Approach

Once diagnosis has been established, a malignant or non-malignant cause of superior vena cava (SVC) syndrome must be determined, as treatment options differ. Treatment involves relieving the symptoms of obstruction and treating the underlying aetiology. There have been no large randomised trials to compare various treatment options, and most data are from case series and expert opinion. Treatment of SVC syndrome is inter-disciplinary and may involve oncology, respiratory, surgery, radiology, vascular, and endovascular specialists.[2]

Acute airway obstruction (without tissue diagnosis)

Presentation with airway obstruction is serious, although rare in current clinical practice. First-line treatment consists of securing the airway and relief of obstructive symptoms.​ This can be achieved with either a combination of corticosteroids and radiotherapy, or percutaneous stenting.[2] Urgent treatment with radiotherapy and corticosteroids should be used only for life-threatening situations. It should be deferred otherwise, due to interference with subsequent histopathological diagnosis. Stenting is increasingly used because the stent can be placed before a tissue diagnosis is available. It is a useful procedure for patients with severe symptoms such as respiratory distress that require urgent intervention.[21][22]​ Meta-analyses have demonstrated that endovascular therapy with stenting has high technical and clinical success rates.[23][24][25]

In the absence of a need for urgent intervention, the management should focus initially on establishing the correct diagnosis.

Malignant obstructions

Malignant causes require further treatment with appropriate chemotherapy, radiation, and/or surgery. Most malignant tumours causing SVC syndrome are sensitive to radiotherapy. Chemotherapy is an effective option for treatment of lung cancer, lymphomas, and germ cell tumours.[26] Thymomas resistant to chemotherapy and radiation may require surgical resection and SVC reconstruction.[27] Selection of therapy will depend on the type of malignancy, staging, and histopathology. See Small cell lung cancer, Non-small cell lung cancer, Non-Hodgkin's lymphoma, Thymic tumour.

Endovascular stenting is performed to achieve more rapid improvement in symptoms and has fewer side effects compared with radiotherapy.[23][24][25]

Second-line treatment is palliative therapy. This includes palliative radiotherapy, chemotherapy or corticosteroids (for lymphomas and thymomas), endovascular stents, or rarely bypass surgery.[1][2]​ In rare cases, surgical decompression can be performed. Thrombolysis with indwelling catheters has also been described in small studies.[28] Supportive treatment consists of diuretics, low-salt diet, avoidance of upper-extremity lines, head elevation, and oxygen.

Benign obstructions

Benign causes can be managed with percutaneous stenting, intravascular thrombolysis, bypass grafting, anticoagulation, or treatment of underlying infectious aetiology.

Underlying infection (e.g., aspergillosis, blastomycosis, histoplasmosis, nocardiosis) should be treated according to local sensitivities. Endovascular stents and more rarely bypass surgery may be required if SVC obstruction persists after treatment of infection.

Catheter(s) should be removed and local thrombolysis and/or short-course anticoagulation should be considered in patients with thrombosis due to central venous catheter(s).[29]

Percutaneous balloon dilatation/stenting is preferred in patients with pacemaker and implantable cardioverter-defibrillator lead-related venous occlusion. Lead explantation may carry a high risk of mortality.[30] Bypass surgery may be an option. Infection of the leads should always be considered as a possibility and evaluated with blood cultures and transoesophageal echocardiogram. Anticoagulation with warfarin should be considered. Data regarding post-procedural anticoagulation are lacking, and practices vary.[2]

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