Diagnóstico prenatal de dos casos de anomalías congénitas del desarrollo venoso sistémico: una entidad muy poco frecuente (España) / Prenatal diagnosis of two cases of congenital systemic venus return anomalies: a rare entity (Spain) / Diagnóstico pré-natal de dois casos de anormalidades congênitas do desenvolvimento venoso sistêmico: uma entidade muito rara (Espanha)

Introducción: las anomalías en el desarrollo del sistema venoso sistémico son una entidad poco frecuente, cuyo diagnóstico ecocardiográfico prenatal y posnatal puede suponer todo un reto. Por un lado, debido a su baja incidencia y por otro, a la dificultad en la correcta realización de los planos ecocardiográficos. No obstante, su diagnóstico es de vital importancia debido a la asociación con cardiopatías congénitas o cromosopatías. Objetivo: describir dos casos de una anomalía congénita cardiovascular poco frecuente. La persistencia de la vena cava superior izquierda con agenesia de la vena cava superior derecha es una de estas anomalías descritas cuya incidencia es muy baja cuando ambas variaciones se presentan conjuntamente. Casos clínicos: presentamos dos casos de recién nacidos sin antecedentes personales o familiares de interés, diagnosticados prenatalmente, cuyos hallazgos ecocardiográficos se confirman en el período posnatal. Conclusiones: ante el hallazgo aislado en el período fetal de esta variación anatómica que asocia dos anomalías del sistema venoso sistémico, cabe destacar la importancia de su confirmación ecocardiográfica posnatal para descartar cardiopatías congénitas de difícil diagnóstico durante la época prenatal. Así mismo, antes de la confirmación ecocardiográfica que será llevada a cabo por el cardiológico infantil, cabe destacar la importancia del pediatra en la primera exploración física y en la anamnesis a la familia para descartar posibles cardiopatías congénitas críticas o posibles síndromes asociados. El diagnóstico prenatal de persistencia de la vena cava superior izquierda con agenesia de la vena cava superior derecha le permitirá tener un alto grado de sospecha de estas patologías asociadas y por tanto llevar a cabo una actuación clínica precoz.

Introduction: anomalies in the development of the systemic venous system are a rare entity, and its prenatal and postnatal echocardiographic diagnosis can be challenging, due to its low incidence as well as to the difficulty in correctly performing echocardiographic imaging planes. However, its diagnosis is key because it is linked to congenital heart disease or chromosomal anomalies. Objective: describe two cases of a rare cardiovascular congenital anomaly. The persistence of the left superior vena cava with agenesis of the right superior vena cava is one of these described anomalies, with very low incidence when both variations occur together. Clinical cases: we present two cases of newborns with no relevant personal or family history, diagnosed prenatally with confirmed echocardiographic findings in the postnatal period. Conclusions: given the isolated finding in the fetal period of this anatomical variation that associates two anomalies of the systemic venous system, we should note the importance of its postnatal echocardiographic confirmation to rule out congenital heart disease that is difficult to diagnose during the prenatal period. Likewise, prior to the echocardiographic confirmation carried out by the pediatric cardiologist, we should stress the importance of the pediatrician diagnosis in the first physical examination and of the family history to rule out possible critical congenital heart disease or possible associated syndromes. The prenatal diagnosis of VCSIP with agenesis of the VCSD will lead to a high degree of suspicion of these associated pathologies and therefore may lead to early clinical action.

Introdução: as anomalias no desenvolvimento do sistema venoso sistêmico são uma entidade rara, cujo diagnóstico ecocardiográfico pré-natal e pós-natal pode ser um grande desafio. Por um lado, pela sua baixa incidência e, por outro, pela dificuldade em realizar corretamente os planos ecocardiográficos. No entanto, seu diagnóstico é vital devido à associação com cardiopatias congênitas ou anormalidades cromossômicas. Objetivo: descrever dois casos de rara anomalia congênita cardiovascular. A persistência da veia cava superior esquerda com agenesia da veia cava superior direita é uma dessas anomalias descritas cuja incidência é muito baixa quando ambas as variações ocorrem juntas. Casos clínicos: apresentamos dois casos de recém-nascidos sem antecedentes pessoais ou familiares significativos, diagnosticados no pré-natal e cujos achados ecocardiográficos foram confirmados no período pós-natal. Conclusões: dado o achado isolado no período fetal desta variação anatômica que associa duas anomalias do sistema venoso sistêmico, devemos ressaltar a importância de sua confirmação ecocardiográfica pós-natal para descartar cardiopatia congênita de difícil diagnóstico no pré-natal . Da mesma forma, antes da confirmação ecocardiográfica que será realizada pelo cardiologista pediátrico, ressaltamos a importância do pediatra no primeiro exame físico e na história familiar para afastar possíveis cardiopatias congênitas críticas ou possíveis síndromes associadas. O diagnóstico pré-natal de VCSIP com agenesia do VCSD permitirá ter um alto grau de suspeita dessas patologias associadas e, portanto, realizar uma ação clínica precoce.

Persistent left superior vena cava as an incidental finding in the introduction of a transient pacemaker: A case report / Vena cava superior izquierda persistente como hallazgo incidental en la introducción de un marcapasos transitorio: informe de un caso

The persistent left superior vena cava is the most common venous anomaly in the systemic drainage in adults and tends to be asymptomatic. The persistent left superior vena cava causes rhythm disorders such as tachyarrhythmias or bradyarrhythmias. We report a case of persistent left superior vena cava diagnosed in a 53-year-old female patient admitted due to an acute coronary syndrome associated with unstable bradycardia. A transvenous peacemaker impressed the left atrium; therefore, a transthoracic echocardiogram was required to diagnose persistent left superior vena cava. The patient needed management with percutaneous intervention; she had an adequate evolution and subsequent discharge from the intensive care unit

La vena cava superior izquierda persistente es la anomalía venosa más frecuente en el drenaje sistémico en adultos; tiende a ser asintomática, pero causa trastornos del ritmo como taquiarritmias o bradiarritmias. Se presenta un caso de vena cava superior izquierda persistente diagnosticada en una paciente de 53 años hospitalizada por un síndrome coronario agudo asociado a bradicardia inestable. Un marcapasos transvenoso dejó una impresión en la aurícula izquierda, por lo que se requirió un ecocardiograma transtorácico para diagnosticar la vena cava superior izquierda persistente. La paciente necesitó una intervención percutánea con una adecuada evolución y se le dio el alta de la unidad de cuidados intensivos.

Abordaje quirúrgico del síndrome de vena cava superior asociado a neoplasia maligna: revisión narrativa / Surgical approach to superior vena cava syndrome associated with malignant neoplasm: A narrative review

Introducción: El síndrome de vena cava superior (SVCS) es una patología poco frecuente, asociado en la mayoría de los casos a neoplasias de origen maligno; la cual en estadios avanzados representa una urgencia médico-oncológica que compromete la vida del paciente. Propósito de la revisión: El objetivo de la revisión es delinear el rol de las diferentes alternativas quirúrgicas y percutáneas para el tratamiento del SVCS. Buscamos reportes en donde se incluye los resultados de mejor supervivencia para los distintos tratamientos actuales. Recientes hallazgos: La literatura médica describe tratamientos como la radioterapia, la quimioterapia, el bypass, la terapia endovascular y la reconstrucción vascular para el manejo del SVCS, sin embargo, no todos responden con la misma eficacia al momento de presentarse una urgencia vital por SVCS; esto producto de los factores intrínsecos y extrínsecos del paciente. Dentro de los factores extrínsecos destaca la condición social la cual, se convierte en un reto al momento de realizar un manejo integral del paciente en ciudades fronterizas, donde los datos que conoce el médico sobre el paciente son limitados o el paciente no tiene un diagnóstico previo que permita orientar el manejo. Conclusiones: El punto de partida del tratamiento del paciente con SVCS consiste en diferenciar la emergencia y la estabilidad mediante la tabla de clasificación de gravedad. En casos emergentes se tiene 2 alternativas: la terapia endovascular y la radioterapia. El abordaje quirúrgico con Baypass está contraindicado y el tratamiento definitivo con reconstrucción vascular con prótesis tiene ventajas y desventajas que deben definirse en forma individual considerando la etiología del cáncer asociado.

Introduction: Superior vena cava syndrome (SVCS) is a rare pathology, associated in most cases with neoplasms of malignant origin; which in advanced stages represents a medical-oncological emergency that compromises the patient's life. Purpose of the review: The objective of the review is to outline the role of the different surgi-cal and percutaneous alternatives for the treatment of SVCS. We look for reports that include the best survival results for the different current treatments. Recent findings: The medical literature describes treatments such as radiotherapy, chemo-therapy, bypass, endovascular therapy and vascular reconstruction for the management of SVCS, however, not all respond with the same efficacy at the time of a vital emergency. by SVCS; this product of the intrinsic and extrinsic factors of the patient. Among the extrinsic fac-tors, the social condition stands out, which becomes a challenge when carrying out an integral management of the patient in border cities, where the data that the doctor knows about the patient is limited or the patient does not have a previous diagnosis. to guide management. Conclusions: The starting point of the treatment of the patient with SVCS consists in differentiating the emergency and the stability by means of the severity classification table. In emergent cases there are 2 alternatives: endovascular therapy and radiotherapy. The surgical approach with Baypass is contraindicated and the definitive treatment with vascular reconstruc-tion with a prosthesis has advantages and disadvantages that must be defined individually considering the etiology of the associated cancer.

Malformación de Dandy-Walker asociada a malformaciones extracraneales en un neonato / Dandy-Walker malformation associated with extracranial abnormalities in a newborn

La hidrocefalia es una condición clínica que consiste en un cúmulo de líquido cefalorraquídeo a nivel encefálico. Una de las causas, poco frecuente, es el síndrome de Dandy-Walker. Se presenta el caso de un recién nacido con diagnóstico prenatal de hidrocefalia secundaria a una malformación de Dandy-Walker y sospecha de genitales ambiguos. Tras el nacimiento, se confirma el diagnóstico prenatal de malformación de Dandy-Walker asociado a manifestaciones extracraneales poco frecuentes como hipospadias interescrotal y dilatación del seno coronario secundario a persistencia de la vena cava superior izquierda. Con este caso clínico queremos exponer la complejidad del síndrome de Dandy-Walker debido a sus múltiples asociaciones, que marcarán el pronóstico del paciente y la necesidad de tratamiento multidisciplinar.

Hydrocephalus is a clinical condition that consists of an accumulation of cerebrospinal fluid around the brain; Dandy-Walker syndrome is a rare cause of it. We present the case of a newborn with prenatal diagnose of hydrocephalus due to a Dandy-Walker malformation, as well as ambiguous genitalia. After birth, diagnosis of Dandy-Walker malformation associated with uncommon extracranial manifestations is confirmed. Specifically, the baby presents interscrotal hypospadias and coronary sinus dilatation due to the persistence of the left superior vena cava. With the exposition of this case, we bring out the complexity of the Dandy-Walker syndrome due to the malformations associated with it; the ones that will determine the prognosis and the need of a multidisciplinary treatment

Heterotopic heart transplantation as a left ventricular biological assistance: a new two-stage method proposal

Abstract Since Barnard's first heterotopic heart transplant in 1974, Copeland's method has been the greatest contribution to heterotopic transplants but has the drawback of donor's right ventricular atrophy. This new method proposes a modification in the anastomosis of the superior vena cava aiming to pre-serve donor's right ventricular function by decompressing the pulmonary territory and reducing the pulmonary arterial pressure, as a biological ventricular assist device. Finally, a second intervention is proposed, where a "twist" is performed to place the donor's heart in an orthotopic position after re-moval of the native heart. A pioneering research on this method received approval from the ethics committee of the Heart Institute of São Paulo. We believe that this method has the potential to im-prove quality of life in a selected group of patients.

The new surface landmarks for blind axillary vein puncture

Abstract Objective: To compare the efficacy of blind axillary vein puncture utilizing the new surface landmarks for the subclavian method. Methods: This prospective and randomized study was performed at two cardiology medical centers in East China. Five hundred thirty-eight patients indicated to undergo left-sided pacemaker or implantable cardioverter defibrillator implantation were enrolled, 272 patients under the axillary access and 266 patients under the subclavian approach. A new superficial landmark was used for the axillary venous approach, whereas conventional landmarks were used for the subclavian venous approach. We measured lead placement time and X-ray time from vein puncture until all leads were placed in superior vena cava. Meanwhile, the rate of success of lead placement and the type and incidence of complications were compared between the two groups. Results: There were no significant differences between the two groups in baseline characteristics or number of leads implanted. There were high success rates for both strategies (98.6% [494/501] vs. 98.4% [479/487], P=0.752) and similar complication rates (14% [38/272] vs. 15% [40/266], P=0.702). Six cases in the control group developed subclavian venous crush syndrome and five had pneumothorax, while neither pneumothorax nor subclavian venous crush syndrome was observed in the experimental group. Conclusion: We have developed a new blind approach to cannulate the axillary vein, which is as effective as the subclavian access, safer than that, and also allows to get this vein without the guidance of fluoroscopy, contrast, or echography.

Diagnóstico prenatal de la vena cava superior izquierda persistente / Prenatal diagnosis of persistent left superior vena cava

RESUMEN Introducción: La persistencia de la vena cava superior izquierda (VCSIP) es la anomalía más común del sistema venoso torácico y puede estar asociadas a otras malformaciones cardiacas. Objetivo: Describir las características ecocardiográficas de la VCSIP y su relación con los factores de riesgo y anomalías asociadas. Método: Se realizó un estudio descriptivo, longitudinal y retrospectivo, con el propósito de contribuir al estudio del diagnóstico prenatal de la VCSIP mediante el análisis de diferentes indicadores. La muestra estuvo conformada por 62 casos con diagnóstico prenatal de VCSIP, en fetos entre 22 y 35 semanas, examinados en el Servicio de Ecocardiografía Fetal del Cardiocentro Pediátrico William Soler, durante el período comprendido desde enero de 2008 hasta mayo de 2012. Resultados: La VCSIP se presentó asociada a cardiopatías congénitas en 33 de los 62 casos. Dentro de estas, las más frecuentes fueron las anomalías troncoconales (38,46%). Conclusiones: La VCSIP es de fácil reconocimiento mediante el ultrasonido de pesquisa y constituye un hallazgo importante para el diagnóstico de las cardiopatías congénitas; por lo cual, ante su sospecha -debido a su frecuente asociación con cardiopatías congénitas- se sugiere ofrecer a la gestante una evaluación detallada del corazón fetal.

ABSTRACT Introduction: The persistence of the left superior vena cava (PLSVC) is the most common anomaly of the thoracic venous system and may be associated with other cardiac malformations. Objective: To describe the echocardiographic characteristics of PLSVC and its relationship with risk factors and associated abnormalities. Method: A descriptive, longitudinal and retrospective study was carried out, in order to contribute to the study of the prenatal diagnosis of PLSVC through the analysis of different indicators. The sample consisted of 62 cases with a prenatal diagnosis of PLSVC, in fetuses between 22 and 35 weeks, examined in the Fetal Echocardiography Department of the Cardiocentro Pediátrico William Soler, during the period from January 2008 to May 2012. Results: The PLSVC was associated with congenital heart disease in 33 of the 62 cases. Among these, the most frequent were conotruncal heart defects (38.46%). Conclusions: The PLSVC is easily recognized by screening ultrasound and represents an important finding for the diagnosis of congenital heart disease; therefore, given its suspicion -due to its frequent association with congenital heart disease- it is suggested to offer the pregnant woman a detailed evaluation of the fetal heart.

Ablación de reentrada nodal en vena cava superior izquierda persistente y ausencia de vena cava superior derecha / Nodal re-entry in patient with persistent left superior vena cava and absent right superior vena cava

Resumen La taquicardia por reentrada nodal es la arritmia más frecuente entre las taquicardias supraventriculares paroxísticas; la variedad lenta-rápida es la más común. En muchos casos la ablación de la vía lenta es el tratamiento definitivo y puede llegar a tener alta tasa de éxito y baja frecuencia de complicaciones. La presencia de una vena cava superior izquierda y la ausencia de vena cava superior derecha con drenaje venoso sistémico superior al seno coronario es una malformación congénita poco frecuente, la cual genera alteraciones anatómicas que dificultan el abordaje tradicional para la ablación de esta arritmia. Se presenta el caso de una paciente con esta condición en la que fue exitosa la ablación mediante ecocardiografía intracardiaca y reconstrucción tridimensional.

Abstract Nodal re-entrant tachycardia is the most common arrhythmia among the paroxysmal atrioventricular tachycardias, with the slow-fast variant being the most common. In many cases, the ablation of the slow pathway is the definitive treatment in many cases, often with a high rate of success and low frequency of complications. The presence of a left superior vena cava and the absence of a right superior vena cava with systematic venous drainage above the coronary sinus is a rare congenital malformation that leads to anatomical anomalies. These make it difficult to use the traditional approach for the ablation of this arrhythmia. The case is presented of a patient with this condition in which the ablation was successful using intracardiac echocardiography and three-dimensional reconstruction.

Vena cava superior izquierda persistente, agenesia de vena cava superior derecha e insuficiencia tricuspídea / Persistent left superior vena cava, absence of the right superior vena cava and tricuspid regurgitation

Resumen La persistencia de una vena cava superior izquierda en la población general representa una de las malformaciones del retorno venoso torácico más frecuentes, sin embargo su asociación con agenesia de la vena cava superior derecha e insuficiencia tricuspídea son hallazgos extremadamente infrecuentes.

Abstract The persistence of a left superior vena cava in the general population represents one of the most frequent thoracic venous return malformations, however its association with abscence of the right superior vena cava and tricuspid regurgitation are extremely rare findings.

Tratamiento intervencionista con stent para el síndrome de vena cava superior con complicación por el uso de marcapasos en un lactante / Stent interventional treatment of superior vena cava syndrome with a pacemaker placement complication in an infant

Resumen Introducción: El marcapasos epicárdico es el método preferido en recién nacidos y niños que pesan menos de 10 kg. Sin embargo, su colocación cuenta con una alta incidencia de fracaso prematuro. Caso clínico: Se presenta el caso de un lactante que requirió la colocación de un marcapasos por bloqueo atrioventricular congénito diagnosticado prenatalmente. El paciente presentaba estenosis crítica de la vena cava superior como complicación secundaria a los cables del marcapasos. La resolución intervencionista mediante un stent en el sitio de estenosis corrigió el síndrome de vena cava superior de forma efectiva. Conclusiones: Este caso representa una alternativa clínicamente viable en el tratamiento del síndrome de vena cava superior, que puede emplearse después de otras terapias convencionales que hayan fallado.

Abstract Background: Epicardial pacemaker is the preferred method for newborns and children under 10 kg of weight. However, its placement presents a high rate of premature failure. Case report: The case of an infant requiring placement of pacemaker by congenital atrioventricular block with prenatal diagnosis is described. The patient showed critical stenosis of the superior vena cava as a complication. Interventional resolution using a stent at the site of stenosis corrected the superior vena cava syndrome effectively. Conclusions: This case represents a clinically viable alternative for the treatment of superior vena cava syndrome, which can be used after other conventional therapies have failed.

Utilidad de la ecocardiografía de contraste con solución salina agitada en el diagnóstico de la persistencia de vena cava superior izquierda / Usefulness of contrast echocardiography with agitated saline at diagnosis of persistence of the left superior vena cava

Resumen La ecocardiografía contrastada con solución salina agitada es una modalidad de imagen establecida. Este método es usualmente utilizado para el diagnóstico de foramen oval permeable, shunts intracardiacos, anomalías del drenaje venoso y shunts intrapulmonares. En este artículo se revisarán aspectos generales de la ecocardiografía contrastada con solución salina, así como métodos diagnósticos enfocados principalmente a la detección de la persistencia de vena cava superior izquierda y sus variantes.

Abstract Echocardiography contrasted with agitated saline solution is an established imaging modality. This method is usually used for the diagnosis of patent foramen ovale, intracardiac shunts and venous drainage abnormalities. In this article, general aspects of the echocardiography contrasted with saline solution will be reviewed, as well as diagnostic methods focused mainly on the detection of the persistence of the left superior vena cava and its variants.

Fibrosis mediastínica post-irradiación: un factor a tener en cuenta en cirugía cardiaca / Post-radiation mediastinal fibrosis: a factor to take into account in cardiac surgery

Resumen La radioterapia mediastínica es uno de los tratamientos fundamentales de las enfermedades malignas torácicas, pero también representa una causa potencial de complicaciones, tanto a corto como a largo plazo. Se presenta una serie de casos de rotura de vena innominada durante esternotomía media en pacientes con fibrosis mediastínica. Los tres casos descritos corresponden a pacientes intervenidos de cirugía cardiaca con antecedentes de radioterapia mediastínica, entre 15-30 años antes de la cirugía, por tres diferentes enfermedades malignas (linfoma de Hodgkin, timoma y cáncer de mama). En los tres casos se reporta rotura de la vena innominada, con desinserción de su origen en la vena cava superior debido a fibrosis mediastínica intensa.

Abstract Although mediastinal radiotherapy is one of the basic treatments of malignant thoracic diseases, it is also a potential cause of short and long-term complications. A series of cases of rupture of the innominate vein during sternotomy are presented in patients with mediastinal fibrosis. The three cases described correspond to patients intervened by cardiac surgery, with a history of mediastinal radiotherapy between 15 to 30 years before the surgery, due to three different malignant diseases (Hodgkin lymphoma, thymoma, and breast cancer). In the three cases, a rupture of the innominate vein is reported; with de-insertion of its origin in the superior vena cava sue to intense mediastinal fibrosis.

Superior vena cava syndrome: endovascular management / Tratamento endovascular da síndrome da veia cava superior

The objective of management of superior vena cava syndrome (SVCS) is to promptly alleviate the uncomfortable symptoms. Conventional approaches do not always achieve results as rapidly as endovascular management with stent placement. Objectives To report the experience with endovascular management of SVCS of a Vascular and Endovascular Surgery Service at a Brazilian university hospital. Methods Symptomatic type III SVCS cases were managed with angioplasty and stent placement in 28 patients aged from 37 to 68 years, between 2002 and 2012. The etiology of SVCS was lung or thoracic cancer in 18 patients, while occlusion of the vein for prolonged use of catheters was the cause in the other 10 cases. Results Superior vena cava occlusion repair was not possible in one oligosymptomatic patient with a very severe lesion. Technical success was achieved in 96.4%. There were two deaths, one due to pulmonary embolism, 24 hours after a successful procedure, and the other due to compression of the airways by tumor mass some hours after the procedure. Clinical success was achieved in all cases of technical success, including one patient who died suddenly, after total regression of SVCS symptoms. Symptoms disappeared 24 hours and 48 hours after management in16 and 8 patients respectively; improvement was slower but progressive after 48 hours in the remaining patients. Conclusions Endovascular stent placement was effective for management of SVCS, with good technical and clinical success rates and provided prompt relief from symptoms

O tratamento da síndrome da veia cava superior (SVCS) visa aliviar, rapidamente, os sintomas desconfortáveis. Os resultados das abordagens convencionais nem sempre são tão rápidos quanto os do tratamento endovascular com colocação de stent. Objetivos Relatar a experiência de um Serviço de Cirurgia Vascular e Endovascular de hospital universitário brasileiro com o tratamento endovascular da SVCS. Métodos Vinte e oito pacientes com SVCS sintomática tipo III foram tratados com angioplastia e colocação de stent, entre 2002 e 2012. A idade variou entre 37 e 68 anos. A SVCS resultou de doença neoplásica pulmonar ou torácica em 18 pacientes e de oclusão da veia por uso prolongado de cateteres em 10 pacientes. Resultados Não foi possível reparo da oclusão da veia em um paciente oligossintomático com lesão bastante grave. O sucesso técnico foi de 96,4%. Houve duas mortes, sendo uma por embolia pulmonar 24 horas após procedimento bem-sucedido e outra por compressão das vias aéreas por massa tumoral algumas horas após o procedimento. Houve sucesso clínico em todos os casos de sucesso técnico, incluindo o paciente que faleceu de repente após ter apresentado regressão total dos sintomas. Os sintomas desapareceram em 24 e 48 horas após o tratamento em, respectivamente, 16 e oito pacientes. A melhora foi mais lenta, embora progressiva, após 48 horas nos demais pacientes. Conclusões A colocação de stent endovascular no tratamento da SVCS foi eficaz, com boas taxas de sucesso técnico e clínico e alívio mais rápido dos sintomas

Tratamiento endovascular del síndrome de Budd-Chiari, a propósito de un caso / Endovascular management for Budd-Chiari syndrome: A case report

El síndrome de Budd-Chiari consiste en la oclusión de la circulación de salida venosa del hígado, desde las sinusoides hepáticas hasta la unión de la vena cava inferior con la aurícula derecha. Esta entidad es muy rara. Las causas son primarias, obstrucción congénita de la vena cava o trombosis espontánea, o secundarias, compresión extrínseca. Si no se trata la obstrucción esta enfermedad, lleva a cirrosis hepática. Se presenta un caso del síndrome de Budd-Chiari de causa primaria por obstrucción membranosa, tratado por vía endovascular de manera exitosa y con resolución de todos los síntomas. Es el primer reporte en Colombia de este tipo de tratamiento (AU)

Budd-Chiari syndrome refers to the occlusion of the venous outflow of the liver, from the hepatic sinusoids to the junction of the inferior vena cava with the right atrium. This entity is very rare. The causes can be divided in primary (congenital vena cava obstruction or spontaneous thrombosis), and secondary (extrinsic compression). If the obstruction is not treated, leads to liver cirrhosis. In this article, we present a case of Budd-Chiari syndrome, primary due to membranous obstruction, successfully managed by the endovascular route with resolution of all symptoms. To our knowledge, this is the first case reported in Colombia utilizing this type of treatment (AU)

The outcomes of superior cavopulmonary connection operation: a single center experience

Abstract Introduction: The superior cavopulmonary connection operation is one of the stages of the palliative surgical management for patients with functionally single ventricle. After surviving this stage, the patients are potential candidates for the final palliative procedure: the Fontan operation. Objectives: This study aimed to analyze the outcomes of superior cavopulmonary connection operations in our center and to identify factors affecting the survival and the progression to Fontan stage. Methods: The outcomes of 161 patients were retrospectively analyzed after undergoing superior cavopulmonary connection operation in our center between 2005 and 2015. Results: The early mortality rate was 2.5%. Five (3.1%) patients underwent takedown of the superior cavopulmonary connection. The rate of exclusion from the Fontan stage was 8.3%. Statistical analysis revealed that elevated mean pulmonary artery pressure preoperatively and the prior palliation with pulmonary artery banding were risk factors for both early mortality and takedown; however, the age, the morphology of the single ventricle and the type of operation were not considered risk factors. Conclusion: The superior cavopulmonary connection operation can be performed with low rate mortality and morbidity; however, the elevated mean pulmonary artery pressure preoperatively and the prior pulmonary artery banding are associated with poor outcomes.

Implante de marcapasso em paciente com veia cava superior esquerda persistente / Pacemaker implant in patients with persistent left superior vena cava

A persistência da veia cava superior esquerda, embora rara, é a anomalia venosa mais encontrada no tórax, com prevalência de 0,3-0,5% na população geral. Em associação com a cardiopatia congênita, sua prevalência aumenta para 2,8-4,3%. Pacientes com cardiopatia congê- nita que necessitem de tratamento cirúrgico podem evoluir, durante a cirurgia, com bloqueio atrioventricular total e necessitar de implante de marcapasso. Apresentamos o caso de uma paciente com cardiopatia congênita, incluindo veia cava superior esquerda persistente, submetida a várias intervenções cirúrgicas, evoluindo com bloqueio atrioventricular total e necessitando de implante de marcapasso bicameral

The persistence of the left superior vena cava, though rare, is the most common venous anomaly in the chest, with a prevalence of 0.3%-0.5% in the general population. When combined with congenital heart disease, its prevalence increases to 2.8%-4.3%. Patients with congenital heart disease who require surgical treatment might evolve to a condition of complete heart block, and consequently require a pacemaker implant. We will present the case of a patient with congenital heart disease, including persistent left superior vena cava, which has been subjected to several surgical interventions, evolving with total ventricular atrial block and requiring a bicameral pacemaker implant

Cateteres venosos centrais de inserção periférica: alternativa ou primeira escolha em acesso vascular? / Peripherally inserted central venous catheters: alternative or first choice vascular access?

Contexto: Os cateteres venosos centrais de inserção periférica (PICC) são dispositivos intravenosos, introduzidos através de uma veia superficial ou profunda da extremidade superior ou inferior até o terço distal da veia cava superior ou proximal da veia cava inferior. Apresentam maior segurança para infusão de soluções vesicantes/irritantes e hiperosmolares, antibioticoterapia, nutrição parenteral prolongada (NPT) e uso de quimioterápicos; demonstram reduzido risco de infecção em comparação a outros cateteres vasculares e maior relação custo/benefício se comparados ao cateter venoso de inserção central (CVCIC). Objetivos: Apresentar os resultados de implantes de PICCs ecoguiados e posicionados por fluoroscopia realizados no Hospital e Maternidade São Luiz (HMSL) Itaim, Rede D'or, Brasil. Métodos: Estudo prospectivo, não randomizado, realizado entre fevereiro de 2015 e novembro de 2016. Utilizou-se protocolo pré-estabelecido pela instituição em casos de solicitação de acesso vascular. Foram analisadas indicações, doenças prevalentes, tipo do cateter implantado, sucesso técnico, complicações relacionadas ao cateter, e estabelecidos critérios de inclusão e exclusão. Resultados: Solicitados 256 acessos vasculares, sendo implantados 236 PICCs (92,1%) e 20 CVCICs (7,9%). Principais indicações: antibioticoterapia prolongada (52,0%), NPT (19,3%) e acesso venoso difícil (16,0%). Houve sucesso técnico em 246 cateteres implantados (96,1%). A veia basílica direita foi a principal veia puncionada em 192 pacientes (75,0%), seguida da braquial direita em 28 pacientes (10,9%). Conclusões: O implante dos PICCs ecoguiados e posicionados por fluoroscopia demonstrou baixa incidência de complicações, reduzidos índices de infecção e é seguro e eficaz em casos de acessos vasculares difíceis, sendo esses cateteres considerados dispositivos de escolha em acesso vascular central

Background: Peripherally inserted central catheters (PICC) are intravenous devices inserted through a superficial or deep vein of an upper or lower extremity and advanced to the distal third of the superior vena cava or proximal third of the inferior vena cava. They offer the advantages of greater safety for infusion of vesicant/irritant and hyperosmolar solutions and enable administration of antibiotics, prolonged parenteral nutrition (PPN), and chemotherapy agents. They also involve reduced risk of infection compared to other vascular catheters and are more cost-effective than centrally inserted venous catheters (CICVC). Objectives: To present the results of our team's experience with US-guided and fluoroscopy-positioned PICC placement at the Hospital and Maternidade São Luiz (HMSL) Itaim, Rede D'or, Brazil. Methods: This was a prospective, non-randomized study, conducted from February 2015 to November 2016. The institution's preestablished protocol was followed when vascular access was requested. Indications, prevalent diseases, type of catheter implanted, technical success, and complications related to the catheters were analyzed and inclusion and exclusion criteria are described. Results: A total of 256 vascular accesses were requested, and 236 PICCs (92.1%) and 20 CICVCs (7.9%) were implanted. The main indications were as follows: prolonged antibiotic therapy (52%), PPN (19.3%), and difficult venous access (16%). Technical successes was achieved in 246 catheter placements (96.1%). The right basilic vein was the most common vein punctured for access, in 192 patients (75%), followed by the right brachial vein, in 28 patients (10.9%). Conclusions: Ultrasound-guided and fluoroscopy-positioned PICC placement had a low incidence of complications, reduced infection rates, and proved safe and effective in cases of difficult vascular access. PICCs can be considered the devices of choice for central vascular access