Syndrome of the superior vena cava

Syndrome of the superior vena cava (SVVV) is a veno-occlusive disease that leads to clinically significant disturbance of venous outflow from the upper vena cava basin.

What causes upper vena cava syndrome?

78-97% of adult SVRV cases are caused by oncological diseases. At the same time, about half of patients with SVPC suffer from lung cancer, and up to 20% with non-Hodgkin's lymphomas. The syndrome of the inferior vena cava appears more rarely in metastatic breast cancer, even less often with other malignant neoplasms affecting the mediastinum and lungs (in Hodgkin's lymphoma, thymoma or germinogenic tumors, SVPV develops in less than 2% of cases).

The causes of SVPC development are compression of the inferior vena cava, tumor invasion, thrombosis or sclerotic change of this vessel.

The syndrome of the superior vena cava of non-tumorous etiology is met with:

• retrosternal craw,
• purulent mediastinitis,
• sarcoidosis,
• silicosis,
• constrictive pericarditis,
• post-radiation fibrosis,
• teratomia of the mediastinum,
• idiopathic mediastinal fibrosis,
• any disease, it is possible to develop SVPV as a result of thrombosis and sclerosis of the vein with prolonged standing of the catheter in the superior vena cava.

In children, the cause of the syndrome of the superior vena cava is a prolonged catheterization of the inferior vena cava, and about 70% of cases of SVPV associated with malignant neoplasms are caused by diffuse large-cell or lymphoblastic lymphomas.

Symptoms of upper vena cava syndrome

The clinical symptoms and severity of the syndrome of the superior vena cava depend on the rate of growth and localization of obstruction, the severity of thrombosis and the adequacy of collateral blood flow. Usually the syndrome develops gradually over a period of several weeks, with collateral blood flow through the v azygos and the anterior thoracic veins in the inferior vena cava. The SVPC has specific and early symptoms.

A specific symptom of SVPV is an increase in venous pressure in the system of the inferior vena cava (head, neck, upper half of the trunk and arm) above 200 cm of water. Art.

The earliest symptom of the syndrome of the superior vena cava is the neck, which does not subside in the orthostasis of the vein. More often the syndrome is detected when a persistent thick edema of the face, neck (symptom of a "tight collar"), upper half of the trunk and the appearance of dyspnea occur. In this case, patients often complain of headache, dizziness, blurred vision, cough, chest pain, general weakness. You can identify dysphagia, hoarseness of the voice, swelling of the lining space, swelling of the tongue.

Symptoms are worse when lying down and with tilts.

With a significant violation of outflow from the veins of the head, thrombosis of the sagittal sinus and edema of the brain are possible.

In rare cases of rapid development of obstruction of the superior vena cava, an increase in venous pressure leads to an increase in ICP, cerebral edema, cerebral vascular thrombosis or hemorrhagic stroke.

Diagnosis of the syndrome of the superior vena cava

Additional instrumental examination is carried out to clarify the localization and character of obstruction of the superior vena cava. The most informative is CT and angiography, according to the results of which it is possible to judge the localization, extent and nature of vein obstruction (thrombosis or compression of the vein from the outside), collateral blood flow, and get a detailed picture of the tumor and its relation to other structures of the mediastinum and thorax. Clarification of tumor localization as a result of CT allows for percutaneous transthoracic biopsy (it is safer than open biopsy or mediastinoscopy) followed by morphological verification of the tumor.

[1], [2], [3], [4], [5]

Treatment of upper vena cava syndrome

General therapeutic measures for upper vena cava syndrome include permanent inhalation of oxygen, the appointment of sedatives that reduce CB and pressure in the system of the superior vena cava, ensuring the improvement of the condition of most patients, bed rest (the head end of the patient's bed should be raised).

Emergency treatment is indicated in severe course of the syndrome of the inferior vena cava with the development of cerebral edema, balloon dilatation of the occluded vein and its stenting. If the operation is successfully performed, immediate relief of the symptoms of SVPV of any etiology is observed, the quality of life is improved and the possibility of additional examination, clarification of nosology, morphological verification, adequate specific treatment (antitumor therapy, surgical treatment, etc.) is provided. The indication for emergency symptomatic irradiation of the region of tumor obstruction of the inferior vena cava is limited to severe SVPV, which is accompanied by airway obstruction, spinal cord compression, or a rapid increase in ICP with the technical impossibility of balloon angioplasty.

Depending on the cause of development of the syndrome of the superior vena cava, the tactics of treatment are different.

• If the cause of SVVV is sclerosis of the vein due to the long standing of the catheter, balloon dilatation of the occluded department (sometimes followed by stenting) is performed. In patients with oncological etiology of SVPV, the stent remains for life.
• With occlusive thrombosis and no contraindications, systemic thrombolysis is effective (streptokinase 1.5 million units intravenously for an hour) followed by the introduction of direct anticoagulants (heparin sodium 5000 ED 4 times a day for a week).
• With the tumor etiology of SVPV, a lasting improvement can be expected only from specific antitumor treatment, the prognosis directly depends on the prognosis of the oncological disease. Small cell lung cancer and non-Hodgkin's lymphomas, the two most common oncologic causes of SVPV, are sensitive to chemotherapy and potentially curable. With these diseases, the resolution of SVPC symptoms can be expected within the next 1-2 weeks after the onset of antitumor therapy. The syndrome recurs after 25% of patients. When large-cell lymphomas and a significant tumor mass of cells localized in the mediastinum, usually resort to combined chemoradiotherapy. Irradiation of the mediastinal organs is indicated in most tumors that are insensitive to chemotherapy, or with an unidentified morphological structure of the tumor. Relief of symptoms of the syndrome of the superior vena cava occurs within 1-3 weeks after the beginning of radiation treatment.

Auxiliary drugs - glucocorticoids (dexamethasone) - have their own antitumor activity in lymphoproliferative diseases and can be used as a "deterrent therapy" with a reasonable assumption of such an etiology of the upper vena cava syndrome before morphological verification. Anticoagulants of direct action are indicated in the treatment of thromboses caused by catheterization of the central veins, especially when spreading thrombi into the brachiocephalic or subclavian veins. However, in conditions of elevated ICP, anticoagulant therapy increases the risk of developing intracranial hematomas, and tumor biopsy with anticoagulant therapy is often complicated and bleeding from it.