echocardiogram improved ventricular function and the gradient in the coarctation area was significantly reduced.

Arteritis de Takayasu en lactante: caso clínico / Takayasu arteritis in childhood: a clinical case

Background: Takayasu arteritis (TA) is a large vessel systemic vasculitis, affecting the aorta and its main branches, leading to stenosis, thrombosis, and aneurysm formation. Incidence of 2.6 cases per million per year has been reported. TA is rare condition in children. Steroids are the main therapy for active disease, however, additional immunosuppressive agents are required in 50 percent of the patients. Objective: To report a case of TA in a young infant, presenting with intestinal necrosis. Case Report: 12 month-old male diagnosed with TA at 6 month-old, treated with second line immunosupressors. He presented with 3 days of abdominal pain, images showed aneurysm and thrombosis of the superior mesenteric artery. Small bowel ischemia was confirmed. The patient underwent multiple surgical interventions with bowel resection, leaving 30 cm of jejunum and 10 cm of terminal ileum. Anticoagulant therapy was started immediately. Intestinal continuity was restored 6 weeks later, patients was discharge home 3 months after surgery in full enteral intake, anticoagulant therapy, and maintains immunosupressor therapy. Discussion: TA in young infants is uncommon, and they can present atypical clinical manifestations. These patients are a real challenge, requiring multidisciplinary care to avoid further morbidities related to the vasculitis.

Introducción: La Arteritis de Takayasu (AT) es una vasculitis de grandes vasos, que afecta a la aorta y sus ramas principales, llevando a estenosis, trombosis, y formación de aneurismas. Se ha reportado una incidencia de 2.6 casos por millón por año, siendo de baja incidencia en niños. La terapia de elección son los corticoides, seguidos de inmunosupresores en casos resistentes. Objetivo: Reportar el caso de un lactante con AT severa, que cursó con necrosis intestinal. Caso clínico: Varón, 12 meses de edad, diagnóstico de AT a los 6 meses, en tratamiento de segunda línea con inmunosupresores. Presentó cuadro de 3 días de evolución de dolor abdominal, imágenes mostraron trombosis de arteria mesénterica superior aneurismática, desarrollando isquemia intestinal. Fue sometido a múltiples intervenciones quirúrgicas con resección intestinal, con 30 cm de yeyuno y 10 cm de ileon terminal remanente. Inició precozmente tratamiento anticoagulante. La reconstitución de tránsito intestinal fue 6 semanas más tarde, paciente fue dado de alta 3 meses después alimentándose completamente por vía enteral, con tratamiento anticoagulante e inmunosupresor ambulatorio. Conclusión: LaAT en lactantes es rara, y las manifestaciones clínicas pueden ser atípicas. Estos pacientes son un desafío para los equipos médicos, y requirien un manejo multidisciplinario para evitar complicaciones mayores.

Arteritis de Takayasu: informe de un caso con compromiso supraaórtico y tratamiento quirúrgico / Takayasu's arteritis: report of a case with supraaortic compromise and surgical treatment

La arteritis de Takayasu es una vasculitis inflamatoria crónica de grandes vasos. Fue descrita por primera vez por el oftalmólogo japonés Nikito Takayasu, en el año 1908. Presentamos un caso de arteritis de Takayasu, diagnosticada clínicamente de acuerdo a los criterios del American College of Rheumatology de 1990. Se trata de una paciente de 23 años que ingresa al Hospital Regional de Talca por un accidente vascular encefálico hemisférico y sintomatología de robo subclavio. Se realiza un angio TAC que muestra compromiso severo (oclusión total) del tercio medio y distal del tronco braquiocefálico, carótida común derecha e inicio de la subclavia derecha. Además, este examen demuestra un flujo retrógrado en la arteria vertebral derecha, compatible con el síndrome de robo subclavio. Se decide corregir la oclusión subclavia mediante angioplastía, sin éxito tanto en el abordaje anterogrado como retrógrado. Finalmente se opta por un bypass protésico (politetrafluoroetileno expandido) desde la carótida común izquierda a la subclavia derecha a través de un túnel subcutáneo en la cara anterior del cuello.

Takayasu's arteritis is a chronic inflammatory vasculitis of large vessels. It was first identified by Japanese ophthalmologist Nikito Takayasu, in 1908. A case of Takayasu's arteritis is presented, clinically diagnosed according to the criteria of the American College of Rheumatology of 1990. This is a 23 year oíd patient, entering Talca's Regional Hospital for a stroke, also presenting symptoms of subclavian steal. An angio CT was made, showing severe commitment (total occlusion) in the middle and distal third of the brachiocephalic trunk, right common carotid and the beginning of right subclavian artery. In addition, this test shows retrograde flow on the right vertebral artery, compatible with the subclavian steal syndrome. It was decided to correct the subclavian occiusion through angioplasty, without success, in both the anterograde and retrograde approach; finally opting for a prosthetic bypass (expanded polytetrafluoroethylene) from left common carotid to right subclavian artery through a tunnel of the subcutaneous front of the neck.

Arterite de Takayasu: aspectos clínicos e terapêuticos em 36 pacientes / Takayasu's arteritis: clinical and therapeutic aspects in 36 patients

CONTEXTO: A arterite de Takayasu é uma vasculite crônica, geralmente com diagnóstico tardio devido à pouca especificidade dos sintomas durante a fase inicial do acometimento vascular. A terapêutica de eleição é o uso de imunossupressores. O procedimento cirúrgico, quando necessário, é sempre evitado na fase aguda. OBJETIVO: Descrever alterações clínicas, laboratoriais e vasculares de arterite de Takayasu no período de 1977 a 2006. MÉTODO: A amostra compreendeu 36 pacientes - 10 brancos, 35 mulheres, idade média de 31,7 anos (±13,7), com prevalência significante na quarta década (p < 0,005). Evolução de 3 anos e período até o diagnóstico de 7,9 anos. Velocidade de hemossedimentação (VHS) e proteína C reativa (PCR) avaliaram atividade da doença, e o duplex scan aferiu a espessura médio- intimal da artéria carótida. RESULTADOS: Hipertensão arterial sistêmica e claudicação de membros superiores e inferiores foram ressaltados em 85,2, 69,5 e 30,5 por cento, respectivamente. O resultado da VHS foi > 60 mm em 50 por cento da amostra (p < 0,005). PCR mg/dL foi realizado em 18, variando de 0,4-25 na admissão para 0,11-1,9 na evolução. Doença auto-imune, tuberculose e HIV correlacionaram-se em 19,4, 8,3 e 2,7 por cento, respectivamente. Lesões aórticas foram significativas em 22 por cento (quatro oclusões, dois aneurismas infra-renais, um torácico). Em 19,4 por cento, foram acometidas artérias renais e subclávias uma oclusão bilateral de carótidas, e em 25 por cento os membros inferiores. A espessura médio-intimal da carótida comum foi estratificada em: > 3 mm, < 3 e > 1,7, < 1,7 e > 1,2 e < 1,2 mm, representando 41,6, 19,4, 8,37 e 30,50 por cento, respectivamente (p < 0,005). Glicocorticóides foram utilizados em 61,1 por cento, azatioprina em 16.6 por cento, e associada a ciclofosfamida em 8,3 por cento. Procedimento cirúrgico ou endovascular foi realizado em 30,5 por cento com dois óbitos por complicações cardiovasculares. CONCLUSÕES: A VHS,...

BACKGROUND: Takayasu arteritis is a chronic vasculitis often with delayed diagnosis due to the nonspecific presentation of clinical symptoms in its initial phase. Treatment includes imunosuppression drugs. Surgical treatment, when necessary, should be avoided in the acute phase. OBJECTIVE: To describe clinical, laboratory and vascular findings in Takayasu's arteritis from 1977 through 2006. METHODS: The sample was comprised of 36 patients (10 Caucasians, 35 women), mean age of 31.7 (±13.7) years, and significant prevalence in the forth decade (p < 0.005). Disease course was 3 years and time until diagnosis was 7.9 years. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were used to assess disease activity, and duplex scan to measure carotid artery intima-media wall thickness. RESULTS: Hypertension was present in 85.2 percent, and upper and lower limb claudication in 69.5 and 30.5 percent, respectively. ESR was > 60 mm in 50 percent of the sample (p < 0.005). PCR mg/dL was performed in 18 cases, ranging from 0.4-25 on admission to 0.11-1.9 during disease course. Autoimmune diseases, tuberculosis and HIV correlated in 19.4, 8.3 and 2.7 percent, respectively. Major aortic lesions were detected in 22.2 percent (four occlusions, two infrarenal aneurysms, one thoracic aneurysm). Other arteries involved renal, subclavian and one carotid occlusion (19 percent), and some level of lower limb occlusion (25 percent). Intima-media thickness was stratified in > 3 mm (41.6 percent), < 3 and > 1.7 (19.4 percent), < 1.7 and > 1.2 (8.37 percent), and < 1.2 mm (30.50 percent) (p < 0.005). Glucocorticoids were used in 61 percent, azathioprine in 16.6 percent, and azathioprine combined with cyclophosphamide in 8.3 percent. Surgical and endovascular procedures were performed in 30.5 percent. Two patients died due to cardiovascular diseases. CONCLUSIONS: Carotid intima-media thickness, PCR, and ESR are important markers for the follow-up of...

Manifestaciones otorrinolaringológicas en la enfermedad de Takayasu / Otolaryngologic manifestations in Takaysasu´s disease

Takayasu arteritis is a chronic idiopathic granulomatous vasculitis, which affects the aorta and its major branches, causing narrowing of the vascular lumen, which explains his particular symptoms. In children, its main manifestations are hypertension, cardiomegaly, increasing of the erythrocyte sedimentation rate, among others. We present a case of a girl aged 16, who has a long history of ozena. At physical examination there are nasal scabs and cacosmia. In the laboratory study highlights high levels of C Reactive Protein and erythrocyte sedimentation rate. The study through angio-Tac, shows a significant narrowing of supraaortic vascular structures.

Enfermedad de Crohn asociada a arteritis de Takayasu / Crohn's disease associated with Takayasu's arteritis

Paciente de sexo femenino, de 26 años de edad, portadora de enfermedad de Crohn (EC) se presenta con aumento de su dolor abdominal, frémito abdominal y disminución de los pulsos periféricos. La tomografía computada y la arteriografía revelaron estenosis oclusivas que incluían las arterias subclavia izquierda, celíaca, renal y mesentérica superior, así como también aorta abdominal. Estos hallazgos son consistentes con arteritis de Takayasu. La paciente fue revascularizada para sortear las lesiones oclusivas de las arterias celíaca y mesentérica superior y las estenóticas de la aorta abdominal. Controles posteriores demuestran que la paciente permanece asintomática desde el punto de vista cardiovascular a los 3 años de la cirugía, habiendo presentado un empuje de su enfermedad inflamatoria intestinal.

A 26-year-old woman with Crohn's disease presented with increase of her abdominal pain, abdominal fremitus and decrease in peripheral pulses. The CT scan and the angiography revealed occlusive stenosis of several arteries: left subclavian, celiac, renal, superior mesenteric and abdominal aorta. This findings were consistent with Takayasu's arteritis. The patient underwent surgical revascularization to bypass the occlusive lesions in celiac and superior mesenteric arteries, and the stenosis in abdominal aorta. Subsequent controls showed that she remains free of cardiovascular symptoms three years after surgery but presents progression of her inflammatory bowel disease.

Enfermedad de Takayasu y embarazo / Takayasu's disease and pregnancy

Vasculitis conocida como la enfermedad sin pulso (abolición de pulsos radiales), así como aortoarteritis inespecífica. Enfermedad rara cuya incidencia es de 2,6 casos/millón/año. Afecta predominante a mujeres (7:1), entre la segunda y tercera década de vida (promedio 25 años). Principalmente se observa en población asiática (1,2,3). De etiología desconocida,de probable origen autoinmune, produce un lento daño arterial con característica de panarteritis (4). Evoluciona en dos etapas, una de tipo inflamatoria, de difícil diagnóstico por síntomas inespecíficos, seguida de una fase crónica (inactiva) (tabla I). La mortalidad documentada es baja, existe una importante morbilidad asociada a la mayoría de los pacientes (1-4). Compromete principalmente a la aorta (58 por ciento su porción descendente 30 por ciento la ascendente y 20 por ciento la abdominal). Además, puede afectar bilateralmente las arterias renales (70 por ciento), subclavias (80 por ciento) y carótidas (44 por ciento). Se clasifica según su ubicación en 4 grupos (Tabla II). Puede asociarse a embarazo. Las publicaciones parecen coincidir en que el embarazo de por sí no afectaría la evolución de la enfermedad y el buen pronóstico materno-fetal (2,3). El diagnóstico, a veces difícil, se basa en la clínica, complementado con eco doppler, arteriografía y resonancia nuclear magnética (de elección en el embarazo) (4). El tratamiento se basa en el uso de corticoides, asociados o no a citostáticos, principalmente metotrexate. Sin embargo, la cirugía sigue jugando un importante rol a largo plazo (1-3,5)

Arteriografía coronaria y aortografía por vía translumbar en un paciente con arteritis de Takayasu / Transumbalar coronary arteriography in a patient with Takayasu's arteritis

Desde su introducción por Dos Santos en 1929, la arteriografía por abordaje translumbar ha sufrido algunas transformaciones. En la actualidad ha sido reemplazadas por otros abordajes percutáneos y está indicada solamente cuando esas vías de acceso han fallado debido a obstrucciones aortoiliacas o subclavias. Este artículo presenta una paciente con arteritis de Takayasu con enfermedad severa de arterias periféricas y angina inestable, a quien se le realizó arteriografía coronaria y aortografía por vía translumbar. Se realiza una revisión de la técnica en mención

Fotocoagulaçäo retiniana na doença de Takayasu / Retinal light coagulation on Takayasy Disease

Um caso de Doença de Takayasu com comprometimento retiniano é descrito bem como o tratamento realizado através de foto-coagulaçäo