Síndrome de vena cava superior: una serie de casos consecutivos en pacientes internados en un hospital terciario universitario / Superior vena cava syndrome: a consecutive case series of in-hospital patients in a tertiary teaching hospital

Introducción: el síndrome de vena cava superior resulta de la obstrucción del flujo sanguíneo a través de este vaso. Casi la totalidad de los casos en la actualidad se asocian con tumores malignos. Existen controversias acerca del manejo apropiado de este cuadro. Actualmente, las terapias endovasculares son consideradas de elección. Materiales y métodos: se recolectaron y describieron, a partir de datos de la historia clínica electrónica, los casos de pacientes mayores de 18 años internados ­de forma consecutiva, que desarrollaron el síndrome­ en el Hospital Italiano de Buenos Aires en 2021. Se constataron las características basales, los tratamientos recibidos y los desenlaces clínicos intrahospitaliarios de cada uno de ellos. Resultados: un total de cinco pacientes fueron incluidos en el presente estudio y seguidos durante su instancia intrahospitalaria. Todos los casos descriptos fueron secundarios a enfermedades oncológicas. La mayoría de los pacientes presentaron un cuadro de moderada gravedad según las escalas utilizadas. En cuatro de cinco pacientes se optó por terapias endovasculares y dos de ellos fallecieron durante la internación. Discusión: existen controversias respecto del tratamiento óptimo del síndrome de vena cava superior, y heterogeneidad en la práctica clínica. Los estudios futuros deberían centrarse en identificar a aquellos pacientes que más probablemente se beneficien de las estrategias terapéuticas endovasculares, anticoagulantes o antiagregantes. (AU)

Introduction: superior vena cava syndrome results from an obstruction of blood flow through this vessel. Currently, almost all cases are associated with malignancies. There are controversies about the optimal management of this syndrome. Endovascular therapies are considered the first-line therapy. Material and methods: we collected clinical, laboratory and pharmacological data from patients admitted at the Hospital Italiano de Buenos Aires, between January 1st and November 1st 2021 with a diagnosis o superior vein cava syndrome. Baseline characteristics, treatment strategies and clinical outcomes were recorded. Results: a total of five patients were included in the present study. All cases were malignancy-related. Most of the patientsdeveloped moderate symptoms. Four out of five patients were treated with endovascular therapies and two patients died during hospitalization. Discussion: controversies regarding optimal management of the superior vena cava syndrome remain. Future research should focus on identifying those patients who are most likely to benefit from endovascular, anticoagulant or antiplatelet therapeutic strategies. (AU)

The largest reported giant ascending aortic aneurysm presented with superior vena cava syndrome

Abstract Giant ascending aortic aneurysm is a rare condition. In this paper, we present an uncommon case of giant ascending aortic aneurysm with a maximal diameter of 14 cm in a 77-year-old woman presenting with unusual symptoms. The patient underwent a successful surgery involving ascending aortic replacement, and was discharged without any complication. After discharge, she was followed regularly and no major problem was observed in her control visits. To the best of our knowledge, our case is the largest ascending aortic aneurysm reported to date in the existing literature.

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.

Upper vena cava syndrome secondary to giant atrial myxoma

SUMMARY Cardiac myxoma is a benign neoplasm, which corresponds to the most common primary heart tumour, responsible for about 50% of the cases. In general, 75-80% of myxomas are located in the left atrium, 18% in the right atrium, and more rarely in the ventricles or multicentric. Right atrial myxoma, in particular, can obstruct the tricuspid valve, causing symptoms of right heart failure, peripheral oedema, hepatic congestion, and syncope. Systemic embolization occurs in 30% of cases, by either tumour fragmentation or total tumour detachment. In the present report, we present a case of a symptomatic patient, who showed a large right intra-atrial lesion, with consequent superior vena cava syndrome, and then underwent surgical resection at admission.

RESUMO O mixoma cardíaco é uma neoplasia benigna, que corresponde ao tumor primário mais comum do coração, responsável por cerca de 50% dos casos. De modo geral, 75 a 80% dos mixomas estão localizados no átrio esquerdo, 18% no átrio direito, e mais raramente, nos ventrículos ou multicêntricos. O mixoma atrial direito, em particular, pode obstruir a válvula tricúspide, causando sintomas de insuficiência cardíaca direita, edema periférico, congestão hepática e síncope. A embolização sistêmica ocorre em 30% dos casos, quer pela fragmentação do tumor ou pelo desprendimento total do mesmo. No presente relato, apresentamos um caso de uma paciente sintomática, que evidenciou grande lesão intra-atrial direita, com consequente síndrome da veia cava superior, sendo, então, submetida a ressecção cirúrgica na internação.

Conducta en el síndrome de vena cava superior (SVCS) / Management in superior vena cava syndrome

Objetivos: Mostrar la conducta realizada en este síndrome. Material y Métodos: Serie retrospectiva de 5 casos tratados entre el 1 de abril de 1995 y el 31 de marzo de 2013. Diagnóstico por imágenes y toma de biopsia con tratamiento específico posterior. Resultados: Todos se debieron a causas tumorales: 2 timomas (uno irresecable), 1 linfoma Hodgkin, 1 leiomiosarcoma de vena cava y 1 caso de cáncer pulmonar a pequeñas células. Fallecieron por progresión de enfermedad 2 casos, 2 están libres de enfermedad a 12 y 11 meses de tratamiento y 1 enfermo se perdió en el seguimiento. Conclusiones: Se define la necesidad de seguir un algoritmo que determine los pasos a seguir ante la presencia de este síndrome.

Objectives: Management in this syndrome. Material and Methods: Retrospective series of 5 cases treated between April 1, 1995 to March 31, 2013. Diagnostic imaging and taking biopsy with subsequent specific treatment. Results: All patients were due to tumor causes: 2 thymomas (one unresectable), 1 lymphoma Hodgkin, 1 Leiomyosarcoma of vena cava and 1 case of small cell lung cancer. Died of disease progression 2 cases, 2 are free of disease to 12 and 11 months of treatment and 1 patient was lost to follow-up. Conclusions: The need to follow an algorithm which determines the steps to follow in the presence of this syndrome is defined.

Dialysis Catheter-Related Superior Vena Cava Syndrome with Patent Vena Cava: Long Term Efficacy of Unilateral Viatorr Stent-Graft Avoiding Catheter Manipulation

Central venous catheters are the most frequent causes of benign central vein stenosis. We report the case of a 79-year-old woman on hemodialysis through a twin catheter in the right internal jugular vein, presenting with superior vena cava (SVC) syndrome with patent SVC. The clinically driven endovascular therapy was conducted to treat the venous syndrome with a unilateral left brachiocephalic stent-graft without manipulation of the well-functioning catheter. The follow-up was uneventful until death 94 months later.

Covered Stent Placement for the Treatment of Malignant Superior Vena Cava Syndrome: Is Unilateral Covered Stenting Safe and Effective?

OBJECTIVE: To evaluate the safety and efficacy of unilateral covered stent placement in patients with malignant superior vena cava (SVC) syndrome. MATERIALS AND METHODS: Between October 2008 and November 2012, expanded polytetrafluoroethylene-covered stent placement for malignant SVC syndrome was performed in 40 consecutive patients (35 men and five women; mean age, 61.4 years; range, 35-81 years). All covered stents were unilaterally placed within the SVC or across the venous confluence when needed to relieve venous obstruction and prevent tumor overgrowth, regardless of patency of contralateral brachiocephalic veins. RESULTS: Stent placement was technically successful in all patients. There were no major complications. Of the 37 patients symptomatic prior to stent placement, 34 (92%) experienced complete symptomatic relief 1-8 days after stent placement. Of the 29 patients who underwent covered stent placement across the venous confluence, nine patients had patent contralateral brachiocephalic veins prior to stent placement. However, no sign of SVC obstruction or contralateral upper extremity venous thrombosis was observed during the follow-up period. Kaplan-Meier analysis revealed median patient survival of 163 days. Stent occlusion occurred in four (10%) of 40 patents. Cumulative stent patency rates at 1, 3, 6, and 12 months were 95%, 92%, 86%, and 86%, respectively. CONCLUSION: Unilateral covered stent placement appears to be a safe and effective method for treating malignant SVC syndrome, despite the location of SVC occlusion.

An unusual cause of intraoperative acute superior vena cava syndrome.

Acute intraoperative superior vena cava (SVC) syndrome is an exceedingly rare complication in the cardiac surgical population. We describe the case of a 71-year-old female undergoing multi-vessel coronary artery bypass grafting who developed acute intraoperative SVC syndrome following internal thoracic artery harvest retractor placement. Her symptoms included severe plethora, facial engorgement and scleral edema, which was associated with hypotension and severe elevation of central venous pressure. Transesophageal echocardiography was crucial in the diagnosis, management, and optimal retractor placement ensuring adequate SVC flow. Potential causes of intraoperative SVC syndrome are reviewed as well as management options.

Síndrome de vena cava superior: forma inicial de presentacion en enfermedades neoplásicas / [Superior Cava Venous syndrome as presentation of neoplasic disease].

Superior Cava Venous Syndrome (SVCS) encompasses a constellation of signs and symptoms resulting from partial or complete obstruction of blood flow through the superior vena cava (SVC) to the right atrium. Thrombosis can be caused by intrinsic (primary) or extrinsic compression (with or without secondary thrombosis). The causes of SVCS can be grouped according to their etiology in non-neoplastic and neoplastic. The causes malignant correspond to 65-90

of them, thrombosis and nonmalignant conditions are another causes. The presentation may be acute, clinically characterized by dyspnea and a characteristic triad (facial, neck and arms edema, cyanosis and collateral circulation). Early detection improves prognosis and is based on clinical data and imaging studies. We describe 8 cases where the SVCS was the first manifestation of neoplastic diseases. Patients were predominantly of middle-aged, male, without association with consumption of tobacco and lymphoma was most frequent diagnosis.

Pseudoaneurisma de aorta como causa de síndrome da veia cava superior: relato de caso / Aortic pseudoaneurysm as cause of superior vena cava syndrome: a case report

A síndrome da veia cava superior representa o conjunto de sinais e sintomas decorrentes da sua obstrução. A síndrome tem como principais etiologias tumores malignos, como o carcinoma broncogênico, o linfoma e a metástase mediastinal. O câncer de pulmão é responsável por 80% dos casos, os linfomas mediastinais por 15%, e 5% correspondem às demais causas. Este relato de caso objetiva apresentar um caso incomum dessa síndrome, ocorrido em um paciente do sexo masculino após ferimento penetrante no tórax, resultando em pseudoaneurisma de arco aórtico e a síndrome da veia cava superior.

The superior vena cava syndrome represents the set of signs and symptoms resulting from obstruction of superior vena cava. The syndrome has as main causes malignant tumors such as bronchogenic carcinoma, lymphoma and mediastinal metastases. Lung cancer accounts for 80% of cases, mediastinal lymphomas by 15% and 5% correspond to other causes. This case report aims to present an unusual case of this syndrome, which occurred in a male patient after penetrating wound in the chest, which developed a pseudoaneurysm of the aortic arch and superior vena cava syndrome.

Leiomiosarcoma primario de mediastino que compromete el sistema de la vena cava superior: resección quirúrgica asociada a reconstrucción venosa / Primary leiomyosarcoma of the mediastinum involving the superior vena cava system: surgical resection and venous reconstruction

Mediastinal tumors have multiple presentation forms and various pathological origins. Sometimes they reach a large size and can involve the system of superior vena cava by compression or infiltration. We present a 48 year old man admited for dyspnea, headache and palpitations in decubitus, for the last two months. The X-ray and chest computed tomography showed an anterior mediastinal tumor and the tru-cut pathological study concluded leiomyosarcoma. He underwent surgical treatment; the tumor compromised the system of the superior vena cava and was resected with ligation and resection the left brachiocephalic trunk. Part of the superior vena cava was reconstructed with autologous pericardium. The right phrenic nerve was surrounded by the tumor mass and was resected in block. The patient recovered satisfactorily and was discharged on the eleventh day post surgery. The histopathologic study of the surgical specimen confirmed a leiomyosarcoma G2 (3,100 gr). Full adjuvant radiotherapy was done. At 15 months after surgery the patient is in good condition without evidence of recurrence or superior vena cava syndrome.

Los tumores del mediastino pueden tener múltiples formas de presentación y diversos orígenes anato-mopatológicos. A veces alcanzan gran tamaño y pueden comprometer por compresión o infiltración el sistema de la vena cava superior. Presentamos el caso de un hombre de 48 años que consultó por disnea, cefalea y palpitaciones en decúbito de dos meses de evolución. En la radiografía y en la tomografía computada de tórax se demostró un tumor de mediastino anterior, el estudio anatomopatológico mediante biopsia trucut concluyó leiomiosarcoma. Se realizó tratamiento quirúrgico, se resecó la masa tumoral que comprometía el sistema de la vena cava superior, se ligó y resecó el tronco venoso braquiocefálico izquierdo o innominado y se reconstruyó parte de la vena cava superior con parche de pericardio autólogo. El nervio frénico derecho se encontraba rodeado por la masa tumoral y se resecó junto con el tumor. El paciente evolucionó satisfactoriamente y fue dado de alta al décimo primer día post operado. El estudio de anatomía patológica de la pieza operatoria confirmó un leiomiosarcoma G2 de 3.100 gramos. Se completó tratamiento con radioterapia post operatoria. A 15 meses post operado el paciente se encuentra en buenas condiciones generales, sin evidencias de recidiva y sin síndrome de vena cava superior.

Tratamento cirúrgico da síndrome da veia cava superior causado por timoma invasivo / Surgical treatment of superior vena cava syndrome caused by invasive thymoma

Paciente do sexo masculino, branco, 57 anos, portador de síndrome da veia cava superior havia 3 meses, devido a timoma invasivo em mediastino médio e anterior, levando a comprometimento da veia cava superior intrínseca e extrinsecamente. Após avaliação por tomografia computadorizada e angiorressonância magnética de tórax, o paciente foi submetido à ressecção radical do timoma - derivação venosa da veia subclávia esquerda para átrio direito, com tubo de PTFE (politetrafluoroetileno). Relevante caso de timoma invasivo ocasionando a oclusão da veia cava superior. A evolução clínica, após 7 meses, foi considerada satisfatória.

We report on a case of a 57 years-old white male, patient, who presented superior vena cava syndrome (SVC) for 3 months, derived from an invasive thymoma in the medium and anterior mediastinum, compromising intrinsic and extrinsic to the SVC. After evaluation by computed tomography and magnetic angioresonance of the thorax, the patient underwent radical resection of the thymoma - bypass from left subclavian vein to right atrium, using polytetrafluoroethylene tube. Relevant case of invasive thymoma causing the occlusion of SVC. The clinic evolution of the patient after 7 months was considered satisfactory.

Superior vena cava syndrome after pulsatile bidirectional Glenn shunt procedure: perioperative implications.

Bidirectional superior cavopulmonary shunt (bidirectional Glenn shunt) is generally performed in many congenital cardiac anomalies where complete two ventricle circulations cannot be easily achieved. The advantages of BDG shunt are achieved by partially separating the pulmonary and systemic venous circuits, and include reduced ventricular preload and long-term preservation of myocardium. The benefits of additional pulsatile pulmonary blood flow include the potential growth of pulmonary arteries, possible improvement in arterial oxygen saturation, and possible prevention of development of pulmonary arteriovenous malformations. However, increase in the systemic venous pressure after BDG with additional pulsatile blood flow is known. We describe the peri-operative implications of severe flow reversal in the superior vena cava after pulsatile BDG shunt construction in a child who presented for surgical interruption of the main pulmonary artery.

Lungs: victim of synchronous double malignancies.

A 20 year young man was referred to our institution with superior vena cava (SVC) syndrome, multiple lung opacities and a mass lesion in the right upper zone (RUZ). CT-guided FNAC from the mass lesion was consistent with the diagnosis of non-small cell lung carcinoma (NSCLC). A lump in his left testis was detected during clinical examination. Both FNAC and excisional biopsy of the testicular mass confirmed the diagnosis of immature teratoma with choriocarcinoma, a form of non-seminomatous germ cell tumour (NSGCT). With chemotherapy all metastatic lesions of lung and SVC syndrome disappeared, and the tumour-marker levels decreased. However, the opacity in RUZ progessed to involve right recurrent laryngeal nerve at thoracic inlet, metastasized to the brain, and the patient expired after 4th cycle of chemotherapy. This case of synchronous double primary malignancies (SDPM) is being reported for its rarity.

Implante de endoprótese na via de entrada do átrio direito monitorado por ultra-som intracardíaco / Endoprosthesis implantation at the entry pathway of the right atrium monitored by intracardiac ultrasound

As obstruções do sistema venoso superior podem ocorrer por trombose espontânea ou na presença de cateteres de hemodiálise ou quimioterapia, e mesmo na presença de compressão extrínseca por tumores intratorácicos. O uso de endopróteses para o tratamento dessas obstruções vem se tornando prática comum e sua indicação inclui situações de risco de embolia pulmonar, síndrome da veia cava superior e perda do acesso vascular. Nesse contexto, o uso do ultra-som intracardíaco pode ser útil na avaliação da extensão e da gravidade da obstrução, além de fornecer informações morfológicas murais e intramurais do sistema venoso. Relatamos o caso de uma paciente com síndrome da veia cava superior por trombose da via de entrada do átrio direito, submetida a implante de endoprótese monitorado por ultra-som intracardíaco, além de discutir o tema por meio de revisão da literatura.

Obstructions of the upper venous system may be due to spontaneous thrombosis or the presence of hemodialysis or chemotherapy catheters, or even the presence of extrinsic compression due to intra-thoracic tumors. The use of endoprosthesis for the treatment of these obstructions has become common practice, and its indications include situations of risk for pulmonary embolism, superior vena cava syndrome, and loss of vascular access. Within this context, the use of intracardiac ultrasound may be useful in evaluating the extension and severity of the obstructive lesion, besides providing important information about the mural and intraluminal morphology of the venous system. The authors report the case of a patient with superior vena cava syndrome caused by thrombosis at the entry pathway of the right atrium, besides discussing the subject by means of literature review.

Superior vena cava syndrome caused by pulmonary amoebic abscess.

Isolated pulmonary amoebiasis without involvement of liver and other systems is extremely rare. Its presentation with superior vena cava (SVC) syndrome is not well documented. The case of 38-year-old male who developed SVC syndrome due to a large pulmonary amoebic abscess, which initially mimicked a pulmonary neoplasm with distal lung abscess is presented here. Subsequent bacteriological examination of the aspirated pus and the sputum along with suggestive serology confirmed the diagnosis of pulmonary amoebic abscess.

Marcapasso bi-atrial epicárdico subxifóide na obstrução da veia cava superior / Bi-atrial subxiphoid epicardial pacemaker in superior vena cava syndrome / Bi-atrial subxiphoid epicardial pacemaker in superior vena cava syndrome

Um paciente portador de marcapasso definitivo bi-atrial-ventricular por fibrilação atrial paroxística e bradicardia sinusal, em uso crônico de anticoagulante oral, apresentou sinais clínicos da síndrome da veia cava superior. A venografia por subtração digital mostrou obstrução total do tronco braquiocefálico venoso direito e grande dificuldade de fluxo sangüíneo da veia inominada para a veia cava superior. A abordagem terapêutica constou da remoção completa do sistema transvenoso seguida de reimplante do sistema bi-atrial-ventricular por técnica epimiocárdica pela via subxifóide assistida por fluoroscopia.

A patient with a bi-atrial-ventricular permanent pacemaker due to paroxystic atrial fibrillation associated to sinus bradycardia, in chronic use of oral anticoagulant, presented clinical signs of superior vena cava syndrome. Digital subtraction venography showed total obstruction of the right brachiocephalic venous trunk and severe stenosis of the connection of the left trunk to the superior vena cava. The therapeutic approach consisted of complete removal of transvenous system followed by re-implant of the bi-atrial-ventricular system using an epicardial subxiphoid access with fluoroscopic assistance