RESUMO
Resumen La enfermedad de Kawasaki es un síndrome febril agudo y autolimitado, de presunta etiología autoinmune, que característicamente afecta a niños menores de 5 años. Esta enfermedad consiste en una vasculitis aguda de pequeños y medianos vasos que favorece la formación de aneurismas en las arterias coronarias. El diagnóstico en la fase aguda es esencialmente clínico, mientras que en la fase crónica suele hacerse al documentar las secuelas vasculares. La importancia de la identificación apropiada de esta enfermedad radica en que muchos de los infartos fatales y no fatales en personas menores de 40 años tienen hallazgos compatibles con este diagnóstico. Se presenta el caso de un adulto joven con dolor torácico y angiotomografía coronaria con hallazgos compatibles con secuelas de enfermedad de Kawasaki del adulto.
Abstract Kawasaki disease is an acute and self-limited febrile syndrome with a presumed autoimmune etiology that characteristically affects children under 5 years of age. Kawasaki disease is an acute vasculitis of small and medium vessels that facilitates the formation of coronary aneurysms. The diagnosis of the disease during the acute phase is essentially clinical, while the diagnosis of Kawasaki disease during the chronic phase is performed when the vascular complications are detected. The importance of proper identification of Kawasaki disease lies in the fact that many of the fatal and non-fatal infarcts in young adults (<40 years) have findings compatible with this diagnosis. This article describes the case of a young patient with chest pain and coronary angiotomography showing sequelae of Kawasaki disease in the adult.
Assuntos
Humanos , Masculino , Adulto , Aneurisma Coronário , Dor no Peito , Angiografia por Tomografia Computadorizada , Síndrome de Linfonodos MucocutâneosRESUMO
A doença renal policística autossômica dominante (DRPAD), uma das anormalidades genéticas mais comuns, é responsável por 10 por cento dos casos de insuficiência renal crônica terminal. O acometimento extrarrenal da DRPAD manifesta-se por cistos no fígado e pâncreas, aneurismas intra e extracranianos, e anormalidades cardíacas valvares, comprometendo a raiz da aorta e as valvas aórtica e mitral. Neste artigo descrevemos o caso de um paciente com aneurisma das artérias coronárias no contexto de outras manifestações sugestivas de DRPAD.
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic abnormalities and is responsible for over 10% of end-stage chronic kidney disease cases. Extrarenal involvement of ADPKD is manifested by liver and pancreatic cysts, intra and extracranial aneurysms and valvular heart disease, involving the aortic root and the aortic and mitral valves. In this article we report a case of a patient with large coronary artery aneurysms in the context of other manifestations suggesting ADPKD.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Aneurisma Coronário/complicações , Aneurisma Coronário/diagnóstico , Aneurisma/complicações , Aneurisma/diagnóstico , Doenças Renais Policísticas/complicaçõesRESUMO
El stent cubierto con membrana se ha reportado como un dispositivo útil para corregir lesiones angiográficas específicas, así como complicaciones potenciales secundarias a procedimientos de intervencionismo coronario. El stent cubierto con membrana de (Jostent-Jomed) está compuesto por una fina capa de politetrafluoroetileno (PTFE) que se localiza entre dos stents metálicos. En esta revisión retrospectiva el stent de PTFE se utilizó en varias lesiones y situaciones angiográficas tanto en arterias coronarias como en injertos venosos. Nuestra experiencia consistió de 17 pacientes que fueron tratados por diferentes indicaciones clínicas y angiográficas: perforación coronaria en dos pacientes (pts), injertos venosos con trombo y cambios degenerativos en ocho (pts), aneurismas coronarios en 4 (pts) y trombo grande refractario a tratamiento habitual en otros 3 casos. Nuestros resultados con el stent de PTFE mostraron una tasa de éxito clínico del 100% sin complicaciones durante la fase hospitalaria y en el seguimiento clínico a 10 ± 3 meses sólo tres pacientes tuvieron algún evento cardíaco adverso mayor. La indicación potencial para el implante del stent de PTFE en injertos venosos todavía no se ha puntualizado; sin embargo a la luz de varios estudios pareciera haber poco beneficio en esta circunstancia. Por otro lado la perforación y el aneurisma coronario parecen ser las indicaciones más recomendables para la aplicación de este dispositivo.
Summary The use of a synthetic membrane-covered stent has been suggested to overcome some specific lesions in coronary arteries and to solve some potential complications that could arise in any procedure of coronary intervention. A synthetic membrane coronary stent graft (JOSTENT-JOMED) is constituted by a membrane of polytetrafluoroethylene (PTFE), fixed between two thin metallic stents. In this clinical review of our laboratory's experience, the Jostent graft was used in several angiographic circumstances in coronary arteries or in vein grafts. Our clinical experience consists of 17 patients that were treated with placement of PTFE stents for different indications: coronary rupture in two patients; thrombus and degenerative vein grafts in eight patients, coronary aneurysms in four patients, and thrombus in coronary arteries in three more. Our clinical results with this device suggest that its implant is feasible and safe with a clinical success of 100% without complications during in-hospital stay; during the clinical 11 ± 3 months follow-up, only three patients exhibited major cardiac events. The indication for PTFE stent in vein grafts with degenerative and thrombus lesions has not been determined yet; however, recently, some randomized trials have shown little benefit in this circumstance. On the other side, coronary vessel rupture and coronary aneurysms are currently the most definitive indications.
Assuntos
Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença das Coronárias/cirurgia , Politetrafluoretileno , Stents , Aneurisma Roto , Aneurisma Roto/cirurgia , Angiografia Coronária , Aneurisma Coronário , Aneurisma Coronário/cirurgia , Doença das Coronárias , Trombose Coronária , Trombose Coronária/cirurgia , Estudos de Viabilidade , Seguimentos , Ensaios Clínicos Controlados Aleatórios como Assunto , Segurança , Fatores de Tempo , Resultado do TratamentoRESUMO
Los aneurismas coronarios son una patología relativamente infrecuente, con una incidencia anual del 1-2%. La causa más frecuente es la ateroesclerosis coronaria y en este caso se suelen asociar a estenosis en las arterias coronarias. Revisamos todos los estudios angiográficos realizados en los últimos 7 años y recogimos los casos de 6 pacientes ingresados por síndrome coronario agudo a los que se realizó coronariografía, demostrando dilataciones aneurismáticas en las arterias coronarias sin relación con estenosis proximales o distales. A pesar de la ausencia de estenosis coronarias, pensamos que la enfermedad ateroesclerótica podría ser la causa mediante: lesión del endotelio y la elástica interna con el depósito de lípidos, células musculares lisas, colágeno, macrófagos y linfocitos T, con afectación final de las capas media y adventicia, y neoformación de vasa-vasorum. Se desconoce la evolución de los aneurismas coronarios sin estenosis significativas asociadas y sin otra causa etiológica tratable. A tenor de la escasa literatura publicada al respecto y con nuestra experiencia creemos que el tratamiento médico conservador podría ser una buena opción en estos casos. En el seguimiento a medio-largo plazo no se registraron eventos cardíacos mayores en ningún paciente.
Coronary artery aneurysms are a relatively infrequent finding with an incidence of 1-2% per year. The most frequent cause is atherosclerosis and, in that case, they are always associated to stenosis of coronary arteries. We reviewed the coronary angiographic studies performed in the past seven years and we identified six patients that were admitted with an acute coronary syndrome, whose angiographic studies showed the presence of aneurysms in, at least, one of the coronary arteries. In these patients, we found no relation between aneurysms and distal or proximal stenosis. Although there was no angiographic evidence of classical coronary atherosclerosis, we think that atherosclerotic disease could have been the etiological cause, due to injury of the endothelium and media by deposits of lipids, smooth muscle cells, collagen, macrophages, and T-lymphocytes that would have damaged the adventitia layers of the vessel wall, stimulating "vasa-vasorum" neovascularization. The evolution of coronary aneurysms without associated stenosis in the same coronary artery and without another potentially treatable cause is unknown. Due to the limited literature regarding this issue and, taking into account our experience, we feel that, in these cases, medical treatment might be a good option as we detected no major cardiac events in any patient at mid and long-term follow up.