II. Pathophysiology

  1. Azygous vein may provide some collateral drainage
  2. Superior Vena Cava Obstruction to flow
    1. Vena cava thrombosis
    2. Mediastinal mass gradually compresses superior vena cava, blocking entry into right atrium
      1. Results in retrograde flow and regional edema

III. Causes

  1. Malignancy
    1. Bronchogenic Carcinoma (80%)
    2. Malignant Lymphoma (15%)
    3. Metastatic Disease (to lung, mediastinum or lymph nodes)
      1. Breast adenocarcinoma
      2. Testicular seminoma
  2. Benign Disease (Rare)
    1. Mediastinal fibrosis
      1. Idiopathic
      2. Histoplasmosis
      3. Actinomycosis
      4. Tuberculosis
    2. Vena Cava thrombosis
      1. Idiopathic
      2. Behcet's Syndrome
      3. Polycythemia Vera
      4. Paroxysmal Nocturnal Hemoglobinuria
      5. Long-term venous catheters, shunts or Pacemakers
    3. Benign Mediastinal tumor
      1. Aortic aneurysm
      2. Dermoid tumor
      3. Goiter
      4. Sarcoidosis

IV. Symptoms

  1. Dyspnea (50%)
  2. Neck and facial swelling (40%)
  3. Swelling of trunk and upper extremities (40%)
  4. Choking sensation
  5. Hoarseness
  6. Head fullness or pressure sensation
  7. Headache
  8. Chest Pain
  9. Shoulder Pain
  10. Cough
  11. Lacrimation
  12. Dysphagia

V. Signs

  1. Thoracic 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

VI. Associated Conditions

  1. Spinal Cord compression

VII. Radiology

  1. Chest XRay shows mass in 90%
    1. Right superior mediastinum (75%)
    2. Hilar Adenopathy (50%)
    3. Right Pleural Effusion (25%)
  2. Chest CT

VIII. Management

  1. Identify mass etiology
  2. Consult Oncology and Cardiothoracic Surgery acutely
    1. Palliative CareConsultation once stabilized
  3. Supportive care
    1. ABC Management
    2. Corticosteroids
    3. Diuretics
    4. Elevate the head of the bed
  4. Reduction in mass size
    1. Radiation Therapy
    2. Chemotherapy
    3. Surgical decompression
  5. Consider Anticoagulation
  6. Intravenous stenting
    1. Common acute management
    2. Improvement is seen within days
    3. Effective even when tumor mass is not reducible

IX. Prognosis

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

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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
Russian VERKHNEI POLOI VENY TROMBOZ, POLOI VERKHNEI VENY SINDROM, VERKHNEI POLOI VENY OBSTRUKTSIIA, ВЕРХНЕЙ ПОЛОЙ ВЕНЫ ОБСТРУКЦИЯ, ВЕРХНЕЙ ПОЛОЙ ВЕНЫ ТРОМБОЗ, ПОЛОЙ ВЕРХНЕЙ ВЕНЫ СИНДРОМ
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: 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