Chylous ascites developing after open thoracoabdominal aortic aneurysm repair in a patient with marfan syndrome

Abstract Chylous ascites is the pathologic accumulation of chylous fluid in the peritoneal cavity, caused by lymphomas, metastatic malignancies, and abdominal surgeries, rarely due to surgical trauma of the cisterna chyli or its major branches. A 24-year-old man with history of Marfan syndrome presented to our hospital with abdominal distention, abdominal pain, fluid in the incision region, and weakness. He had underwent an elective open aneurysm repair surgery nine days before for thoracoabdominal aortic aneurysm. Computed tomography revealed massive fluid collection in the abdominal cavity, which was drained surgically. He was diagnosed with chylous ascites and was discharged after conservative treatment.

Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Thoracic aortic aneurysm surgery in marfan patients: a perspective from the uk

Abstract Objective: Cardiovascular complications in Marfan patients include progressive aortic root dilation which can precipitate acute aortic dissection, ruptured aorta, severe aortic regurgitation, or all the aforementioned. Such complications can be fatal and the cause of death prior to any surgical intervention. We set out to identify the Marfan population in England and Wales and present their surgical outcomes. Methods: A total of 306 patients with Marfan syndrome who underwent aortic root surgery were identified between April 2007 and March 2013 from NICOR database. We examined the perioperative characteristics of such cohort along with in-hospital outcomes and survival. Results: Root and ascending segment procedures on Marfan patients performed in 3.3% of the total cohort by NICOR root surgery patients. The median reported age was 40 years (IQR = 29-49 years) and 100 (32.7%) were female. Of the patients analysed, 17.3% were treated non-electively and 68.6% of them received concomitant valve procedure. The in-hospital mortality was 2.0%. Reoperation for bleeding was required in 8.2% of patients and 1.3% of them suffered a cerebrovascular accident (CVA). Mortality at 1 year was reported as 5.5%. Conclusion: The outcomes of surgery on the root and ascending aorta in Marfan patients in the United Kingdom are satisfactory; however, the overall complexities of this patient population are not well understood and would benefit from further investigations.

Rotura de aneurisma de artéria poplítea em paciente clinicamente diagnosticado com síndrome de Marfan / Ruptured popliteal artery aneurysm in a patient with a clinical diagnosis of Marfan syndrome

Resumo A artéria poplítea é o principal local para a ocorrência de aneurismas periféricos. Suas formas de apresentação agudas são potencialmente ameaçadoras à viabilidade do membro e à vida, dentre as quais destacamos a sua rotura. Apesar de ser um evento raro, sua rotura demanda rápida proposta de intervenção para satisfatório desfecho terapêutico. O tratamento padrão-ouro é o cirúrgico convencional e se dá pela interposição de veia safena magna. Trabalhos feitos nas últimas décadas vêm encontrando associações entre a síndrome de Marfan e aneurismas periféricos. Este relato apresenta um caso de um aneurisma de artéria poplítea esquerda roto tratado com sucesso em um paciente de 82 anos diagnosticado clinicamente como portador de síndrome de Marfan previamente desconhecida.

Abstract The popliteal artery is the main site of occurrence of peripheral aneurysms. Acute presentations constitute a potential threat to limb viability and to life, especially in the event of rupture. Rupture is a rare event, but one that demands an immediate intervention decision to achieve a satisfactory treatment outcome. The gold standard treatment is conventional surgery, effecting repair by interposition of a great saphenous vein graft. Studies conducted in recent decades have found associations between Marfan Syndrome and peripheral aneurysms. This report presents a case of a ruptured left popliteal artery aneurysm successfully treated in an 82-year-old patient clinically diagnosed with previously unknown Marfan syndrome.

Facoanafilaxia por cristalino mergulhado na Síndrome de Marfan / Phacoanaphylactic by dislocation of the lens in the Marfan Syndrome

Resumo A síndrome de Marfan é uma doença de herança autossômica dominante e que afeta o tecido conjuntivo com manifestações fenotípicas que envolvem os sistemas esquelético, cardiovascular e ocular. As principais manifestações oculares são a subluxação do cristalino, a miopia e o descolamento da retina. O objetivo deste artigo foi relatar a conduta clínico-cirúrgica de um paciente portador da síndrome de Marfan com cristalino luxado para a cavidade vítrea e que evoluiu com severa reação facoanafilática caracterizada por um glaucoma secundário severo e descompensação corneana.

Abstract Marfan syndrome is an autosomal dominant inheritance disease that affects connective tissue with phenotypic manifestations involving the skeletal, cardiovascular and ocular systems. The main ocular manifestations are the subluxation of the lens, myopia and retinal detachment. The aim of this article was to report the clinical and surgical management of a patient with Marfan syndrome with luxated lens for the vitreous cavity and who developed a severe phacoanaphylactic reaction characterized by severe secondary glaucoma and corneal decompensation.

Marfan Syndrome: Clinical Presentation with Congenital Glaucoma? / Síndrome de Marfan: ¿presentación clínica con glaucoma congénito?

ABSTRACT Congenital glaucoma (CG), diagnosed in the first year of life, can fall into three main groups: primary CG, glaucoma associated with congenital anomalies, and secondary glaucoma of infants. The associated congenital anomaly of CG includes the autosomal dominant: Marfan Syndrome (MS), the phenotypic features of which would rarely be evident in the first year of life. Multiple other associated autosomal dominant, autosomal recessive, X-linked and chromosomal conditions that can present with CG need to be excluded. Hence, this is a retrospective diagnosis and is the first known documented case report in an Afro-Caribbean with MS presenting with CG at six weeks of age. The Marfanoid features became apparent in late childhood and adolescence.

RESUMEN El glaucoma congénito (GC), diagnosticado en el primer año de vida, puede clasificarse en tres grupos principales: GC primario, glaucoma asociado con anomalías congénitas, y glaucoma secundario de lactantes. La anomalía congénita asociada con el GC incluye el trastorno autosómico dominante conocido como síndrome de Marfan (SM), cuyas características fenotípicas serían raramente evidentes en el primer año de vida. Otras múltiples condiciones asociadas - autosómicas dominantes, autosómicas recesivas, ligadas al cromosoma X, y cromosómicas - que pueden presentarse con el GC, necesitan ser excluidas. Por lo tanto, éste es un diagnóstico retrospectivo, a la par que el primer reporte de caso documentado de que se tenga noticia en relación un bebé afrocaribeño con SM, que presentaba GC a las seis semanas de la edad. Las características marfanoides se hicieron evidentes en la última etapa de la niñez y en la adolescencia.

Evaluación de las características de la disección aórtica en la población mexicana mediante angiotomografía computarizada / Evaluation of the characteristics of aortic dissection by computed angio-tomography in the Mexican population

Resumen Objetivo: Revisión y análisis de la disección aórtica (DA) en la población mexicana. Método: Revisión retrospectiva de 434 expedientes electrónicos de pacientes con angiotomografía de aorta entre noviembre de 2014 y octubre de 2015. Se obtuvo una muestra de 32 pacientes con diagnóstico de DA de primera vez. Se realizó un análisis de las DA según género, grupo etario, clasificación de Stanford/De Bakey y mortalidad a 6 meses del diagnóstico. Se realizó análisis de significación estadística mediante la Chi-cuadrada para las variables independientes de género, síndrome de Marfan, hipertensión arterial sistémica y enfermedad ateromatosa calcificada en asociación con subtipos, sitios de reentrada y datos de hipoperfusión. Resultados: El 65.6% de los pacientes fueron masculinos, con un promedio de edad de 54.5 años, y el 34.4% fueron femeninos, con un promedio de edad de 42.5 años. El subtipo B/3 fue el más frecuentemente diagnosticado. La tasa de mortalidad a 6 meses fue del 18.7%. Se halló asociación significativa con p marginal en pacientes con síndrome de Marfan y subtipos de DA según Stanford (p = 0.0506), así como asociación significativa en pacientes con aneurisma de aorta abdominal y subtipos de DA según Stanford (p = 0.047104). Conclusiones: La DA es una emergencia en la cual el diagnóstico por imagen y el manejo oportuno son fundamentales para mejorar el pronóstico. En nuestra muestra encontramos asociación significativa de pacientes con antecedente de síndrome de Marfan y aneurisma aórtico abdominal con disecciones según la categoría de Stanford. El resto de las variables independientes no mostraron asociación significativa, en probable relación con el tamaño de la muestra.

Abstract Objective: To review aortic dissection (AD) in the Mexican population. Method: A retrospective study was conducted using 434 medical records of patients with aortic angio-tomography between November 2014 and October 2015. A sample was obtained of 32 patients with a first time diagnosis of AD. An analysis was performed of the dissections according to gender, age group, Stanford/De Bakey classification, and mortality rate 6 months after diagnosis. Statistical analysis was performed by obtaining the Chi squared index for the independent variables of gender, Marfan syndrome, systemic arterial hypertension, as well as calcified atheromatous disease in association with dissection subtypes, re-entry sites, and hypo-perfusion signs. Results: The patients included 65.6% males with a mean age of 54.5 years, and 34.4% females with mean age of 42.5 years. The most common dissection subtype was B/3. Mortality rate at 6 months was 18.7%. There was a significant association, with a marginal P in patients with Marfan syndrome and Stanford subtypes of AD (P = .0506). There was a significant association in patients with abdominal aortic aneurysm, when compared with Stanford subtypes of AD (P = .047104). Conclusions: AD is an emergency in which diagnosis and timely management are essential to improve prognosis. In the sample presented here, a significant association was found in patients with a history of Marfan syndrome and abdominal aneurysms with dissections according to the Stanford classification. The rest of the independent variables did not show any significant association, probably related to the size of the sample.

Iris-claw intraocular lens implantation in children with ectopia lentis / Implante de lente intraocular de fixação iriana em crianças com ectopia lentis

ABSTRACT Purpose: Artisan iris-claw lens implantation (AICLI) is a surgical technique for treating ectopia lentis. We aimed to compare visual outcomes and possible long-term complications of AICLI surgery in pediatric patients with ectopia lentis with or without a diagnosable hereditary disease. Methods: Seventeen children with non-traumatic ectopia lentis were retros pectively classified into two groups: group 1 included children with a diagnosable hereditary disease (11 patients, 65%), and group 2 included children without any definable hereditary disease (six patients, 35%). Patients were evaluated for post-surgical refraction, best-corrected visual acuity, and clinical follow-up complications. Results: The average follow-up time was 38 months, and the average age of the patients was 103 ± 53 months (30-196 months). Best-corrected visual acuity values were significantly increased in both groups after surgery (p<0.05). Target refraction values were achieved at a rate of 47% in group 1 and 22% in group 2. Post-surgery complications, such as lens dislocation (36%, 11 eyes of 10 patients) and hypotonia (10%, three eyes of three patients) were observed in both groups, and retinal detachments (10%, three eyes of three patients) were observed in three patients from group 1. Conclusions: Compared with previous similar studies, this study utilized the largest pediatric patient group and had the longest post-surgery follow-up time. Moreover, it is advisable that pediatric patients with non-traumatic ectopia lentis be carefully screened for any underlying hereditary disease, especially diseases related to connective tissue metabolism.

RESUMO Objetivo: A implantação de lentes intraoculares de fixação iriana em garra (AICLI) é uma técnica cirúrgica para o tratamento de ectopia lentis. Nosso objetivo foi comparar resultados visuais e possíveis complicações em longo prazo da cirurgia de AICLI em pacientes pediátricos com ectopia lentis com ou sem doença hereditária diagnosticável. Métodos: Dezessete crianças com ectopia lentis não-traumática foram classificadas retrospectivamente em dois grupos: o grupo 1 com pacientes apresentando doença hereditária diagnosticável (11 pacientes, 65%) e o grupo 2 com pacientes sem qualquer doença hereditária definível (6 pacientes, 35%). Os pacientes foram avaliados quanto à sua refração pós-operatória, acuidade visual melhor corrigida e complicações. Resultados: O tempo médio de seguimento foi 38 meses. A média de idade dos pacientes foi de 103 ± 53 meses (30-196 meses). Os valores de acuidade visual me lhor corrigida aumentaram significativamente em ambos os grupos (p<0,05). Os valores de refração alvo foram alcançados a uma taxa de 47% no grupo 1 e 22% no grupo 2. Complicações pós-operatórias como luxação da lente (36%, 11 olhos de 10 pacientes) e hipotonia (10%, 3 olhos de 3 pacientes) foram observados nos dois grupos e foram observados descolamentos de retina (10%, 3 olhos de 3 pacientes) em 3 pacientes do grupo 1. Conclusões: Em comparação com relatos anteriores na literatura, este estudo utilizou um grupo maior de pacientes pediátricos e tempo de seguimento pós-operatório mais longo. É aconselhável que pacientes pediátricos com ectopia lentis não-traumática sejam cuidadosamente selecionados em relação a doença subjacente hereditária, especialmente as doenças relacionadas com o metabolismo do tecido conjuntivo.

Meningocele sacro anterior que simula quiste de ovario tabicado / Anterior sacral meningocele simulating ovarian cyst baffled

El meningocele sacro anterior es una rara anomalía genética asociada a enfermedades del tejido conectivo como el síndrome de Marfán, síndrome de Loeys-Dietz, la neurofibromatosis tipo 1 y otras. El objetivo del presente trabajo es presentar el caso de un feto con meningocele sacro anterior, inicialmente diagnosticado como un gran quiste de ovario multilobulado, en una paciente con diagnóstico de síndrome de Marfan. Se trata de una herniación del saco dural, a través de agujeros sacros amplios y deformados por ectasia dural, ocupada mayormente por líquido cefalorraquídeo. La punción de estas estructuras o su rotura en el acto operatorio se ha asociado a complicaciones graves e incluso la muerte(AU)

The anterior sacral meningocele is a rare genetic anomaly associated with connective tissue diseases such as Marfan syndrome, Loeys-Dietz syndrome, neurofibromatosis type 1 and others. The aim of this paper is to present the case of a fetus with anterior sacral meningocele, initially diagnosed as a large multilobulated ovarian cyst in a patient diagnosed with Marfan syndrome It is a herniation of the dural sac, through large sacral foramina, and deformed by dural ectasia, occupying mostly by cerebrospinal fluid. Puncture or rupture these structures during surgery has been associated with serious complications and even death(AU)

Aneurismas da Aorta Torácica: Avaliação Genética e de Imagem para Cirurgias Eletivas / Thoracic Aortic Aneurysm: Genetic and Image Evaluation for Elective Surgeries

Evidências mostram que variações genéticas de um único gene, denominadas “mutações genéticas”, predispõem indivíduos aos aneurismas e dissecções da aorta e seus ramos. Com a identificação dessas mutações, diretrizes sugerem o manuseio no diagnóstico, o momento ideal para a correção cirúrgica e a identificação de indivíduos sob alto risco de ruptura ou dissecção e suas respectivas famílias. Essas mutações podem se apresentar de forma “sindrômica”, com identificação fenotípica simples ou apresentação “familiar”. Nos indivíduos sem características fenotípicas, a ocorrência familiar da mutação genética deve ser investigada através da história familiar, exames de imagem e através de marcadores genéticos.

Evidence shows that genetic variations in a single gene called gene mutations predispose individuals to aneurysms and dissections of the aorta and its branches. With the identification of these mutations, guidelines suggest that diagnosis management is the idealtime for surgical correction and the identification of individuals at high risk of rupture or dissection and their families. These mutations may be present in a syndromic way with simple phenotypic identification or family presentation. In individuals without phenotypic characteristics, familial occurrence of genetic mutation should be investigated through the family history, imaging scansand through genetic markers.

Emergent cardiopulmonary bypass during pectus excavatum repair.

Pectus excavatum is a chest wall deformity that produces significant cardiopulmonary disability and is typically seen in younger patients. Minimally invasive repair of pectus excavatum or Nuss procedure has become a widely accepted technique for adult and pediatric patients. Although it is carried out through a thoracoscopic approach, the procedure is associated with a number of potential intraoperative and post-operative complications. We present a case of cardiac perforation requiring emergent cardiopulmonary bypass in a 29-year-old male with Marfan syndrome and previous mitral valve repair undergoing a Nuss procedure for pectus excavatum. This case illustrates the importance of vigilance and preparation by the surgeons, anesthesia providers as well as the institution to be prepared with resources to handle the possible complications. This includes available cardiac surgical backup, perfusionist support and adequate blood product availability.

Síndrome de Marfan con disección aórtica asociada a tromboembolismo venoso e hiperhomocisteinemia / Marfan syndrome associated with aortic dissection, venous thromboembolism and hyperhomocysteinemia

El síndrome de Marfan es un desorden genético infrecuente, cuyas manifestaciones clínicas afectan a los sistemas cardiovascular, ocular y músculo esquelético, la gravedad de las manifestaciones cardiovasculares son generalmente responsables de la mortalidad de estos pacientes. La enfermedad tromboembólica venosa está íntimamente relacionada con diversos factores de riesgo, congénitos, adquiridos, mixtos o desconocidos. La hiperhomocisteinemia es un factor de riesgo moderado para tromboembolismo venoso. Presentamos el caso de una mujer de 47 años de edad con síndrome de Marfan asociado a disección aórtica tipo A indolora, que simultáneamente padeció trombosis venosa de miembros inferior y superior izquierdos con embolia de pulmón. Al realizar el cribado de trombofilia se constató hiperhomocisteinemia. Con la terapéutica instituida evolucionó favorablemente.

Marfan syndrome is an infrequent genetic disorder of connective tissue whose clinical manifestations mainly affect the cardiovascular, ocular and musculoskeletal systems. Serious cardiovascular manifestations are generally the cause of mortality of Marfan patients. Thromboembolic venous disease is intimately related to different risk factors: inherited, acquired, mixed or unknown; hyperhomocysteinemia is a moderate risk factor for venous thromboembolism. We present the case of a 47-year-old woman with Marfan syndrome associated to a painless type A aortic dissection, who simultaneously suffered venous thromboembolism of left upper and lower limbs with pulmonary embolism. Hyperhomocysteinemia was found through thrombofilia screening. The patient's condition has evolved favorably.

Importancia de la búsqueda ecográfica de aneurisma de aorta abdominal en pacientes con síndrome de Marfan / Importance of the ecographic search of the aortic aneurysm patients with Marphan Syndrome

Características de esta patología y de un caso clínico, con el objetivo de presentar a la ecografía abdominal como método de screening y seguimiento para la búsqueda de lesiones de la aorta abdominal, asociadas a esta enfermedad.

Disección aórtica tipo B y embarazo en un síndrome de Marfán: manejo y resultado / Type B aortic dissection and pregnancy in Marfan syndrome

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.

Marfan syndrome. Correct diagnosis can save lives

Marfan syndrome is a heritable disorder of the connective tissue that affects many systems of the body. However, the most serious complication in patients with Marfan syndrome is progressive enlargement of the aortic root, which may lead to aortic dissection, rupture, or aortic regurgitation. Prevention of these life threatening complications is very important in the management of this condition. A 39-year-old Omani man presented with progressive shortness of breath and eventually underwent major but successful cardiac surgery. It is very important to recognise Marfan syndrome early as preventive actions are possible if the condition is diagnosed before complications occur

Marfan's syndrome: successful term pregnancy after repair of a dissecting thoracic aortic aneurysm at 21 weeks gestation

We report a 38 year-old Chinese woman with Marfan' syndrome that had an acute type A aortic dissection and severe aortic regurgitation at 21 weeks of gestation. Emergency aortic valve repair and ascending aortic replacement was successfully performed under circulatory arrest with mild hypothermia. The patient underwent a cesarean section and delivered a healthy baby at 38 weeks

Análise do tratamento cirúrgico da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica / Analysis of aortic root surgery with composite mechanical aortic valve conduit and valve-sparing reconstruction

OBJETIVO: Análise comparativa dos resultados imediatos e tardios da reconstrução da raiz da aorta com o tubo valvulado e com a preservação da valva aórtica. MÉTODOS: No período de novembro de 2002 a setembro de 2009, 164 pacientes com idade média de 54 ± 15 anos, sendo 115 do sexo masculino, foram submetidos ao tratamento cirúrgico da raiz da aorta. Foram 125 tubos valvulados e 39 reconstruções da raiz da aorta com preservação da valva aórtica. Dezesseis por cento dos pacientes eram portadores de síndrome de Marfan e 4,3 por cento apresentavam valva aórtica bivalvulada. Cento e quarenta e quatro (88 por cento) pacientes foram acompanhados durante tempo médio de seguimento de 41,1 ± 20,8 meses. RESULTADOS: A mortalidade hospitalar total foi de 4,9 por cento; sendo 5,6 por cento nas operações com tubo valvulado e 2,6 por cento nas preservações da valva aórtica (P<0,05). Não houve diferença na sobrevida (IC 95 por cento=86 por cento-96 por cento, P=0,1) e na sobrevida livre de reoperação (IC 95 por cento=85 por cento-90 por cento, P=0,29). As sobrevidas livres de complicações como sangramento, tromboembolismo e endocardite foram favoráveis às operações com a preservação da valva aórtica, respectivamente (IC 95 por cento=70 por cento95 por cento, P=0,001), (IC 95 por cento=82 por cento-95 por cento, P=0,03) e (IC 95 por cento=81 por cento95 por cento, P=0,03). A análise multivariada mostrou que a creatinina maior ou igual a 1,4 mg/dl, a operação de Cabrol e a insuficiência renal dialítica foram preditores de mortalidade, respectivamente, com chance de ocorrência (OR) de 6 (IC 95 por cento=1,8-19,5; P=0,003), OR de 12 (IC 95 por cento=3-49,7; P=0,0004) e OR de 16 (IC 95 por cento=3,6-71,3; P=0,0002). CONCLUSÕES: A reconstrução da raiz da aorta apresenta baixa mortalidade precoce e tardia, sobrevida livre de complicações elevada e baixa necessidade de reoperação. Durante o seguimento tardio, a reconstrução da raiz da aorta com preservação da valva aórtica apresentou menor incidência de sangramento, de fenômenos tromboembólicos e de endocardite.

OBJECTIVE: Comparative analysis of early and late results of aortic root reconstruction with aortic valve sparing operations and the composite mechanical valve conduit replacement. METHODS: From November 2002 to September 2009, 164 consecutive patients with mean age 54 ± 15 years, 115 male, underwent the aortic root reconstruction (125 mechanical valve conduit replacements and 39 valve sparing operations). Sixteen percent of patients had Marfan syndrome and 4.3 percent had bicuspid aortic valve. One hundred and forty-four patients (88 percent) were followed for a mean period of 41.1 ± 20.8 months. RESULTS: The hospital mortality was 4.9 percent, 5.6 percent in operations with valved conduits and 2.6 percent in the valve sparing procedures (P <0.05). There was no difference neither in survival (95 percent CI = 86 percent - 96 percent, P= 0.1) nor in reoperation-free survival (95 percent CI = 85 percent - 90 percent, P = 0.29). The survival free of complications such as bleeding, thromboembolism and endocarditis were favorable to the valve sparing operations, respectively (95 percent CI = 70 percent - 95 percent, P = 0.001), (95 percent CI = 82 percent - 95 percent P = 0.03) and (95 percent CI = 81 percent - 95 percent, P = 0.03). Multivariate analysis showed that creatinine greater than 1.4 mg/dl, Cabrol operation and renal dialysis were predictors of mortality, respectively, with occurrence chance of 6 (95 percent CI = 1.8 - 19.5, P = 0.003), 12 (95 percent CI = 3 - 49.7, P = 0.0004) and 16 (95 percent CI = 3.6 - 71.3, P = 0.0002). CONCLUSIONS: The aortic root reconstruction has a low early and late mortality, high survival free of complications and low need for reoperation. During the late follow-up, valve sparing aortic root reconstructions presented fewer incidences of bleeding, thromboembolic events and endocarditis.

Procedimiento de Bentall en la enfermedad aneurismática de la aorta ascendente: mortalidad hospitalaria / Bentall procedure in ascending aortic aneurysm: hospital mortality

Introducción: La enfermedad aneurismática de la aorta ascendente (EAAA) se caracteriza por su baja frecuencia, comportamiento heterogéneo, riesgo de rotura y disección, que conllevan elevada mortalidad, por lo que la cirugía electiva es fundamental. Se han desarrollado diversos procedimientos quirúrgicos, considerándose la técnica de Bentall el estándar de referencia. Se describe la mortalidad hospitalaria de la EAAA tratada quirúrgicamente mediante el procedimiento de Bentall. Material y métodos: Estudio descriptivo en el que se incluyeron 23 pacientes con EAAA operados entre el 1 de marzo de 2005 y el 30 de septiembre de 2008; la información fue obtenida de los expedientes clínicos. Resultados: Los 23 pacientes correspondieron a 1.2 % de las cirugías efectuadas. Edad media de 46 años (rango 16 a 74), sexo masculino 83 %. Etiología: degeneración inespecífica de la capa media con implicación valvular 43 %, aorta bivalva 22 %, síndrome de Marfán, de Turner y aneurismas posestenóticos, 9 % cada uno. Enfermedad de Takayasu y espondilitis anquilosante, 4 % cada uno. Enfermedad cardiaca asociada en seis (26 %): coartación aórtica (2), cardiopatía isquémica (1), comunicación interauricular (1), insuficiencia mitral severa (1) y rodete subaórtico (1). Procedimientos realizados: cirugía de Bentall 20 (87 %), aortoplastia con prótesis valvular tres (13 %). Complicaciones: sangrado anormal con reintervención 17 %, neumonía nosocomial 13 %, arritmias 13 %, choque séptico 9 %. Mortalidad tres (13 %): choque séptico y fibrilación ventricular. Conclusiones: La mortalidad hospitalaria para la cirugía de Bentall fue semejante a la registrada en otros centros especializados. Los eventos relacionados con la patología aórtica, técnica quirúrgica, prótesis valvular aórtica y la disfunción ventricular izquierda, obligan a realizar estudios de seguimiento a largo plazo.

BACKGROUND: Ascending aortic aneurysm disease (AAAD) shows a low frequency, heterogeneous behavior, high risk of rupture, dissection and mortality, making elective surgery necessary. Several procedures have been developed, and the Bentall technique is considered as the reference standard. The objective was to describe the hospital mortality of AAAD surgically treated using the Bentall procedure. METHODS: We carried out a descriptive study. Included were 23 patients with AAAD who were operated on between March 1, 2005 and September 30, 2008 at our hospital. Data were obtained from clinical files, and descriptive statistics were selected for analysis. RESULTS: The study population was comprised of 23 patients with an average age of 46 years; 83% were males. Etiology was nonspecific degeneration of the middle layer with valve implication in 43%, bivalve aorta in 22%, Marfan syndrome, Turner's syndrome and poststenotic aneurysms each represented 9%, and Takayasu disease and ankylosing spondylitis 4% each. Associated heart disease was reported in six (26%) patients as follows: aortic coarctation (2), ischemic cardiopathy (1), atrial septal defect (1), severe mitral insufficiency (1) and subaortic membrane (1). Procedures carried out were Bentall surgery in 20 (87%) patients and aortoplasty with valve prosthesis in three (13%) patients. Complications reported were abnormal bleeding with mediastinal exploration (17%), nosocomial pneumonia (13%), arrhythmia (13%), and septic shock (9%). Mortality was reported in three (13%) patients due to septic shock and ventricular fibrillation. CONCLUSIONS: Surgical mortality with the Bentall procedure is similar to published results by other specialized centers. Events related to the basic aortic pathology, surgical technique, aortic valve prosthesis and left ventricular dysfunction encourage longterm studies with follow-up.