Objective: Superior vena cava syndrome (SVCS) comprises a cluster of clinical manifestations secondary to partial or complete obstruction of the superior vena cava (SVC), which increases risk of upper extremity deep venous thrombosis (UEDVT). Up to 65% of cases are attributed to malignancy but recent evidence has reported an increased incidence of nonmalignant causes due to increased use of indwelling devices. The clinical course of SVCS ranges from asymptomatic presentation to severe neurologic, cardiovascular and respiratory manifestations. Thus, concern for increased morbidity burden is significantly higher when untreated. We aim to summarize current evidence for treatment of SVCS.
Methods: We performed a literature review of studies published from 2013 to 2023 in PubMed, Medline, Google Scholar and Cochrane. MeSH terms for our search included “superior vena cava syndrome”, “treatment”, “venous thrombosis”, “deep venous thrombosis”, “central venous obstruction”, “central venous thrombosis, “thoracic central venous occlusion”, “complications”, “surgical repair”, and “endovascular therapy”. Included publications corresponded to reviews, systematic reviews, clinical trials, meta-analysis, and consensus documents.
Results: Out of 42 matching results, 19 papers met the inclusion criteria. Up to 14.9% of patients with cancer and UEDVT develop SVCS. The greatest predictor of survival in malignant SVCS is the aggressiveness of the underlying malignancy. Chemotherapy and nonoperative management is primarily indicated in patients without life-threatening symptoms. Open surgery is usually indicated for patients with malignant invasion. Percutaneous stent insertion provides superior outcomes for symptomatic relief and SVC obstruction versus radiation therapy. Thrombolysis prior to stenting is preferred for malignant thrombotic SVC obstruction. Benign SVCS is caused by central venous catheters and pacemakers in up to 80.6% of cases, angioplasty and stenting is the most common intervention (73.6%), and 26.9% of cases require secondary procedures. Mediastinal fibrosis is the main indication for open repair; 15% of patients who undergo open repair require re-intervention due to graft thrombosis, and 28.4% undergo secondary procedures before discharge. Anticoagulants or thrombolysis are indicated upon admission for both benign and malignant SVCS. No differences in patency rates have been shown according to stent type. In case of SVC thrombosis with concomitant UEDVT, venous lysis in addition to stenting with or without venoplasty and/or stent placement is superior in comparison to venous lysis without intervention. No standardized endovascular technique has been established for SVC thrombosis.
Conclusions: Approach patterns for SVCS have significant variability. Physicians must determine the most suitable intervention on a case-dependent basis. Thrombolysis should be initiated regardless of etiology in emergent settings assuming the patient can tolerate the potential administration of lytic agents. Further evidence is required to establish standardized clinical guidelines for SVCS treatment.
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