[Superior Vena Cava Syndrome due to Metastatic Cardiac Tumor:Report of a Case].

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.

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.

Percutaneous Endovascular Stent Placement for Treatment of Malignant Superior Vena Cava Syndrome: A Retrospective Review.

BACKGROUND: We assessed the safety as well as the efficacy of self-expanding stent placement for the treatment of malignant superior vena cava syndrome (SVCS), besides identifying the predictable probable factors for the clinical improvement of endovascular stent treatment in SVCS. METHODS: The study reviewed 112 patients (92 men) with malignant SVCS retrospectively from January 2015 to December 2020. RESULTS: Out of total 112 patients, 106 stents were successfully placed in 102 patients, however 4 patient's occlusions could not be passed and 6 patient's procedure was abandoned due to intraluminal thrombus as detected in venography. In 92 patients, complete resolution of syndrome was observed within 72 hrs but 10 patients did not to intervention. In 102 patients, procedure-related 8 complications were noted including stent migrations (n = 4), pulmonary embolism (n = 2), and pulmonary edemas (n = 2). Besides after stenting, 3 hemorrhages with anticoagulation therapy were observed with 4 recurrences at 22, 36, 51 and day 58 in 6 months. The pressure gradient across the lesion (≥ 20mmHg) was used as a predictor for clinical efficacy of stent therapy for SVCS. CONCLUSIONS: Endovascular stent insertion is a safe and effective intervention for malignant SVCS, especially for those with pressure gradient across the lesions ≥ 20mmHg.

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.

Refractory cerebrospinal fluid leak following posterior fossa surgery secondary to superior vena cava obstruction: a case report.

We report a case of refractory post-operative cerebrospinal fluid leak from a posterior fossa operative site secondary to superior vena cava construction. The patient developed the leak after a posterior fossa craniotomy and resection of a cerebellar lung metastasis. The leak persisted despite conservative measures and lumbar drain insertion. Suspicion of superior vena cava obstruction was raised following clinical examination. She was managed with stenting of the superior vena cava, with cessation of leak.

[Superior Vena Cava Syndrome due to Large Cell Neuroendocrine Carcinoma Originated in the Mediastinum].

We report a case of a 53-year-old man with superior vena cava( SVC) syndrome due to large cell neuroendocrine carcinoma (LCNEC) in the mediastinum. His chief complaint was general fatigue. On physical examination, both jugular veins were distended and his face and bilateral upper limbs were swollen. Enhanced chest computed tomography (CT) scan demonstrated a heterogenous tumor of approximately 50 mm in diameter in the middle mediastinum, which infiltrated into the SVC and right atrium, and caused SVC syndrome. Since SVC syndrome developed rapidly, the tumor was resected and the occluded SVC was replaced with a ringed polytetrafluoroethylene graft under cardiopulmonary bypass. After surgery, SVC obstruction was resolved with improvement of the initial symptoms. The patient had an uneventful recovery and was discharged from our hospital. The tumor was diagnosed as LCNEC histologically. He received postoperative adjuvant systemic chemotherapy. Unfortunately, he died of extensive brain and bone metastasis 10 months after the operation. However, we believe that surgical relief from SVC syndrome improved quality of the rest of his life.

[Hemangioendothelioma of the right innominate vein]. / Gemangioendotelioma pravoi plechegolovnoi veny.

The authors report a rare case of hemangioendothelioma of the right innominate vein complicated by superior vena cava syndrome. Considering development of enlarged venous collaterals, we performed circular resection of superior vena cava together with the right and left innominate veins without replacement. There were no postoperative complications. The use of vascular prosthesis in patients with long-standing superior vena cava compression and enlarged collaterals is associated with high risk of thrombosis and subsequent pulmonary embolism. Safe circular resection of superior vena cava without replacement is possible after intraoperative test clamping.

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.

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.

Safety and Efficacy of Stent Graft Implantation for Malignant Superior Vena Cava Syndrome.

BACKGROUND: Patients with malignant superior vena cava syndrome (SVCS) usually require urgent treatments due to a high potential risk of early mortality. Stent implantation can rapidly improve the symptoms of SVCS, which may be beneficial to subsequent anti-tumor therapy. The aim of the study was to evaluate the clinical outcomes of stent graft implantation for the treatment of superior vena cava (SVC) obstruction caused by non-small cell lung carcinoma (NSCLC) with acute post-stenting occlusion. METHODS: Between October 2014 and December 2019, 16 patients were selected for stent graft implantation. Technical success and clinical efficacy were assessed. Stent patency and patient survival rates, as well as the complications were analyzed. RESULTS: There were 17 stent grafts implanted in 16 patients. The technical success was 100%. The residual stenosis after initial implantation was 64.0 ± 9.0%. The stent expanded to an optimal size in 5.5 ± 2.2 days after the initial deployment. Migration occurred when deploying of the stent graft in one patient; this stent graft was successfully stabilized by a second one. No other complications related to the procedure were found except one migration. At 1, 3, 6, 9 and 12 months, the cumulative survival rates were 100%, 75%, 56%, 19% and 0%, respectively. The mean OS was 173 days. The median survival was 166 days. CONCLUSIONS: Stent graft can be safely used in patients with SVC obstruction with a good long-term patency rate.

Percutaneous paraspinal access to the azygos vein to create a neo-vena cava.

We present the case of a child with congenital heart disease repaired in infancy with diffuse central venous occlusions resulting in central venous insufficiency, superior vena cava (SVC) syndrome, and intracranial bleeds. He presented to the catheterization laboratory for multiple transcatheter interventions to recanalize central venous channels which were unsuccessful by conventional methods. Therefore, extravascular venous channels were created to decompress his upper body by creation of a neo-SVC and his lower body with a neo-azygos vein to the neo-SVC. The latter procedure required direct percutaneous access from a paraspinal approach in order to obtain continuity with the azygos vein. At latest follow-up the patient is clinically well and asymptomatic.

Successful venoplasty of superior vena cava stenosis in a patient with a total artificial heart after orthotopic heart transplantation due to primary graft failure.

BACKGROUND: With the limited number of available suitable donor hearts resulting in plateaued numbers of heart transplantations, short- and long-term mechanical circulatory support devices, including the implantation of total artificial hearts (TAHs) are modalities that are increasingly being used as treatment options for patients with end-stage heart failure. The superior vena cava syndrome has been described in this context in various disease processes. We report successful venoplasty for superior vena cava syndrome in a patient with a TAH. CASE PRESENTATION: A 65-year-old man with a history of nonischemic cardiomyopathy had received a left ventricular assist device, and then 2 years later, underwent orthotopic heart transplantation using the bicaval anastomosis technique. The postprocedural course was complicated by primary graft failure, resulting in the need for implantation of a TAH. About 5 months after TAH implantation, he started to develop complications such as volume retention, swelling of the upper extremities, and was diagnosed to have a superior vena cava syndrome. The patient underwent a successful venoplasty of his superior vena cava by interventional radiology with resolution of upper body edema, normalization of renal, and liver function. CONCLUSION: Potential fatal complications caused by catheter or wire entrapment in the right-sided mechanical valve of a TAH have been reported. We describe a safe method for the treatment of superior vena cava syndrome in patients with TAH.

Macrocephaly Secondary to Superior Vena Cava Syndrome.

Cardiac defects in neonates are often associated with neurological or neuroanatomical anomalies. We present a patient who developed macrocephaly secondary to superior vena cava syndrome, resultant from repair of her congenital tetralogy of Fallot. She was managed conservatively with serial imaging and close observation from the neurosurgical aspect, and her head growth stabilized without requiring ventriculoperitoneal shunting after stent placement in her superior vena cava. In patients with macrocephaly secondary to cardiac issues, cerebrospinal fluid diversion is often performed quickly. For our patient - who had no obvious signs of worsening intracranial hypertension or symptoms beyond macrocephaly - treatment of her overall cardiovascular issues was successful in achieving stability of her head circumference growth.

Salvaging the chemoport using catheter-assisted deflection during stenting of the superior vena cava.

Patients with malignancy who develop superior vena cava (SVC) obstruction may require stenting to relieve their symptoms. Some of these individuals also have an indwelling chemoport for concomitant chemotherapy. We present a case where stenting was accomplished after catheter-assisted deflection of the chemoport catheter tubing via a single groin access. It can save procedure time whilst salvaging the device.

Half a century of continuous pacing: a living witness to the evolution of a technology.

To chart the development of pacing technology and its pitfalls we present the experience of a patient who has benefitted from it but also suffered as a result of it from its earliest days. A 53-year-old physician was referred to us with obstruction of the superior and inferior vena cava on a background of more than 50 years of continuous ventricular pacing and 24 previous pacemaker-related interventions. In a single surgical procedure, his existing pacing system and redundant leads were extracted, the superior vena cava was reconstructed, and a new biventricular pacing system with epicardial leads was implanted. Pacemakers can maintain life and preserve the quality of life for many decades. The quality of this therapy has improved due to advances in the technology and in techniques. Maintaining safe pacing in the very long term requires labour, patience, and ingenuity.

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.

Total Endovascular Repair of Post-Trauma Ascending Aortic Pseudoaneurysm and Secondary Superior Vena Cava Syndrome.

BACKGROUND: Clavicular fracture or sternoclavicular luxation is observed in 10% of all polytrauma patients and is frequently associated with concomitant intrathoracic life-threatening injuries. Posterior sternoclavicular luxation is well known to induce underlying great vessels damage. The gold standard treatment usually is a combined orthopedic and cardiovascular surgical procedure associating vascular repair, clavicular open reduction, and internal fixation. METHODS: A 59-year-old wheelchair ridden, institutionalized woman, known for psychiatric disorder, severe scoliosis, malnutrition, and chronic obstructive pulmonary disease was admitted in our hospital for chronic chest pain 3 months after a stairway wheelchair downfall. A thoracic computed tomography (CT) scan revealed a voluminous ascending aortic pseudoaneurysm (63 × 58 mm, orifice 5 mm) consecutive to perforation following posterior sternoclavicular luxation. The patient refused all therapies and was lost to follow-up. Six months later, she was readmitted for a symptomatic superior vena cava syndrome. Thoracic CT scan revealed pseudoaneurysm growth with innominate vein thrombosis and superior vena cava subocclusion. Pseudoaneurysm orifice was stable. In the presence of symptoms with massive facial edema and inability to open her eyelids, the patient accepted an endovascular treatment. RESULTS: The procedure was performed under general anesthesia using both fluoroscopic and transesophageal echocardiographic guidance. Through a femoral arterial access, a 10-mm atrial septal defect occluder device was used to seal successfully the pseudoaneurysm orifice. The superior vena cava was then opened with a 26-mm nitinol high radial force stent through a femoral venous access. Postoperative course was uneventful. At 3-month follow-up, the patient remains symptom free and a CT scan confirmed pseudoaneurysm thrombosis and superior vena cava permeability. CONCLUSION: Post-traumatic sternoclavicular posterior luxation is a cause of great vessels and ascending aorta injuries. Minimally invasive endovascular approaches can be considered to treat vascular injuries and their consequences, especially in elderly patients and those at high risk for surgery.

Is Long-Term Anticoagulation Required after Stent Placement for Benign Superior Vena Cava Syndrome?

PURPOSE: To identify whether symptom relief and stent patency vary with use of long-term anticoagulation after stent placement for benign superior vena cava (SVC) syndrome. MATERIALS AND METHODS: Patients with benign SVC syndrome treated with stent placement between January 1999 and July 2017 were retrospectively identified (n = 58). Average age was 49 years (range, 24-80 y); 34 (58%) were women, and 24 (42%) were men. Average follow-up was 2.4 years (range, 0.1-11.1 y, SD 2.6). Of cases, 37 (64%) were due to a long-term line/pacemaker, and 21 (36%) were due to fibrosing mediastinitis. After stent placement, 36 (62%) patients were placed on long-term anticoagulation, and 22 (38%) were not placed on anticoagulation. Percent stenosis was evaluated on follow-up imaging by dividing smallest diameter of the stent by a normal nonstenotic segment of the stent and multiplying by 100. RESULTS: Technical success was achieved in all cases. There was no significant difference in number of patients who reported a return of symptoms characteristic of benign SVC syndrome between the anticoagulated (16 of 36; 44.4%) and nonanticoagulated (11 of 22; 50%) groups (P = .68). There was no significant difference in the mean percent stenosis between the anticoagulated (40.4% ± 34.7% [range, 0-100%]) and nonanticoagulated (32.1% ± 29.2% [range, 1.7%-100%]) groups (P = .36). No significant difference was found in the time (days) between date of procedure and date of return of symptoms (anticoagulated, 735.9 d ± 1,003.1 [range, 23-3,851 d]; nonanticoagulated, 478 d ± 826.6 [range, 28-2,922 d]) (P = .49). There was no difference in primary patency between groups (P = .59). Finally, 1 patient (2.8%) in the anticoagulated group required surgical intervention, whereas none in the nonanticoagulated group required surgical intervention. CONCLUSIONS: No significant difference was observed in clinical and treatment outcomes in patients who did and did not receive anticoagulation after stent placement for benign SVC syndrome. Management of benign SVC syndrome after stent placement may not require anticoagulation if confirmed by additional studies.

When There Is a Will, There Is Always a Way: Superior Vena Cava Recanalization Using a Hepatic Vein Access.

Superior vena cava (SVC) obstruction is a major complication of different benign, malignant, or iatrogenic etiologies. Angioplasty is the standard of care when conservative treatment fails. We hereby report a hepatic vein percutaneous access when conventional venous access fails or is not available to perform vascular recanalization in a young patient with SVC obstruction.

Right Atrial Anomalous Muscle Bundle Presenting with Acute Superior Vena Cava Syndrome and Pulmonary Embolism: Surgical Management.

BACKGROUND: An anomalous muscle bundle (AMB) crossing the right atrial cavity represents a pathologic finding with unproved clinical significance. This congenital anomaly may be difficult to recognize via echocardiography and could be confused with other intracavitary lesions. METHODS: We report the case of a 53-year-old woman presented to the cardiovascular service with acute superior vena cava (SVC) syndrome and submassive pulmonary embolism. RESULTS: The patient underwent venography, confirming SVC stenosis. A ventilation/perfusion lung scan showed 2 sizable perfusion defects because of pulmonary embolism. Magnetic resonance imaging and echocardiography imaging demonstrated a right atrium (RA) mass. Surgery was then carried out using standard cardiopulmonary bypass; the right atrial muscle bundle was excised, and SVC reconstruction was performed. The patient was discharged uneventfully and remains symptom-free at 2-year follow-up. CONCLUSIONS: In cases of nonmalignant pathology of SVC syndrome, appropriate studies should be conducted to exclude potential congenital abnormalities such as this AMB in the RA. Open-heart surgery is a viable treatment option in select cases.