Venous congestion of the head, neck, and upper extremities resulting from impaired venous flow through the superior vena cava (SVC) to the right atrium.

Epidemiology

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Etiology

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• Malignant SVC syndrome (most common) (extravascular)
  • The following entities account for > 80% of all SVC syndrome cases:
    • Non-small cell lung cancer (NSCLC)
      • Pancoast tumor
    • Small cell lung cancer (SCLC)
    • Non-Hodgkin lymphoma (NHL)
  • Less common: metastatic cancer (usually breast cancer), germ cell tumors, thymoma, mesothelioma
• Nonmalignant SVC syndrome (intravascular)
  • Most commonly caused by thrombosis associated with an intravascular device (e.g., dialysis catheter, pacemaker wire)

Pathophysiology

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Clinical features

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• Hemodynamic symptoms
  • Edema of the upper extremities and face (facial plethora)
  • Prominent venous pattern on the chest, face, and upper extremities
  • Jugular venous distension
• Symptoms and signs of congestion of the neck
  • Dyspnea
  • Cough and hoarseness
• Neurological symptoms
  • Headache
  • Dizziness

Warning

Evaluate frequently for signs of laryngeal edema, hemodynamic instability, and ↑ ICP.

Diagnostics

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Treatment

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