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.

Surgical Planning by 3D Printing for Primary Cardiac Schwannoma Resection

We report herein a case of benign cardiac schwannoma in the interatrial septum. A 42-year-old woman was transferred from a clinic because of cardiomegaly as determined by chest X-ray. A transthoracic echocardiography and chest computed tomography examination revealed a huge mass in the pericardium compressing the right atrium, superior vena cava (SVC), left atrium, and superior pulmonary vein. To confirm that the tumor originated from either heart or mediastinum, cine magnetic resonance imaging was performed, but the result was not conclusive. To facilitate surgical planning, we used 3D printing. Using a printed heart model, we decided that tumor resection under cardiopulmonary bypass (CPB) through sternotomy would be technically feasible. At surgery, a huge tumor in the interatrial septum was confirmed. By incision on the atrial roof between the aorta and SVC, tumor enucleation was performed successfully under CPB. Pathology revealed benign schwannoma. The patient was discharged without complication. 3D printing of the heart and tumor was found to be helpful when deciding optimal surgical approach.

Superior vena cava thrombosis with peripartum dilated cardiomyopathy

A 30 years multiparous female with history of emergency caesarean section 10 days back was referred to us with cough, severe breathlessness at rest, orthopnea with pain in neck and arms. Clinical examination revealed signs of heart failure. Echocardiography showed ejection fraction of 15%, with no right ventricular strain. A diagnosis of peripartum cardiomyopathy was made. Doppler ultrasound of neck veins showed bilateral internal jugular vein thrombosis. Subsequent multislice CT examination showed thrombosis of superior vena cava and both internal jugular veins [with collateral formation] and pulmonary embolism. There were no mediastinal abnormalities on the CT scan. Her thrombophilia screen and CT scan brain was normal. She was managed in collaboration with cardiologist. Following treatment with subcutaneous enoxaparin therapy and warfarin her symptoms of upper limb pain improved. She responded very well to medical therapy for heart failure with marked improvement of NYHA functional class

Utilidad de la Endoprótesis en Síndrome de vena cava superior / Benefits of the endoprosthesis in superior vena cava syndrome

El síndrome de vena cava superior, se corresponde a la obstrucción de la misma, siendo su causa más frecuente la etiología neoplásica. Objetivos: Se realiza el análisis y la presentación del siguientecaso clínico con el objetivo de considerar la utilidad de laendoprótesis. Caso Clínico: Paciente de 61 años, masculino que ingresa porsíndrome de vena cava superior. Se le realiza Tac Torácica con presencia de tumor hiliar derecho en ubicación retrocavopretraqueal que estenosa a la vena cava superior. Con mala evolución del cuadro se decide colocación de endoprotesis vascular. Aliviando la sintomatología para luego realiza eldiagnostico. Discusión: dado que el tratamiento de esta entidad es tratar lacausa de la obstrucción, se debe tener en cuenta la repercusión que genera, llevando inclusive a la muerte del paciente. Por lo que es de utilidad resolver la dificultad con la colocación de una endoprotesis, para luego estudiar y diagnosticar la etiología y asícondicionar un tratamiento adecuado...

The superior vena cava syndrome, corresponds to the obstruction of the superior vena cava, and its most common cause neoplastic etiology. Objectives: Analysis and presentation of the following case inorder to consider the usefulness of the endoprosthesis is made. Case presentation: A 61 year old male was admitted with superior vena cava syndrome. He takes Tac Thoracic presence of right hilar tumor location retrocavopretraqueal stenosing thesuperior vena cava. With bad symptom onset vascular endoprosthesis placement is decided. Relieving the symptoms and then performs the diagnosis. Discussion: Because the treatment of this entity is to treat thecause of the obstruction, must take into account the impact it creates, even leading to death. It is useful to solve the difficulties with the placement of a stent, and then study and diagnose theetiology and thus an appropriate treatment...

IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart

A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up.

Stenting of the Superior Vena Cava and Left Brachiocephalic Vein with Preserving the Central Venous Catheter in Situ

Stenting of the central veins is well established for treating localized venous stenosis. The techniques regarding catheter preservation for central venous catheters in the superior vena cava have been described. We describe here a method for stent implantation in the superior vena cava and the left brachiocephalic vein, and principally via a single jugular venous puncture, while saving a left sided jugular central venous catheter in a patient suffering from central venous stenosis of the superior vena cava and the left brachiocephalic vein.

Rare variants of total anomalous pulmonary venous connection to coronary sinus-echocardiographic recognition and surgical correction.

Total anomalous pulmonary venous connection (TAPVC) to the coronary sinus is a well-known entity but variations in connection sites are known to occur, the commonest among them is mixed connection. Here we describe two rare variants of TAPVC to coronary sinus. Group I (3 cases) in which there were dual sites of connection and group II (2 cases), TAPVC to coronary sinus was associated with a persistent left superior vena cava (LSVC). This was seen in total number of 45 cases of isolated TAPVC and 8 cases of TAPVC to coronary sinus between 2000 and December 2005 in our institute. All patients underwent surgical correction. In both of these groups, surgical correction may pose a challenge, which is discussed.

Internal thoracic vein draining into the extrapericardial part of the superior vena cava: a case report

The internal thoracic veins are venae comitantes of each internal thoracic artery draining the territory supplied by it and usually unite opposite the third costal cartilage. This single vein enters the corresponding brachiocephalic vein. We present a variation of right internal mammary vein draining into superior vena cava in a 45-year-old male cadaver. Likely development and clinical significance of the vein are discussed.

As veias torácicas internas são veias comitantes de cada artéria torácica interna drenando o território suprido por ela e geralmente se unem em frente à terceira cartilagem costal. Esta única veia entra na veia braquicefálica correspondente. Apresentamos uma variação da veia mamária interna direita drenando para a veia cava superior em um cadáver masculino de 45 anos. O provável desenvolvimento e a significância clínica da veia são discutidos.

Functioning adrenocortical carcinoma with superior vena cava and upper airway obstructions.

BACKGROUND: Adrenocortical carcinoma (ACC) is one of the most aggressive endocrine malignancies with a dismal prognosis. Typically, the tumor is large and has regional invasion or distant metastasis at initial presentation. OBJECTIVE: To describe an unusual case of functioning ACC presenting with superior vena cava (SVC) and upper airway obstruction. MATERIAL AND METHOD: A 23-year-old man with cushingoid appearance was evaluated for a neck mass and SVC syndrome. Hormonal assessment and neck mass biopsy including immunohistochemistry study were performed RESULTS: Cushing's syndrome was confirmed by elevated 24-hr urinary free cortisol and no suppressible cortisol level after standard low dose (2 mg/day) of dexamethasone suppression test. Computerized tomography (CT) study revealed a huge left suprarenal mass and multiple mediastinal lymph nodes compressing SVC and trachea. Histopathological findings of the neck mass were compatible with metastatic ACC. CONCLUSION: The present report describes a functioning ACC patient with an unusual metastatic site causing SVC and upper airway obstruction. His hospital course was progressively worsened due to peptic perforation and decompensated respiratory failure, which led him to expire.

Pregnancy complicated by superior vena cava thrombosis and pulmonary embolism in a patient with behcet disease and the use of heparin for treatment

Behcet disease is a multisystemic vasculitis of unknown origin. Vascular involvement has been considered to result from systemic vasculitis, occurs in 5-10% of these patients. We report a 34-year-old pregnant woman complicated by superior vena cava thrombosis, and pulmonary embolism in a patient with Behcet disease

Vena cava superior izquierda y anomalías con que se asocia / Left superior vena cava and associated anomalies

Para conocer la frecuencia de la vena cava superior izquierda (VCSI) persistente y las cardiopatías congénitas asociadas, se estudiaron con el método secuencial segmentario 66 corazones de 1,277 necropsias pertenecientes al Instituto Nacional de Cardiología "Ignacio Chávez". Se determinó: situs atrial, tipos y modos de conexión atrioventricular y ventriculoarterial, morfología del retorno venoso sistémico superior y las cardiopatías asociadas. 32 corazones tuvieron situs solitus (grupo I) y 34 situs simétrico (grupo II) (30 con dextroisomerismo y 4 con levoisomerismo). El grupo I mostró doble vena cava superior, la izquierda se continuó con el seno venoso coronario; 25 sin tronco venoso braquiocefálico izquierdo, presente en 6, 5 con luz estrecha y dilatada en una (atresia de la válvula de Tebesio). Las cardiopatías congénitas más frecuentes fueron los defectos septales ventriculares, troncoconales y de la conexión atrioventricular. En el grupo II no existió seno venoso coronario y la conexión venosa se hizo en el techo atrial. 19 corazones presentaron doble vena cava superior y en 15 fue unilateral izquierda. Las cardiopatías congénitas presentes fueron complejas con patrones de asociación. La VCSI se origina por la persistencia de la continuidad de la vena cardinal común anterior izquierda con el cuerno izquierdo del seno venoso al no involucionar la porción proximal de dicha vena. Su presencia tiene interés quirúrgico cuando existen cardiopatías congénitas asociadas.

In order to asses the frequency of persistent left superior vena cava as well as the associated congenital heart disease, 66 hearts were studied from 1277 necropsies of the pathologic collection of the Instituto Nacional de Cardiología "Ignacio Chávez". They were analyzed with the sequential segmental approach. It was determined: atrial situs types and modes of atrioventricular and ventricular arterial connections, morphology of the superior systemic venous return and associated cardiopathies. 33 hearts had situs solitus (group I) and 34 hearts had isomeric situs (Group II) (30 with dextroisomerism and 4 with levoisomerism). The group I showed double superior vena cava, the left one had continuation with the sinus coronary; in 25 of them the left brachiocephalic vein was absent, in 6 this vein was present, 5 with narrow lumen and in one it was dilated (specimen with atresia of the Tebesian valve). The more frequent congenital heart disease were ventricular septal defects, troncoconal cardiopathies and anomalies in the atrioventricular connection. The hearts of group II did not have coronary sinus; the venous conections were in the atrial roof. 19 hearts had double superior vena cava and 15 specimens had only the left one. The congenital heart disease in this group were complex with multiple patterns of association. Left superior vena cava is developed as a consequence of persistence of the continuation of the left anterior and left common cardinal veins with the left horn of sinus venosus when the proximal segment of these veins did not disappear. The left superior vena cava has surgical significance when congenital heart disease is present. (Arch Cardiol Mex 2003; 73:175-184).

Pseudolesion in left lobe of the liver due to superior vena cava obstruction

We report a case of lymphoma in which abnormal strong enhancement in the medial segment of left lobe of liver during arterial phase of triphasic helical computed tomography due to superior vena cava obstruction

Utilización de "stents" autoexpansibles en el síndrome de vena cava superior / Use of selfexpandable endoprosthesis in upper cava vein syndrome

Antecedentes: el SVCS responde a causas malignas en más del 90 por ciento de los casos. La conjunción de disnea y encefalopatía previene sobre un rápido curso ominoso. El tratamiento con quimio y/o radioterapia (a excepción de los linfomas) no suele tener éxito inmediato. La cirugía de derivación resulta difícil y sumamente riesgosa en pacientes con poca expectativa de supervivencia prolongada. Objetivos: demostrar la utilidad del empleo de endoprótesis auto expansibles para repermeabilizar la VCS. Lugar de aplicación: Centro Asistencial Universitario. Diseño: estudio observacional retrospectivo. Material y método: entre 1994 y 1999 fueron tratados 21 pacientes portadores de SVCS, con endoprótesis autoexpansibles. Hubo 15 hombres y 6 mujeres. La edad media fue 53 años (rango 26-70). La etiología fue benigna en 2 casos (trombosis por catéter) y maligna en 19. Once pacientes fueron tratados previamente con quimio y/o radioterapia. La colocación del "stent" se efectuó por punción percutánea vía femoral en 18 casos y por vena basílica en 3. Se utilizó un introductor 10 F valvulado y pre-dilatación con balón de angioplastía de 8-10 mm de diámetro. Los "stents" implantados fueron del tipo Wallstent Boston Scientific, cuyo diámetro osciló entre 10-16 mm. Quince pacientes efectuaron posteriormente tratamiento con quimio y/o radioterapia. Resultados: todos los procedimientos resultaron técnicamente exitosos, con desaparición de los síntomas dentro de las primeras 24-48 hs. Un paciente en el cual se demoró la indicación falleció después de la implantación del "stent" como consecuencia de insuficiencia respiratoria por neoplasia avanzada. La supervivencia media fue de 7 meses. Conclusiones: la utilización de endoprótesis para recanalización de la VCS ofrece excelentes resultados en manos de equipos experimentados con rápida remisión del cuadro clínico

Atresia de veia cava superior / Superior vena cava syndrome: case report

Um caso de atresia de veia cava superior em uma criança de cinco anos é relatada. Essa anomalia rara apresentou como sintomatologia única uma circulaçÝo colateral exuberante em tronco, axilas e membros superiores. A dificuldade de manuseio da patologia e a quase inexistência de observaçSes similares na literatura justificam o presente relato

Persistencia de la vena cava superior izquierda: hallazgos escanográficos y significado clínico

Se presentan los hallazgos escanográficos de tres pacientes en quienes se documentó escanográficamente la persistencia anormal de la vena cava superior izquierda. Se revisa la embriología así como los hallazgos radiológicos y especialmente escanográficos en esta entidad, al igual que las implicaciones fisiopatológicas y clínicas de la misma. Se revisan los criterios diagnósticos en escanografía y los diagnósticos diferenciales.