Superior vena cava syndrome (SVCS) is commonly caused by mediastinal malignancies. Early identification through clinical signs and imaging is critical to avoid complications including cerebral and laryngeal edema, and cardiogenic shock. We present a case of large cell neuroendocrine carcinoma causing superior and inferior vena cava compression that responded well to radiotherapy and chemotherapy.

Malignant cardiac tumor is a rare tumor with extremely poor prognosis, and metastatic cardiac tumor causes superior vena cava( SVC) syndrome. A 52-year-old man visited a clinic with a chief complaint of facial edema. Contrast-enhanced computed tomography( CT) revealed a mass in the right atrium( RA)obstructing the SVC. Echocardiography revealed a mass about to incarcerate the tricuspid valve orifice. The patient was transferred to our institution for emergency surgery. Tumor resection was performed under general anesthesia. A cardiopulmonary bypass was established with cannulate in the ascending aorta, in the RA through the right femoral vein, and in the left ventricle for venting. The RA was incised, and the tumor was resected. The SVC was incised, and the tumor and blood clots were removed. Because adhesion between vessel wall and the mass was tight, complete mass removal and recanalization of the SVC was not attempted. Pathological diagnosis was metastatic squamous cell carcinoma. All imaging studies failed to identify primary lesions. The clinical course was uneventful, and the patient was discharged on postoperative day 17. Four months postoperatively, chemotherapy for squamous cell carcinoma was initiated. The patient is alive at approximately 28 months postoperatively.

Obstruction of the superior vena cava (SVC) is a rare complication after cardiac surgery in infants and children. We present the case of an 8-year-old boy who underwent bi-directional Glenn shunt followed by takedown of Glenn shunt and complete repair for cyanotic congenital heart disease. After 4 years of surgery, the child developed features of superior vena caval (SVC) syndrome. Echocardiography and CT angiography revealed complete obstruction of SVC without any forward flow. Transcatheter intervention was performed successfully to re-canalize and stent the SVC to maintain its patency. The patient was doing well at follow-up appointments, with good laminar flow through the stent. In conclusion, transcatheter management of post cardiac surgery SVC obstruction was successful in this patient.

OBJECTIVE: To retrospectively analyze the technical and long-term clinical outcome of angioplasty and stenting using the Venovo™ venous stent for the treatment of malignant and benign superior vena cava (SVC) occlusive disease.
METHODS: Consecutive patients treated with the Venovo™ venous stent for SVC occlusive disease were included. SVC obstruction symptoms were classified according to the Kishi score. The Wilcoxon signed-rank test was used for testing significance of changes. Technical success, defined as correct placement of the stent, completely covering and re-expanding the obstruction, between groups was tested using the Fisher exact test. Overall survival was calculated using the Kaplan-Meier method.
RESULTS: Fifty-five patients underwent stent insertion for symptomatic benign (n = 13; 24%) or malignant (n = 42; 76%) SVC occlusive disease. A significant drop in Kishi score, mean 3.91 before versus mean 1.02 after the procedure (P < 0.0001), was observed. In one patient (1.8%), an additional balloon-expandable stent was needed to manage incomplete expansion of the nitinol stent. In one patient, a procedure-related lung embolic complication was noted. Early thrombotic occlusion of the stent occurred in one patient. Late symptomatic restenosis occurred in 3 patients. Overall primary stent patency and primary-assisted stent patency were 86% (95% CI 66-95) and 97% (95% CI 83-100) at 1-year follow-up and 98% (95% CI 87-100), 98% (87-100) at 2-year follow-up, respectively.
CONCLUSIONS: In this retrospective analysis, angioplasty and stent placement using the Venovo™ venous stent is safe and clinically effective for the treatment of both benign and malignant SVC occlusive disease. Reintervention for symptomatic restenosis is rare.

Superior vena cava syndrome (SVCS) is a clinical picture resulting from the obstruction of superior vena cava. SVCS usually causes symptoms and signs related to the head, the neck, the upper extremities and the upper thorax. We report a case of unusual clinical presentation of SVCS in a 25-year-old male patient who suffered from lymphoblastic lymphoma in childhood. Clinicians should be aware of abdominal wall presentations of SVCS especially in patients having undergone central venous catheterization, even years before. SVCS could be an emergency situation, requiring specific treatment or contraindicating surgical procedures.
Le syndrome de la veine cave supérieure (SVCS) est une entité nosologique résultant de l’obstruction de la veine cave supérieure. Un SVCS comporte habituellement des symptômes et des signes cliniques localisés à la région cranio-cervicale, aux membres supérieurs et au niveau thoracique supérieur. Nous rapportons un cas de présentation clinique inhabituelle de SVCS chez un patient de 25 ans aux antécédents de lymphome lymphoblastique dans l’enfance. En pratique clinique, il est important de garder à l’esprit l’existence de présentations inhabituelles pariétales abdominales de SVCS, en particulier chez les patients ayant subi un cathétérisme veineux central, même plusieurs années auparavant. Un SVCS peut nécessiter un traitement spécifique, parfois en urgence et contre-indiquer certaines interventions chirurgicales.

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The hemodialysis reliable outflow (HeRO) graft was designed for patients with failed arteriovenous accesses caused by an obstructed central venous outflow or patients who have exhausted all upper arm options. We describe a patient with severe stenosis of the superior vena cava (SVC) who experienced recurrent episodes of infection of permanent tunneled catheters, resulting in repeated hospitalizations. We successfully used the HeRO graft after angioplasty of the SVC. This indication for placing the HeRO graft has not been emphasized and mentioned previously in the literature. We also reviewed the literature regarding comparisons of this technique with other options.

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.
METHODS: PubMed, Scopus, and Cochrane Library databases were searched for studies up to December 2022.
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.
CONCLUSIONS: 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.

BACKGROUND: Data on transvenous (TV) lead-associated superior vena cava (SVC) syndrome are limited. The management of this problem might require a multidisciplinary approach, often involving transvenous lead extraction (TLE) followed by angioplasty and stenting.
OBJECTIVE: The purpose of this study was to describe the management and outcome of TV lead-associated SVC syndrome.
METHODS: We retrospectively identified patients with a diagnosis of SVC syndrome and TV leads at Emory Healthcare between 2015 and 2023.
RESULTS: Fifteen patients with lead-related SVC syndrome were identified. The cohort average age was 50 years. Symptoms included swelling of the face, neck, and upper extremities (67%); shortness of breath (53%); and lightheadedness (40%). Patients had an average of 2 ± 0.7 leads crossing the SVC, with a lead dwell time of 9.8 ± 7.5 years. Thirteen patients were managed with TLE, followed by SVC stenting and angioplasty in 10 and angioplasty alone in 2; 1 patient had no intervention after TLE. One patient was managed with anticoagulation, and another had angioplasty and stenting with lead jailing. One patient experienced SVC perforation and cardiac tamponade during SVC stenting, which was managed successfully with a covered stent and pericardiocentesis. Among the 12 patients with TLE and angioplasty ± stenting, 7 underwent reimplantation of a transvenous lead. Two of those patients had symptoms recurrence, and none of the 5 patients without lead reimplantation had recurrence of symptoms.
CONCLUSIONS: Lead-related SVC syndrome management requires a multidisciplinary approach often including TLE followed by angioplasty and stenting. Avoiding TV lead reimplantation might help reduce symptoms recurrence.

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