II. Pathophysiology

  1. Superior Vena Cava Obstruction to flow
    1. Internal Vascular Invasion
      1. Vena cava thrombosis (uncommon complication of implantable devices, pacers, AICD, Dialysis Catheter)
      2. Tumor infiltration
    2. External Compression (e.g. right lung or Mediastinal Mass)
      1. Most common Mediastinal Masses include Lung Cancer and Lymphoma
      2. Mediastinal Mass gradually compresses superior vena cava, blocking entry into right atrium
      3. Results in retrograde flow and regional edema
  2. Superior Vena Cava Obstruction Compensation
    1. SVC is a thin walled, low pressure large vein 2 cm in width and 4-6 cm in diameter
    2. Upper body venous engorgement distributes the fluid
    3. High venous pressures within SVC may be compensated with collateral circulation over 1-2 weeks
      1. Example: Azygous vein may provide some collateral drainage

III. Causes

  1. Malignancy (60% of all cases)
    1. Bronchogenic Carcinoma (80% of malignancy causes)
    2. Malignant Lymphoma (15% of malignancy causes)
    3. Metastatic Disease (to lung, mediastinum or Lymph Nodes)
      1. Breast adenocarcinoma
      2. Testicular seminoma
  2. Vena Cava thrombosis (majority of non-malignant cases)
    1. Long-term venous catheters (40% of thrombosis cases)
    2. Idiopathic
    3. Behcet's Syndrome
    4. Polycythemia Vera
    5. Paroxysmal Nocturnal Hemoglobinuria
    6. Long-term shunts
    7. Long-term Pacemakers
  3. Other Benign Disease (Rare)
    1. Mediastinal fibrosis
      1. Post-Radiation Therapy
      2. Idiopathic
      3. Histoplasmosis
      4. Actinomycosis
      5. Tuberculosis
    2. Benign Mediastinal tumor
      1. Aortic aneurysm
      2. Dermoid tumor
      3. Goiter
      4. Sarcoidosis

IV. Symptoms

  1. Presentations are typically subacute and progressive
  2. Dyspnea (50%)
    1. Worse with bending forward
  3. Neck and facial swelling (40-82%)
    1. Head fullness or pressure Sensation
    2. Provoked by lying supine
  4. Swelling of trunk and upper extremities (40%)
  5. Local Airway Compression
    1. Cough
    2. Dysphagia
    3. Choking Sensation
    4. Hoarseness
    5. Lacrimation
    6. Nasal Congestion
  6. Headache
    1. Common presenting complaint
    2. Secondary to Increased Intracranial Pressure
  7. Referred Pain
    1. Chest Pain
    2. Shoulder Pain
  8. Neurologic
    1. Dizziness (provoked by bending forward)
    2. Visual changes or Blurred Vision
    3. Headaches
    4. Syncope
    5. Altered Mental Status

V. Signs

  1. Thoracic chest vein distention (65%)
  2. Neck vein distention (55%)
  3. Facial Edema (55%)
  4. Tachypnea (40%)
  5. Plethora of the face and Cyanosis (15%)
  6. Edema of upper extremities (10%)
  7. Paralysis of Vocal Cords (3%)
  8. Horner's Syndrome (3%)
  9. Distended antecubital veins
  10. Tongue Swelling
  11. Pemberton's Sign
    1. Patient elevates both arms and observe face for one minute
    2. Positive if significant facial swelling (plethora) and Cyanosis within the first minute
  12. Clear lungs despite Dyspnea and Tachypnea
    1. See Causes of Dyspnea with Clear Lung Sounds

VI. Associated Conditions

VIII. Imaging

  1. Chest XRay
    1. Mass identified in 90%
    2. Right superior mediastinum widening (75%)
    3. Hilar Adenopathy (50%)
    4. Right Pleural Effusion (25%)
  2. Chest CT with IV Contrast (preferred)
    1. Identifies mass lesions and sites of SVC obstruction and thrombosis
    2. Collateral vessel development is a sensitive and specific marker of SVC Syndrome
      1. Test Specificity: 96%
      2. Test Sensitivity: 92%
  3. Ultrasound
    1. Consider in the evaluation for upper extremity Deep Vein Thrombosis
  4. Other Imaging modalities
    1. MR Venogram
    2. Cavogram

IX. Management

  1. Severity of airway and vascular compromise varies by timing of progression
    1. Rapid progression may not allow for compensation, resulting in acute airway compromise (uncommon)
  2. Supportive care for airway compromise
    1. ABC Management
    2. High-Dose Corticosteroids
      1. Most effective in steroid-responsive malignancy such as Lymphoma or thymoma
    3. Elevate the head of the bed
    4. Avoid Diuretics
  3. Identify mass etiology
  4. Reduction in mass size
    1. Emergent Intervention RadiologyConsultation
    2. Other modalities
      1. Radiation Therapy (e.g. Non-Small Cell Lung Cancer)
      2. Chemotherapy
      3. Surgical decompression
  5. Consult Oncology and Cardiothoracic Surgery acutely
    1. Palliative CareConsultation once stabilized
  6. Anticoagulation with Heparin
    1. Indicated for venous thrombosis
    2. Consult for catheter directed Thrombolysis or thrombectomy in hemodynamically unstable patients
  7. Endovascular stenting
    1. Common acute management with 85 to 100% success rate
    2. Improvement is seen within days
    3. Effective even when tumor mass is not reducible

X. Prognosis

  1. Poor prognostic sign
    1. Predicts 90% mortality in 3 years
    2. Median survival: 6 months

XI. References

  1. Long, Long and Koyfman (2020) Crit Dec Emerg Med 34(11): 17-24
  2. Swaminathan and Hedayati (2022) EM:Rap 22(6): 7-9
  3. Higdon (2018) Am Fam Physician 97(11):741-8 [PubMed]

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Related Studies

Ontology: Superior Vena Cava Syndrome (C0038833)

Definition (NCI_NCI-GLOSS) A condition in which a tumor presses against the superior vena cava (the large vein that carries blood from the head, neck, arms, and chest to the heart). This pressure blocks blood flow to the heart and may cause coughing, difficulty in breathing, and swelling of the face, neck, and upper arms.
Definition (NCI_CTCAE) A disorder characterized by obstruction of the blood flow in the superior vena cava. Signs and symptoms include swelling and cyanosis of the face, neck, and upper arms, cough, orthopnea and headache.
Definition (NCI) Obstruction of the blood flow in the superior vena cava caused by a malignant neoplasm, thrombosis, or aneurysm. It is a medical emergency requiring immediate treatment. Signs and symptoms include swelling and cyanosis of the face, neck, and upper arms, cough, orthopnea, and headache.
Definition (MSH) A condition that occurs when the obstruction of the thin-walled SUPERIOR VENA CAVA interrupts blood flow from the head, upper extremities, and thorax to the RIGHT ATRIUM. Obstruction can be caused by NEOPLASMS; THROMBOSIS; ANEURYSM; or external compression. The syndrome is characterized by swelling and/or CYANOSIS of the face, neck, and upper arms.
Concepts Disease or Syndrome (T047)
MSH D013479
SnomedCT 63363004, 155450008
English Superior Vena Cava Obstruction, Superior Vena Cava Syndrome, SVC - Super vena cava obstruc, SVC - Superior vena cava obstruction, Superior vena cava obstruction, SUPERIOR VENA CAVA OBSTRUCTION, SVCO-Super vena cava obstruct, SVCO - Superior vena cava obstruction, superior vena cava syndrome (diagnosis), superior vena cava syndrome, SVC syndrome, Superior vena caval obstruction, SVC obstruction, Superior Vena Cava Syndrome [Disease/Finding], cava superior syndromes vena, superior vena caval obstruction, caval obstruction superior vena, superior vena cava obstruction, superior vena caval syndrome, svc obstruction, svc syndrome, vena cava superior syndrome, Superior vena cava syndrome, Superior vena cava syndrome (disorder)
Dutch vena cava superior obstructie, vena cava superior syndroom, Syndroom, vena-cava-superior-, Vena-cava-superiorobstructie, Vena-cava-superiorsyndroom
French Obstruction de la VCS, Obstruction de la veine cave supérieure, Syndrome de la veine cave supérieure, Syndrome de compression de la veine cave supérieure
German Verschluss der Vena cava superior, Obstruktion der Vena cava superior, Vena cava superior, Vena-cava-superior-Syndrom, Vena-cava-superior-Verschluß
Portuguese Obstrução da veia cava superior, Síndrome de veia cava superior, Obstrução da Veia Cava Superior, Síndrome da Veia Cava Superior
Spanish Obstrucción de la vena cava superior, Obstrucción de la VCS, Síndrome de la vena cava superior, síndrome de la vena cava superior (trastorno), síndrome de la vena cava superior, Obstrucción de la Vena Cava Superior, Síndrome de la Vena Cava Superior
Japanese 上大静脈閉塞, ジョウダイジョウミャクヘイソク, 上大静脈閉塞症候群, 上大静脈血栓症, 上大静脈閉塞症, ジョウダイジョウミャクショウコウグン, SVC症候群, 上大静脈症候群, SVCヘイソク, SVC閉塞
Swedish Vena cava superior-syndrom
Finnish Yläonttolaskimo-oireyhtymä
Czech Obstrukce horní duté žíly, Obstrukce HDŽ, Syndrom horní duté žíly, syndrom venae cavae superioris, syndrom horní duté žíly
Italian Ostruzione della vena cava superiore, Sindrome della vena cava superiore
Polish Zespół żyły głównej górnej
Hungarian Felső vena cava syndroma, SVC obstructio, Vena cava superior obstructiója
Norwegian Vena cava superior-syndrom

Ontology: Collar of Stokes (C0265073)

Concepts Disease or Syndrome (T047)
SnomedCT 24187003
English Collar of Stokes, Collar of Stokes (disorder)
Spanish collar de Stokes (trastorno), collar de Stokes

Ontology: Occlusion of superior vena cava (C1336532)

Definition (NCI) Blockage of the lumen of the superior vena cava.
Concepts Finding (T033)
SnomedCT 470759007
Italian Occlusione della vena cava superiore
Japanese 上大静脈閉塞, ジョウダイジョウミャクヘイソク
Czech Okluze vena cava superior
English Occlusion of superior vena cava (disorder), Occlusion of superior vena cava, Superior caval vein obstruction, Obstruction of superior vena cava, Superior vena cava occlusion, Superior Vena Cava Occlusion
Spanish oclusión de vena cava superior, oclusión de vena cava superior (trastorno), Oclusión de la vena cava superior
Hungarian Vena cava superior elzáródása
Portuguese Oclusão da veia cava superior
Dutch vena cava superior-occlusie
French Occlusion de la veine cave supérieure
German Verschluss der Vena cava superior