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)

[A Case of Superior Vena Cava Syndrome Due to Recurrent Breast Cancer Effectively Treated by Trastuzumab Deruxtecan].

A 64-year-old female underwent Bt+Ax surgery due to ER(-), PR(-), HER2(+), ycT3N1M0, Stage ⅢA right breast cancer. After cancer recured in the chest wall, whole-breast radiation therapy was performed, followed by ddAC, and T- DM1. After 12 courses of T-DM1, CT scan and physical findings showed no evidence of metastases, so chemotherapy was suspended with strict follow-up. Seven months later, a chest wall recurrence with pleural dissemination was found and 9 courses of PER plus HER plus eribulin therapy was administered until disease progression. T-DM1 was re-administered but disease progressed after 2 courses accompanied by SVC syndrome due to 8 cm mediastinal lymph node metastasis which caused respiratory discomfort and face edema. We administered T-DXd and after the first course respiratory symptoms vanished, and after 3 courses lymph node metastasis shrunk extremely in the CT imaging. SVCS is one of the oncologic emergencies, in which palliative radiotherapy may be typically selected for the relief of symptoms, and intravascular stents are used in urgent cases. Surprisingly, we experienced a case of SVC syndrome caused by breast cancer metastasis, effectively treated by T-DXd.

Superior vena caval obstruction: a rare presentation of Behcet's disease.

A 25-year-old Indian man presented with low-grade fever followed by gradually increasing swelling of neck and face. Physical examination showed bilateral neck swelling, facial swelling and dilated veins in the upper chest. Superior vena cava (SVC) obstruction due to an underlying malignancy was suspected. CT thorax showed large saccular aneurysm with thrombosis of bilateral subclavian arteries of which the right one caused external compression of right innominate vein draining into the SVC. A history of recurrent oral and scrotal ulcers was obtained following which skin pathergy test was done, which was suggestive of a diagnosis of Behcet's disease (BD). He responded to treatment with steroids and azathioprine. This report illustrates that rare nonmalignant cause such as BD could also present with SVC obstruction.

Thrombolytic therapy in preterm infants: Fifteen-year experience.

OBJECTIVE: To report a single-center experience with thrombolytic therapy using recombinant tissue plasminogen activator (rt-PA) in preterm neonates with severe thrombotic events, in terms of thrombus resolution and bleeding complications. STUDY DESIGN: This retrospective study included 21 preterm neonates with severe venous thrombotic events admitted to the neonatal intensive care unit, identified in our pharmacy database from January 2001 to December 2016, and treated with rt-PA until complete or partial clot lysis, no-response or bleeding complications. Our primary outcome was thrombus resolution. RESULTS: Twenty-one preterm neonates were treated with rt-PA for an average of 2.9 cycles. Seventeen patients (80.9%) had superior vena cava thrombosis and superior vena cava syndrome. All patients had a central venous catheter, parenteral nutrition, mechanical ventilation, and sepsis. Fifteen patients (71.4%) were extremely preterm, 11 (52.4%) were extremely low birth weight, and seven (33.3%) were very low birth weight. The patency rate was 85.7%, complete lysis occurred in 11 (52.4%) patients, and partial lysis in seven (33.3%). Minor bleeding occurred in five (23.8%) patients, three patients (14.2%) had clinically relevant nonmajor bleeding events, and major bleeding occurred in six (28%) patients. CONCLUSION: In this study, the rate of thrombus resolution in preterm neonates treated with rt-PA were similar to the percentages reported in children and adolescents, with a high rate of bleeding. Therefore, rt-PA thrombolytic therapy should only be considered as a treatment option for severe life-threatening thrombosis in premature neonates for whom the benefits of the thrombolytic treatment outweigh the risks of bleeding.

Thrombotic risk factors in patients with superior vena cava syndrome undergoing chemotherapy via femoral inserted central catheter.

OBJECTIVE: Our study aimed to scrutinize the incidence and risk factors of femoral inserted central catheter (FICC)-related thrombosis in patients with superior vena cava syndrome (SVCS) undergoing chemotherapy. METHODS: A retrospective analysis of patients with SVCS undergoing chemotherapy who received FICC catheterization at the Xiangya Hospital, Central South University, Changsha City, Hunan Province between May 2012 and February 2019 was performed. Both asymptomatic thrombosis and symptomatic thrombosis were diagnosed by color doppler ultrasound (CDUS). Univariate and multivariate logistic regression analyses were performed to identify patient-, insertion-, and catheter-related factors. RESULTS: Eight hundred and seventy-four patients with SVCS undergoing chemotherapy, with a total of 157,180 catheter days were enrolled in our study. FICC-related thrombosis was detected in 144 patients, and yielding an overall incidence of 16.47% or 0.92 events per 1000 catheter days. Of these, 19(2.17%) patients had symptomatic thrombosis. The mean time interval between FICC insertion and thrombosis onset was (10.40 ±â€¯6.32) days and the mean catheter indwelling time was (179.84 ±â€¯46.15) days. The history of deep venous thrombosis, treatment with vascular endothelial growth factor (VEGF) inhibitor (bevacizumab), puncture site (mid-thigh, groin), tip position and catheter size showed association with FICC-related thrombosis. Treatment with VEGF inhibitor [odds ratio (OR) = 2.779; 95%confidence interval (CI): 1.860-4.153; P < 0.001] and puncture site at the groin (OR = 10.843; 95%CI: 6.575-17.881; P < 0.001) were identified as independent risk factors of FICC-related thrombosis. CONCLUSION: Treatment with VEGF inhibitor and puncture site at the groin during FICC catheterization were considered as high-risk factors in FICC-related thrombosis.

Use of rivaroxaban for treatment of cranial vena cava syndrome secondary to transvenous pacemaker lead thrombosis in a dog.

A six-year-old Brussels griffon was presented for cervical swelling three months after implantation of a transvenous pacemaker. Transthoracic echocardiography demonstrated a thrombus associated with the pacemaker lead, partially obstructing right atrial inflow. The laboratory findings were consistent with protein-losing nephropathy. Initial medical therapy consisted of rivaroxaban (0.68 mg/kg orally every 24 hours), clopidogrel (2.5 mg/kg orally every 24 hours), and enalapril (0.5 mg/kg orally every 12 hours). Resolution of cervical and thoracic edema was noted within two weeks of initiating therapy. Recheck echocardiography two months and one year later revealed decreasing thrombus size despite worsening proteinuria. To the authors' knowledge, this is the first documented use of rivaroxaban for successful medical treatment of cranial vena cava syndrome caused by intracardiac pacemaker lead thrombosis in a hypercoagulable patient.

Thrombosis, anticoagulation and outcomes in malignant superior vena cava syndrome.

Anticoagulation is often used in superior vena cava syndrome (SVCS) associated with cancer (i.e malignant SVCS), even without thrombosis, but its effect on outcomes has not been reported. We aimed to determine factors and outcomes associated with thrombosis and anticoagulation in malignant SVCS. Patients with malignant SVCS diagnosed on computerized tomography (CT) were retrospectively included, indexed at diagnosis and followed for 6 months using medical records. The cohort included 183 patients with malignant SVCS of which 153 (84%) were symptomatic. Thirty of the 127 patients (24%) with a reviewable baseline CT had thrombosis of the SVC or tributaries at diagnosis. Patients with baseline thrombosis more often had symptomatic SVCS (p < 0.01). 70% (21/30) of patients with thrombosis and 52% (49/97) of those without thrombosis at baseline received anticoagulation, most often at therapeutic doses. Thrombosis occurred in 5/39 patients with anticoagulation (13%) compared to 2/18 (11%) of those without, during follow-up (p = 0.85). Anticoagulation was associated with a reduction in risk of SVC stent placement during follow-up that did not reach statistical significance (HR 0.47, 95% CI 0.2-1.13, p = 0.09). Major bleeding occurred in 7 (4%) patients, six of whom received anticoagulation (four therapeutic and two intermediate dose). Neither thrombosis nor anticoagulation affected survival. Anticoagulation is commonly used as primary prevention but its benefit remains to be proven. The role of reduced-dose anticoagulation in non-thrombotic malignant SVCS should be prospectively assessed.

Behçet's disease; A rare refractory patient with vena cava superior syndrome treated with infliximab: a case report and review of the literature.

Objectives: Behçet's disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of vessels.The usage of TNF-α antagonists is increasing in different involvements of BD that is inadequately controlled by standard immunosuppressive regimens. Methods: Here we reported a rare BD case that is controlled by only infliximab (IFX) treatment. Results: A 24-years-old male patient was diagnosed as BD with recurrent oral and genital ulcerations,uveitis,femoral vein thrombosis and HLA-B5 positivity.He had had terminal ileitis,epididymitis,bilateral internal iliac artery aneurysm and superior sagittal sinus thrombosis after the diagnosis.He admitted to our outpatient clinic with dyspnea,orthopnea,chest pain and facial swelling.We detected facial edema,plethora,venous distention on neck and chest.We suspected vena cava superior syndrome(VCSS) and performed CT angiography that demonstrated high-grade vena cava superior stenosis and thrombus where SVC opens to the right atrium.Patient was anticoagulated with warfarin and a shunt was implanted between the left brachiocephalic vein and right atrium.Colchicine and interferon-alfa treatment were continued and 1mg/kg steroid was added.But the patients' symptoms relapsed one month later and his shunt was revised with balloon angioplasty.5mg/kg IFX was started.We  performed a CT-angiography annually for two years.VCSS and other symptoms of BD are resolved and have not relapsed yet. Conclusions: Vascular involvement of BD are generally treated with immunosuppressive agents such as steroids and immunomodulators.IFX was found effective and well tolerated in the treatment of intestinal,neurological and vascular involvement of BD especially in patients with poor response or intolerance to conventional therapy.This case report supports IFX as a new therapeutic option for patients with vascular BD.

Superior vena cava syndrome due to central port catheter thrombosis: a real life-threatening condition.

Superior vena cava syndrome (SVCS) represents undoubtedly a rare life-threatening condition. Herein, we present a rare case of a 69-year-old woman, with a history of hepatic flexure tumor and an indwelling central venous port, presenting with acute signs and symptoms of SVCS due to thrombosis of the catheter. The patient was treated with intravenous anticoagulation and fibrinolytic therapy and showed regression of symptoms. It is reported that central venous catheters are routinely used in clinical practice mainly in oncological cases for chemotherapy, parenteral nutrition or dialysis. However, complications related to implantation technique, care, or maintenance of these catheters may arise. High index of suspicion for SVCS should always arise when a patient presents with common symptoms and long-term central catheters, in order to avoid unfavorable outcomes. Local thrombolysis appears to be a safe and effective therapy for port catheter-associated thrombosis.

Thrombosis of the superior vena cava due to a Hickman catheter: successful management by rt-PA thrombolysis.

INTRODUCTION: Venous thrombosis is a frequent complication of long-term venous access. Its management is well defined in most cases, but some specific clinical situations have not yet been definitively standardized.Thrombosis may occur at the catheter tip and involve the superior vena cava (SVC) and/or the right atrium (RA). In such cases, while the need for a rapid intervention to relieve the venous obstruction and avoid embolism is obvious, the best management is still to be established. CASE DESCRIPTION: We report the case of a 25-year-old woman with symptomatic thrombosis of SVC and RA, associated with a Hickman catheter, which was successfully treated by recombinant tissue plasminogen activator (rt-PA). CONCLUSIONS: According to the literature, thrombolytic therapy with rt-PA may be considered the treatment of choice in symptomatic thrombosis of SVC secondary to long-term catheter, uncertainty still exists about dosage, optimal rate of infusion, and optimal duration of treatment, as well as the criteria for choosing local versus systemic infusion.

Thrombolytics for late superior caval vein thrombus in a patient with tricuspid atresia and single-lung Glenn anastomosis.

BACKGROUND: Those with cyanotic heart disease have an elevated bleeding risk but also are hypercoaguable. Treating haemodynamically significant thrombi in this unique cohort poses a monumental challenge. Case A 29-year-old women with tricuspid atresia and left pulmonary artery atresia presented with superior caval vein syndrome. She had a right modified Blalock-Taussig shunt as a neonate. A left modified Blalock-Taussig shunt performed later failed to establish flow to her left lung. At age 5, she had a Fontan procedure to the right lung but could not tolerate the physiology and had a low cardiac output syndrome. The Fontan was taken down and she was left with a Glenn anastamosis to the right pulmonary artery. She did well for years until she had dyspnea, upper extremity oedema and "facial fullness". On examination she was tachycardic, hypotensive, and more desaturated than baseline. She also had facial plethora. Decision-making Echocardiogram showed a large 9 × 3 mm nearly occlusive thrombus in the superior caval vein at the bifurcation of the left and right innominate veins. An emergent venogram confirmed the location and size of the thrombus. Given the thrombus burden and potential for distal embolisation through the Glenn to the single functional lung, we chose to treat the patient with thrombolytics. She had uncomplicated ICU course and was sent home on warfarin. Follow-up echocardiogram showed complete resolution of clot. CONCLUSION: This case shows the importance of history and physical exam in caring for this complex cohort of adult patients with CHD.

"The Scalpel or the Needle for Superior Vena Cava syndrome"?

Acute Superior Vena Cava (SVC) syndrome from thrombosis is an increasingly recognized complication of intravascular devices. We present a 31 year old woman with an infusion port placed for chemotherapy who developed acute SVC obstruction. A computerized tomograpy (CT) of chest revealed an occlusive thrombus within the SVC extending into the right atrium. Catheter-guided thrombolysis and surgical thrombectomywere felt to impose prohibitive risks. Worsening symptoms led to the use of systemic thrombolysis with tissue plasminogen activator (t-PA) leading to dramatic improvement in symptoms. A repeat CT revealed a reduction of the right atrial thrombus and SVC occlusion had resolved.