RESUMEN

El síndrome de Marfán es una enfermedad autosómica dominante del tejido conectivo, multisistémica y pronóstico determinado por las complicaciones cardiovasculares. Se presenta una paciente de 32 años, multípara, con el diagnóstico conocido de cinco años, posterior a disección aórtica toraco-abdominal (Standford B) y embarazo actual no planificado. Es manejada con betabloqueadores y las imágenes seriadas de aorta muestran disección estable en el segmento entre subclavia izquierda e ilíaca derecha con 41,8 mm en su diámetro mayor, sin comprometer la irrigación de órganos abdominales. Ingresa a las semana 32 para evaluación multidisciplinaria y parto programado. El examen con resonancia nuclear magnética de columna lumbosacra evidenció ectasia dural marcada a nivel de raíz S2. La evaluación fetal demostró un crecimiento en percentil 20 con bienestar hemodinámico y ecocardiografía normal. Previa inducción de madurez pulmonar y con 34+3 semanas, se efectúa cesárea electiva bajo anestesia espinal continua, con nacimiento sin compresión del fondo uterino, esterilización tubaria y postoperatorio inmediato en Unidad Coronaria por 48 horas. La evolución materna es sin incidentes. El recién nacido presentó enterocolitis necrotizante con buena respuesta al tratamiento médico.

Marfan syndrome is a multysistemic an autosomal-dominant disorder of connective tissue and cardiovascular complications determine its prognosis. We present a 32 year-old patient diagnosed five years previously after thoraco-abdominal aneurysm Standford B, and a current unplanned pregnancy. She was treated with beta blockers and evaluated with serial images of the aorta showing a stable thoraco-abdominal aneurysm initiated at the left subclavian artery to the right iliac artery of 41.8 mm at maximum diameter. The patient was hospitalized at 32 weeks to be evaluated in a multidisciplinary team planned delivery. Nuclear Magnetic Resonance on maternal spine detected dural ectasia at S2 and fetal evaluation shows growing at 20 percentile, hemodinamical wellbeing and normal echocardiography. After corticosteroids at 34 +3 weeks a cesarean section with tubal sterilization is performed, with continuous spinal anesthesia, without uterus compression and inmediate postoperative care at the Coronary Unit for 48 hours with satisfactory maternal evolution. The newborn presented a necrotizing enterocolitis at 7 days improving with medical therapy.

Asunto(s)

RESUMEN

OBJETIVO: Chamar a atenção para as complicações, que podem surgir em qualquer fase da Doença de Kawasaki (DK), para os fatores de risco que contribuem para o aparecimento dessas complicações e para as possíveis sequelas da doença, sejam elas transitórias ou permanentes. MÉTODOS: Estudo prospectivo (coorte clínica) realizado entre abril de 2002 e abril de 2009 de 115 pacientes com DK internados no serviço de Reumatologia Pediátrica do Hospital Geral do Distrito Federal. Todos os pacientes foram sequencialmente avaliados com exames clínicos e laboratoriais, ecocardiogramas com Doppler, imitanciometria, potenciais evocados auditivos, avaliação psicológica, exame oftalmológico e, em um paciente com coreia, angiorressonância magnética cerebral. Em todos os pacientes foram aplicados questionários avaliando a possível presença de dificuldades cognitivas, emocionais, comportamentais e sociais. RESULTADOS: Vinte e cinco pacientes (21,7 por cento) apresentaram aneurismas de coronárias. Trinta e oito pacientes (33 por cento) apresentaram perda auditiva neurossensorial durante a doença aguda e subaguda, e 13 pacientes (11,3 por cento) mantiveram a perda auditiva seis meses após a primeira avaliação. Outras complicações observadas foram: paralisia facial em um paciente (0,9 por cento), ataxia na fase aguda e subaguda em 11 pacientes (9,5 por cento), complicações oftalmológicas em 15 pacientes (13,2 por cento), constatando-se uveíte em 13, edema de papila em um paciente e hemorragia conjuntival em outro. Um paciente apresentou coreia (0,9 por cento) sendo que a angioressonância magnética evidenciou alterações compatíveis com isquemia cerebral. Em um paciente constatou-se a presença de aneurisma de aorta torácica (0,9 por cento), e outro apresentou vasculite necrosante que evoluiu com gangrena periférica e perda da ponta da língua (0,9 por cento). Alterações de comportamento durante a convalescença (20 por cento) foi observada em 23 crianças. CONCLUSÃO: A DK pode evoluir com complicações diversas, mesmo meses após a fase aguda da doença, eventualmente resultando em sequelas permanentes. Quanto mais precoce forem o diagnóstico e a intervenção terapêutica com a administração de IgG IV, menor será a ocorrência de complicações. Presença de trombocitose, anemia e de atividade inflamatória elevada e por tempo prolongado são fatores de risco para o aparecimento de complicações.

OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD) stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort) conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7 percent) had coronary aneurisms. Thirty eight patients (33 percent) had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3 percent) maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9 percent), ataxia in acute and subacute phases in 11 (9.5 percent); 15 patients had ophthalmologic complications (13.2 percent), with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9 percent), with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9 percent) and another patient had a necrotizing vasculitis progressing to peripheral gangrene and tongue tip loss (0.9 percent). Behavioral changes over convalescence were observed in 23 children. CONCLUSION: KD may progress with several complications even within months of the disease acute phase, eventually resulting in permanent sequelae. The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication arising.

Asunto(s)

RESUMEN

O tratamento endovascular das doenças da aorta torácica progrediu muito rapidamente nos últimos anos, principalmente nas lesões da aorta descendente e que possuem uma zona adequada de fixação proximal e distal. Hoje em dia as endopróteses disponíveis no mercado nacional permitem tratar aneurismas da aorta torácica descendente, dissecção da aorta do tipo B, hematomas e úlceras de aorta, e lesões traumáticas. O objetivo deste artigo é analisar os estudos recentes publicados e avaliar os resultados encorajadores do tratamento de casos desafiadores como as dissecções de aorta do tipo B e lesões traumáticas da aorta. Além disso, avaliamos os resultados iniciais da nova geração de endopróteses desenvolvidas para o tratamento e aneurismas tóraco-abdominais e justa-renais. Com esta ampla revisão esperamos expandir a população de pacientes que podem ser tratados por este método. Devido a condições de emergências, doenças associadas, muitos pacientes não têm opções cirúrgicas e com resultados apresentados com o tratamento endovascular das dissecções do tipo B e lesão traumática da aorta; a cirurgia endovascular passa a ser a primeira opção no tratamento destas doenças.

Treatment of thoracic aortic lesions using endografts has rapidly progressed.Clinical trials are underway evaluating stent graft use in a variety of applications. Endovascular repair can successfully treat challenging aortic indications such as type B dissection, traumatic injury and transection.The goal of this paper is to analyze the recent results of endovascular repair. Investigators are working to improve upon encouraging initial experiences and determine the degree to which endovascular repair can successfully treat challenging aortic diseases.We also analyze the next-generation endovascular systems that are designed to treat thoraco-abdominal aneurysms and para-renal aneurysms.The effort of this paper is to expand the population of patients that can be treated. Due to emergent conditions, comorbid concerns, high risk for surgery, and difficult anatomies, many patients have insufficient options for treating their life-threatening aortic diseases. For those patients with type B aortic dissections and traumatic aortic rupturel...

Asunto(s)

RESUMEN

The aortic arch aneurysm is a complex disease with a growing incidence in our population. We report a 64 year-old female with an atherosclerotic aortic arch aneurysm with a maximum diameter of 9.4 cm and evidence of contained rupture. We performed a complete aortic arch replacement in a single period of 100 minutes of circulatory arrest with deep hypothermia and brain protection with anterograde cerebral perfusión through the right axillary artery. The patient had a complete recovery without evidence of neurological damage and was extubated on the next morning. One year later, the patient is asymptomatic and the peri-aortic haematoma was completely reabsorbed.

Asunto(s)

RESUMEN

Background: Dissections that involve the ascending aorta are classified as type A, regardless of the site of the primary intimal tear, and all other dissections as type B. Type B dissections can have fatal ischemic and hemorrhagic complications. In the chronic state, dilatation and rupture can be mortal. Endovascular surgery is a therapeutic alternative, considering the high rate of complications of conventional surgery Aim: To report the results of endovascular treatment of type B aortic dissection. Material and methods: Report of 36 treated patients (30 males) aged 43 to 87 years, with a type B aortic dissection. Seventy eight percent were hypertensive and 39 percent smoked. The diagnosis was conñrmed by CAT sean. Acute patients were treated for complications and chronic patients, for dilatation. In the operating room, an endoprothesis was placed through the femoral artery, to cover the tear. The tear was located and the lumens were differentiated using angiography and transesophageal echocardiography. Results: All procedures were successful. In 16 acute dissections the indications were malperfusion syndrome or unmanageable hypertension in seven patients and imminent rupture or persistent pain in nine. Twenty chronic patients were operated due to dilatation (mean 6 cm). One patient died due to cardiac failure. One patient had a transient paraparesia and two had pulmonary embolism. No patient died in a follow up períod ranging from 2.5 to 74 months. Four patients required a new aortic endovascular procedure due to progressive dilatation or endoleak. Conclusión: Endovascular treatment of type B aortic dissection has good immediate andlong term results.

Asunto(s)

RESUMEN

As doenças do arco aórtico que necessitam de tratamento cirúrgico ainda exibem taxas de morbimortalidade relativamente elevadas, apesar dos avanços progressivos dos cuidados clínicos e cirúrgicos perioperatórios. A parada circulatória com hipotermia profunda, com perfusão cerebralretrógrada ou perfusão cerebral seletivaanterógrada associadas, mostraram-se úteis em reduzir as complicações desse tipo de cirurgia. Porém, as taxas de acidente vascular encefálico (AVE) e sangramento, dentre outros, ainda precisam de redução adicional. Neste artigo, relata-se uma nova técnica para cirurgias do arco aórtico, utilizando perfusão cerebral seletiva anterógrada ebilateral, através do isolamento do tronco braquicefálico (TBE) e carótida esquerda. Uma das vantagens observadas com essa nova técnica é queela permite a perfusão em separado do cérebro, que é mantido em temperatura mais baixa (200 C a 250C) em relação ao resto do corpo (250 C), possibilitandoo reaquecimento mais rápido do paciente ao final da cirurgia e reduzindo o tempo de circulação extracorpórea e discrasias sangüíneas. Ao final da cirurgia, pelas características anatômicas dos enxertos, a revisão da hemostasia é mais fácil. E, finalmente, no caso de necessidade de uso posteriorde uma endoprótese aórtica, esta pode ser facilmente colocada até próximo da válvula aórtica, sem comprometer o fluxo sangüíneo cerebral.

Aortic arch diseases that need surgical treatment still show relatively high morbi-mortality rates despite the rapid advances in clinical and surgical perioperative care. Deep hypothermic circulatory arrest with retrograde cerebral perfusion or anterograde selective cerebral perfusion showed tobe useful in reducing the complications of this type of surgery. However, stroke and bleeding rates, among others, still need further reduction. In this article, we describe a new technique for Aortic arch surgery with bilateral antegrade selective cerebral perfusion by isolation of innominate and left carotid arteries. Among the advantages of this techniqueis the possibility of perfusion separated from the brain, which is maintained at a lower temperature (200 C-250 C) in relation to the rest of the body (250C).This allows for faster rewarming of the patient following surgery as well as for shorter time of extracorporeal circulation. Another advantage isthat since the cerebral perfusion is not interrupted at any stage and is bilateral, the surgeon has more time to work on the aortic arch. At the end of surgery, the anatomical characteristics of the grafts allow for easier hemostasis review. Finally, an aortic endoprosthesis can be easily placed even closer tothe aortic valve without compromising the cerebral blood flow, if further needed.

Asunto(s)