A Systematic Review and Meta-analysis of 24 Month Patency After Endovenous Stenting of Superior Vena Cava, Subclavian, and Brachiocephalic Vein Stenosis.

OBJECTIVE: This systematic review and meta-analysis aimed to appraise recent evidence assessing patency outcomes at various time points in patients with superior vena cava, subclavian, and brachiocephalic vein stenosis who had undergone stenting. DATA SOURCES: PubMed, Scopus, and Cochrane Library databases were searched for studies up to December 2022. REVIEW METHODS: Measured outcomes included technical success rate, primary, primary assisted, and secondary patency at various time points. A subgroup analysis was also conducted to compare malignant and benign obstruction. GRADE was used to assess the certainty of evidence. RESULTS: Thirty nine studies reporting outcomes in 1 539 patients were included in the meta-analysis. Primary patency up to one year after the procedure was 81.5% (95% CI 74.5 - 86.9%). Primary patency declined after one year to 63.2% (95% CI 51.9 - 73.1%) at 12 - 24 months. Primary assisted patency and secondary patency at ≥ 24 months were 72.7% (95% CI 49.1 - 88.0%) and 76.6% (95% CI 51.1 - 91.1%). In the subgroup analysis, primary patency was significantly higher in patients with a malignant stenosis compared with a benign stenosis at 1 - 3 and 12 - 24 months. No significant difference was seen for pooled secondary patency rates when comparing the malignant and benign subgroups. GRADE analysis determined the certainty of evidence for all outcomes to be very low. CONCLUSION: Stenting is an effective intervention for benign and malignant stenosis of the superior vena cava, subclavian, and brachiocephalic veins. Primary patency rates were good up to one year after the procedure, with 81.5% of stents retaining patency at 6 - 12 months. Patency rates declined after one year, to 63.2% primary and 89.3% secondary patency at 12 - 24 months, showing improved outcomes following re-intervention. High quality evidence is lacking. More research is needed to investigate patency outcomes and the need for surveillance or re-intervention programs.

Emergency radiation therapy in modern-day practice: Now or never, or never again ?

Radiation therapy plays a fundamental role in oncological emergencies such as superior vena cava syndrome (SVCS) and metastatic epidural spinal cord compression (MESCC). These are two examples of critical complications of metastatic cancer in terms of pain and functional impact (respiratory and/or neurological). The aim of this review is to explore the current indications, treatment options and outcomes for emergency radiotherapy regarding to these complications.Regarding SVCS, studies are mostly retrospective and unanimously demonstrated a beneficial effect of radiotherapy on symptom relief. Spinal cord compression remains an indication for urgent radiotherapy, and should be combined with surgery when possible. The innovative stereotactic body radiotherapy (SBRT) showed promising results, however this technique requires small volumes and more time preparation and therefore is often unsuitable for SVCS and MESCC emergencies.This review concluded that radiotherapy has a central role to play within a multimodal approach for SVCS and MESCC treatment. Further prospective studies are needed to confirm the effectiveness of radiation and establish the criteria for selecting patients to benefit from this treatment option.

If not angioedema, what is it? Diagnostic approach to facial edema.

Facial edema is a relatively frequent clinical presentation encountered in patients seen in allergology and dermatology clinics. The differential diagnosis is broad, and sometimes the definitive diagnosis can be a challenge for the clinician. Facial angioedema itself encompasses different etiopathologies (histaminergic, bradykinergic, etc.) that must be distinguished from other causes of facial edema, such as allergic contact dermatitis, granulomatous conditions, inflammatory causes, infections, neoplasms or paraneoplastic syndromes, autoimmune diseases, among other entities hereby referred as miscellanea. A proper diagnostic approach is essential to order the appropriate tests, as well as to prescribe a targeted treatment. This review focuses on entities that present with facial edema and summarize their characteristic clinical features.

End-stage renal disease in a critical patient with superior vena cava syndrome with central vein catheters inserted via the superficial femoral vein: A case study.

Superior vena cava syndrome (SVCS) is caused by obstruction to the blood flow through this vein. Indwelling central venous devices, such as cardiac pacemakers and haemodialysis catheters have emerged as the most common benign aetiology of SVCS. SVCS is particularly severe in patients with end-stage renal disease who require continuous renal replacement therapy plus infusion therapy. The presence of SVCS results in a reduction of available venous access for affected patients. Therefore, venous access plays a crucial role in the management of these patients. The importance of dealing with vascular access (VA) in critical patients with these conditions cannot be overstated. This case describes an 81-year-old man with respiratory failure who had end-stage renal disease complicated with SVCS. Using ultrasound-guided puncture, we inserted a peripherally inserted central catheter (PICC) into the superficial femoral vein to meet his infusion requirements in intensive care. After successful placement, the catheter tip position was adjusted using imaging to position the tip relative to the haemodialysis catheter. Whenever patients with severe renal dysfunction are treated, central veins should be preserved. Safe PICC access is possible via the superficial femoral vein to protect the last central VA for rational use. This meets urgent needs for infusion and deserves promotion.

Treatment of pacemaker-induced superior vena cava syndrome by direct oral anticoagulant.

BACKGROUND: The use of cardiac implantable electronic devices has grown substantially over the past two decades, lead-related vascular issues are commonly encountered in clinical practice. Superior vena cava (SVC) syndrome due to pacemaker leads is an uncommon complication. Anticoagulation remains the mainstay of therapy to restore some degree of patency and relieve swelling. However, there are limited clinical trials on direct oral anticoagulants (DOACs). CASE PRESENTATION: We report a case of an 80-year-old man who developed SVC syndrome after transvenous pacemaker implantation with symptoms of obstruction that were significantly relieved after four months of DOACs. His symptoms had completely resolved nine months later. CONCLUSIONS: DOACs are effective in the treatment of SVC syndrome after pacemaker implantation, representing an important new approach. It is a very good choice for patients who do not want to undergo interventional therapy.

CIC-rearranged sarcoma presenting with superior vena cava syndrome: case report.

CIC-rearranged sarcomas are rare mesenchymal neoplasms belonging to the family of undifferentiated small round cell sarcomas. This report details the case of a 45-year-old man presenting with symptoms of mediastinal compression, radiological diagnosis of a mediastinal mass and rapid evolution to full-blown superior vena cava syndrome. The emergency was successfully managed with a pharmacological approach. Formulation of a pathological diagnosis of CIC-rearranged sarcoma was initially supported by fluorescence in situ hybridisation findings and later validated by next-generation sequencing, which showed CIC-DUX4 gene fusion. A chemotherapy regimen was started with immediate benefits for the patient. The spectrum of pathological entities able to cause superior vena cava syndrome is wide, and recognition of rare causes is important to tailor the therapeutic approach to the specific disease. This is, to the best of our knowledge, the first report of CIC-rearranged sarcoma presenting with superior vena cava syndrome.

Symptom relief, prognostic factors, and outcome in patients receiving urgent radiation therapy for superior vena cava syndrome : A single-center retrospective analysis of 21 years' practice.

PURPOSE: Superior vena cava syndrome (SVCS) often results from external vessel compression due to tumor growth. Urgent symptom-guided radiotherapy (RT) remains a major treatment approach in histologically proven, rapidly progressive disease. Despite several publications, recent data concerning symptom relief and oncological outcome as well as potential confounders in treatment response are still scarce. METHODS: We performed a retrospective single-center analysis of patients receiving urgent RT between 2000 and 2021 at the University Medical Center Göttingen. Symptom relief was evaluated by CTCAE score during the RT course. Effects of variables on symptom relief were assessed by logistic regression. The impact of parameters on overall survival (OS) was evaluated using Kaplan-Meier plot along with the log-rank test and by Cox regression analyses. Statistically significant (p-value < 0.05) confounders were tested in multivariable analyses. RESULTS: A total of 79 patients were included. Symptom relief was achieved in 68.4%. Mean OS was 59 days, 7.6% (n = 6) of patients showed long-term survival (> 2 years). Applied RT dose > 39 Gy, clinical target volume (CTV) size < 387 ml, concomitant chemotherapy, and completion of the prescribed RT course were found to be statistically significant for OS; applied RT dose and completion of the prescribed RT course were found to be statistically significant for symptom relief. CONCLUSION: Symptom relief by urgent RT for SVCS was achieved in the majority of patients. RT dose and completion of the RT course were documented as predictors for OS and symptom relief, CTV < 387 ml and concomitant chemotherapy were predictive for OS.

Leadless pacemakers as a new alternative for pacemaker lead-related superior vena cava syndrome: A case report.

Superior vena cava (SVC) syndrome is a rare disease induced by thrombosis and consequent occlusion of SVC, negatively affecting morbidity and mortality. The incidence of SVC syndrome from central venous catheters and pacemaker or defibrillator leads is increasing. Optimal treatment of pacemaker or defibrillator-related SVC syndrome is not well defined. Lead extraction causes mechanical trauma to the vessel wall. In addition, subsequent device implantation on the contralateral side can be an added factor for venous occlusion. The use of leadless pacemakers could be an interesting option to reduce the risk of SVC restenosis after lead extraction. We report a clinical case of PM leads-related SVC syndrome referred to our centers and treated with transvenous lead extraction, leadless pacemaker implantation and subsequent percutaneous angioplasty and stenting of the SVC and left innominate vein.

Thrombotic superior vena cava syndrome: a national emergency department database study.

Literature regarding etiology and trends of incidence of major thoracic vein thrombosis in the United States is limited. To study the causes, complications, in-hospital mortality rate, and trend in the incidence of major thoracic vein thrombosis which could have led to superior vena cava syndrome (SVCS) between 2010 and 2018. Data from the nationwide emergency department sample (NEDS) that constitutes 20% sample of hospital-owned emergency departments (ED) and in-patient sample in the United States were analyzed using diagnostic codes. A linear p-trend was used to assess the trends. Of the total 1082 million ED visits, 37,807 (3.5/100,000) (mean age 53.81 ± 18.07 years, 55% females) patients were recorded with major thoracic vein thrombosis in the ED encounters. Among these patients, 4070 (10.6%) patients had one or more cancers associated with thrombosis. Pacemaker/defibrillator-related thrombosis was recorded in 2820 (7.5%) patients, while intravascular catheter-induced thrombosis was recorded in 1755 (4.55%) patients. Half of the patients had associated complication of pulmonary embolism. A total of 59 (0.15%) patients died during these hospital encounters. The yearly trend for the thrombosis for every 100,000 ED encounters in the United States increased from 2.17/100,000 in 2010 to 5.98/100,000 in 2018 (liner p-trend < 0.001). Yearly trend for catheter/lead associated thrombosis was also up-trending (p-trend 0.015). SVCS is an uncommon medical emergency related to malignancy and indwelling venous devices. The increasing trend in SVCS incidence, predominantly catheter/lead induced, and the high rate of associated pulmonary embolism should prompt physicians to remain vigilant for appropriate evaluation.

Surgical treatment for Behcet's disease with acute superior vena cava syndrome.

BACKGROUND AND AIM OF THE STUDY: Behcet's disease (BD) is a multisystem vasculitis with unknown etiology. The involvement of superior vena cava (SVC) is reported in less than 2% of patients with BD. METHODS: We report a patient with acute edema of neck and face associated with dyspnea as the primary manifestation. So a diagnosis of superior Vena Cava syndrome (SVCS) was made and the thickening wall of SVC was resected. An Operation was performed under cardiopulmonary bypass to remove the mass and thrombus for avoiding for pulmonary embolism. RESULTS: The diagnosis of Behcet's disease (BD) didn't not be made until the recurrent oral and genital ulceration occurred 2 weeks later. The patient taked aspirin and prednisolone orally as prescribed and no recurrence were observed during the 30 months follow-up. CONCLUSIONS: BD should be suspected in patients presenting with SVCS, when there is thickening of SVC, whether thrombosis or not. Early diagnosis and treatment are essential for management of BD.

Development and validation of a nomogram for assessing survival in extensive-stage small-cell lung cancer patients with superior vena cava syndrome referred for thoracic radiotherapy: a comparison of upfront vs. consolidative approaches.

PURPOSE: This study sought to design and validate a nomogram capable of predicting outcomes in extensive-stage small-cell lung cancer (ES-SCLC) patients with superior vena cava syndrome (SVCS) based upon the timing of their radiotherapy treatment. METHODS: We retrospectively analyzed data from 175 ES-SCLC patients with SCVS, comparing outcomes between those that underwent upfront thoracic radiotherapy (initial radiotherapy with simultaneous chemotherapy) and those that underwent consolidative thoracic radiotherapy (following 4-6 cycles of chemotherapy). Significant predictors of patient outcomes were identified using a Cox proportional hazard model and were used to construct our nomogram. This model was subsequently validated using receiver operating characteristic (ROC) curves, concordance index (C-index) values, and a risk classification system in order to evaluate its discriminative and predictive accuracy. RESULTS: The overall survival (OS) of ES-SCLC patients with SVCS that underwent chemotherapy (CT), consolidative thoracic radiotherapy (cc-TRT), and upfront thoracic radiotherapy (cu-TRT) was 8.2, 11.7, and 14.9 months, respectively (p < 0.001), with respective progression-free survival (PFS) durations of 3.3, 5.0, and 7.3 months (p < 0.001). A multivariate regression analysis revealed age, gender, ECOG performance status, sites of tumor metastasis, and treatment approach to all be independent predictors of survival outcomes. A nomogram was therefore developed incorporating these factors. C­index values upon internal and external validation of this nomogram were 0.7625 and 0.7959, respectively, and ROC and calibration curves revealed this model to be accurate and consistent. CONCLUSIONS: We found that upfront thoracic radiotherapy in combination with chemotherapy may be associated with a positive impact on outcomes in ES-SCLC patients with SVCS.

Long Term Results of Bypass Graft to the Right Atrium in the Management of Superior Vena Cava Syndrome in Dialysis Patients.

BACKGROUND: Superior vena cava (SVC) occlusion in dialysis patients is a serious complication that can cause SVC syndrome and vascular access dysfunction. While endovascular therapy has advanced to become the first line of treatment, open surgical treatment may still be needed occasionally. However, no long term outcome data has been previously reported. METHODS: We performed a retrospective review of 5 dialysis patients treated with bypass graft to the right atrium from 2012 to 2014. Four patients had severe dysfunction of their upper arm dialysis access as well as superior vena cava syndrome, and one patient with a femoral tunneled dialysis catheter (TDC) had SVC occlusion. None of the patients were candidates for lower extremity access creation or peritoneal dialysis (PD). Three patients underwent a left brachiocephalic-right atrial bypass and 2 underwent a bypass from the cephalic fistula to the right atrium. RESULTS: All procedures were technically successful and maintained function of the arteriovenous fistulas or allowed creation of a new upper extremity dialysis graft. One-year secondary patency rate of the bypass was 100%. Longer follow up revealed that one patient died of leg sepsis and another one of a stroke within 14 months after the procedure. Another patient did well for 16 months when recurrent graft thrombosis occurred; and ultimately the graft failed after 31 months despite multiple interventions. Two patients maintained bypass graft patency during a follow up of 78 months; however, they underwent multiple endovascular interventions (23) and open vascular access procedures (4) to maintain hemodialysis function. CONCLUSION: Bypass grafts to the right atrium in dialysis patients with SVC occlusion are successful in maintaining function of already existing vascular access or new ones. Long term secondary patency can be achieved but requires strict follow up and a proactive endovascular strategy to treat lesions in the access and or the bypass graft.

Shock due to superior vena cava obstruction detected with point of care ultrasound.

Superior Vena Cava (SVC) syndrome is caused by SVC obstruction by external compression or intraluminal thrombus. Patients with the condition can present with upper body swelling, shortness of breath and shock. This case report highlights the use of point-of-care ultrasound (POCUS) to evaluate a patient with SVC syndrome in the emergency department. The test offers many advantages over computed tomography (CT), venography, and magnetic resonance imaging which are limited in hemodynamically unstable patients. A 60-year-old male presented with acute respiratory distress and shock. The POCUS showed the presence of a right lung consolidation and SVC thrombus. CT revealed the presence of a large mediastinal mass causing compression of the SVC with clot seen inside the vessel. The patient was thrombolysed with intravenous streptokinase and his hemodynamics improved. Further investigation confirmed the diagnosis of lymphoma. The SVC can be visualized with transthoracic echocardiography using either the suprasternal, right supraclavicular or right parasternal approach. In this case, the presence of consolidation of the right lung mass provided an acoustic window for the visualization of the SVC using the right parasternal view, thereby allowing for more rapid diagnosis and management.

Clinical analysis of 48 cases of malignant superior vena cava syndrome.

BACKGROUND: The aim of our study was to observe and compare the curative effect of radiotherapy, chemotherapy, and combined radiotherapy and chemotherapy, as well as comprehensive treatment on superior vena cava syndrome (SVCS) caused by malignant etiology. METHODS: A total of 48 patients with malignant SVCS admitted to our hospital from 2015 to 2020 were selected in this study. According to the different treatment methods, they were divided into radiotherapy group (group 1, 10 cases), chemotherapy group (group 2, 8 cases), combined radiotherapy and chemotherapy group (group 3, 22 cases), and comprehensive treatment group (group 4, 8 cases). RESULTS: There were no significant differences in efficacy and side effects among the four groups (all P > 0.05). Group 4 (median survival time of 36 months) could provide longer survival time than groups 1, 2, and 3 (median survival time of 10 months, 13.5 months, and 12 months, respectively). CONCLUSIONS: For patients with severe symptoms or good prognosis, comprehensive treatment could be selected to improve the quality of life and prolong the survival period; for patients with mild symptoms, radiotherapy, chemotherapy, or combined radiotherapy and chemotherapy could also reduce the symptoms of SVCS and treat tumor lesions.

Combined cut down and endovascular retrieval of orphaned ventriculoatrial shunt with stenting of chronic superior vena cava occlusion.

Revascularization of the superior vena cava (SVC) in the context of symptomatic luminal obstruction is a therapeutic intervention performed for SVC syndrome of benign or malignant etiology. Venous occlusion can preclude future access and cause symptoms ranging from mild chest discomfort to the more serious effects of SVC syndrome. This case report demonstrates the treatment of a novel case of SVC syndrome arising from a previously placed SVC stent. An intravascular, extraluminal orphaned ventriculoatrial shunt was used to go through the SVC but around the existing lumen-limiting stent to place a new larger stent for revascularization. This case highlights the need for an innovative approach for complex foreign body retrieval and treatment of chronic SVC occlusion.

Superior vena cava syndrome during veno-venous extracorporeal membrane oxygenation for COVID-19.

Superior vena cava (SVC) syndrome is typically associated with malignant tumors obstructing the SVC, but as many as 40% of cases have other etiologies. SVC obstruction was previously described during veno-venous extracorporeal membrane oxygenation therapy (VV ECMO) in children. In this report, we describe a woman with adult respiratory distress syndrome resulting from infection with coronavirus-19 who developed SVC syndrome during VV ECMO. A dual-lumen ECMO cannula was inserted in the right internal jugular vein, but insufficient ECMO circuit flow, upper body edema, and signs of hypovolemic shock were observed. This clinical picture resolved when the right internal jugular vein was decannulated in favor of bilateral femoral venous cannulae. Our report demonstrates that timely recognition of clinical signs and symptoms led to the appropriate diagnosis of an uncommon ECMO complication.

Outcome of stenting in central venous obstruction of benign etiology.

Background: The aim of this study is to evaluate long term outcome in patients treated for benign superior vena cava (SVC) syndrome by endovascular techniques. Patients and methods: Between 2015 and 2018, 62 patients suffering from central venous obstruction of benign etiology underwent balloon angioplasty with stent placement for venous obstruction in our department. Patency was assessed clinically, using duplex ultrasound in all patients or with CT-phlebography in selected cases. Results: Median age was 60 years (23-83), forty-one patients (66%) had central venous devices. Swelling of the arm and face were the main symptoms (71%). During the median follow up of 22 months (9-38), cumulative primary patency was 71% after venous stenting. The cumulative assisted primary and the secondary patency were 85% and 92%, respectively. Conclusions: Recanalization and stenting of central vein obstruction has turned out to be the technique of reference and provides satisfactory mid-term patency rates. After adjusting for the risk factors, presence of AV-fistula remained a significant risk factor for recurrent stenosis or loss of patency after intervention.

Hepatic "hot-spot" on angio-computed tomography: the role of inferior Sappey's and ensiform veins.

Hepatic "hot spots" in anterior paraumbilical hepatic segments of patients suffering from superior vena cava syndrome may be revealed by angio-computed tomography. They may be due to a collateralizing system, the epigastric-paraumbilical venous system (EPVS), which enters the liver as a "third inflow". We report a typical case emphasizing the role of the ensiform and inferior Sappey's veins which constitute typical anatomic components of the EPVS.

[Diffuse large B-cell lymphoma with mediastinal bulky mass associated with complication of repeated sinus arrest].

A 71-year-old woman presented to a clinic with the chief complaint of facial edema and dyspnea; chest radiography showed mediastinal mass shadow and right pleural effusion. Computed tomography guided biopsy of the mediastinal mass had been performed by her previous doctor, and she was diagnosed with diffuse large B-cell lymphoma. She was referred to our hospital for chemotherapy. Electrocardiography performed before initiating chemotherapy showed sinus arrest for about 4 s, and Holter electrocardiography showed sinus arrest for up to about 7.4 s, which was repeatedly observed 6 times, indicating sick sinus syndrome (SSS). The mediastinal mass completely excluded the superior vena cava, and considering the risk of infection, an extracorporeal pacemaker was not inserted. We believed that the tumor effect was the cause of sinus arrest; hence, chemotherapy initiation was prioritized. R-CHOP therapy preceding vincristine and prednisolone was started, and sinus arrest was not observed after initial treatment. SSS may have been caused by carotid hypersensitivity syndrome that involved the exclusion of carotid artery pressure receptors by the tumor or the direct stimulation of the vagus nerve by microtumor infiltration.

A review of endovascular stenting for superior vena cava syndrome in fibrosing mediastinitis.

Fibrosing mediastinitis (FM) is a rare disorder of inflammation and fibrosis involving the mediastinum. The formation of fibroinflammatory mass in the mediastinum can lead to obstruction of mediastinal structures and cause severe debilitating and life-threatening symptoms. Superior vena cava syndrome (SVCS) is a dreaded complication of FM with no medical therapy proven to be efficacious. Spiral vein grafting has long been utilized as first-line therapy for SVC syndrome due to FM. Endovascular repair with stents and angioplasty for malignant causes of SVC syndrome is well established. However, there are limited data on their utility in SVC syndrome due to FM. We present two cases of SVC syndrome due to FM treated with endovascular stenting and a detailed review of current literature on its utility in SVCS due to benign causes.