Superior Vena Cava Syndrome 

  • Author: Todd A Nickloes, DO, FACOS; Chief Editor: Vincent Lopez Rowe, MD   more...
 
Updated: Aug 13, 2010
 

Background

Superior vena cava syndrome (SVCS) is obstruction of blood flow through the superior vena cava (SVC). It is a medical emergency and most often manifests in patients with a malignant disease process within the thorax. A patient with superior vena cava syndrome (SVCS) requires immediate diagnostic evaluation and therapy. See the images below.

Superior vena cava syndrome (case 1). The patient Superior vena cava syndrome (case 1). The patient was a 35-year-old man with a 3-year history of progressive upper-extremity and fascial swelling. The patient had undergone treatment for histoplasmosis in the past. CT scan shows a narrowed superior vena cava with adjacent calcified lymph nodes and posterior soft tissue thickening. Superior vena cava syndrome (case 1, cont'd). SonoSuperior vena cava syndrome (case 1, cont'd). Sonogram shows markedly damped venous waveform with complete loss of normal venous pulsatility and minimal respiratory variation.

William Hunter first described the syndrome in 1757 in a patient with syphilitic aortic aneurysm.[1] In 1954, Schechter reviewed 274 well-documented cases of superior vena cava syndrome (SVCS) reported in the literature; 40% of them were due to syphilitic aneurysms or tuberculous mediastinitis.[2] In more recent times, these infections have gradually decreased as the primary cause of superior vena cava (SVC) obstruction. Lung cancer, particularly adenocarcinoma, is now the underlying process in approximately 70% of the patients with superior vena cava syndrome (SVCS).[3, 4, 5] However, up to 40% of the causes are due to nonmalignant causes.[6]

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Pathophysiology

The superior vena cava (SVC) is the major drainage vessel for venous blood from the head, neck, upper extremities, and upper thorax. It is located in the middle mediastinum and is surrounded by relatively rigid structures such as the sternum, trachea, right bronchus, aorta, pulmonary artery, and the perihilar and paratracheal lymph nodes. It extends from the junction of the right and left innominate veins to the right atrium, a distance of 6-8 cm. It is a thin-walled, low-pressure, vascular structure. This wall is easily compressed as it traverses the right side of the mediastinum.[7]

Obstruction of the superior vena cava (SVC) may be caused by neoplastic invasion of the venous wall associated with intravascular thrombosis or, more simply, by extrinsic pressure of a tumor mass against the relatively fixed thin-walled superior vena cava (SVC). Postmortem examinations reveal that complete superior vena cava (SVC) obstruction is the result of intravascular thrombosis in combination with extrinsic pressure. Incomplete superior vena cava (SVC) obstruction is more often secondary to extrinsic compression without thrombosis. Other causes include compression by intravascular arterial devices. The incidence is on the rise in line with the increased usage of endovascular devices.[4]

An obstructed superior vena cava (SVC) initiates collateral venous return to the heart from the upper half of the body through 4 principal pathways. The first and most important pathway is the azygous venous system, which includes the azygous vein, the hemiazygous vein, and the connecting intercostal veins. The second pathway is the internal mammary venous system plus tributaries and secondary communications to the superior and inferior epigastric veins. The long thoracic venous system, with its connections to the femoral veins and vertebral veins, provides the third and fourth collateral routes, respectively.

Despite these collateral pathways, venous pressure is almost always elevated in the upper compartment if obstruction of the superior vena cava (SVC) is present. Venous pressure as high as 200-500 cm H2 O has been recorded in patients with severe superior vena cava syndrome (SVCS).

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Epidemiology

Frequency

United States

Superior vena cava syndrome (SVCS) develops in 5-10% of patients with a right-sided malignant intrathoracic mass lesion. In 1969, Salsali and Cliffton observed superior vena cava syndrome (SVCS) in 4.2% of 4960 patients with lung cancer; 80% of the tumors inducing superior vena cava syndrome (SVCS) were of the right lung.[8] In 5 large series of small cell lung cancer, 9-19% of patients demonstrated superior vena cava syndrome (SVCS). In 1987, Armstrong and Perez found superior vena cava syndrome (SVCS) in 1.9% of 952 patients with lymphoma.[9]

Mortality/Morbidity

  • Survival in patients with superior vena cava syndrome (SVCS) depends mainly on the course of the underlying disease. No mortality, per se, results directly from mild venous congestion.
  • In patients with benign superior vena cava syndrome (SVCS), life expectancy is unchanged.
  • If superior vena cava syndrome (SVCS) is secondary to a malignant process, patient survival correlates with the histology of the tumor. Patients with signs and symptoms of laryngeal and cerebral edema have the most life-threatening manifestations of this syndrome and are in danger of sudden death. Clinical observations show that approximately 10% of patients with a bronchogenic carcinoma and 45% of patients with lymphoma treated with irradiation live at least 30 months. In contrast, patients with untreated malignant superior vena cava syndrome (SVCS) survive only approximately 30 days.[5]

Race

The frequency of superior vena cava syndrome (SVCS) in different races depends largely on the frequency of lung cancer and lymphomas in these populations.

Sex

  • Malignant causes of superior vena cava syndrome (SVCS) are most commonly observed in males because of the high incidence of lung cancer in this population.
  • In contrast, no sex difference is observed in cases related to benign causes.

Age

  • Malignant causes of superior vena cava syndrome (SVCS) are predominantly observed in individuals aged 40-60 years.
  • Benign causes account for most of the cases diagnosed in individuals aged 30-40 years.
  • Obstruction of the superior vena cava (SVC) in the pediatric age group is rare and has a different etiologic spectrum.
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Contributor Information and Disclosures
Author

Todd A Nickloes, DO, FACOS  Assistant Professor, Department of Surgery, Division of Trauma/Critical Care, University of Tennessee Medical Center-Knoxville

Todd A Nickloes, DO, FACOS is a member of the following medical societies: American College of Osteopathic Surgeons, American Medical Association, American Osteopathic Association, Association for Academic Surgery, Eastern Association for the Surgery of Trauma, Society of Critical Care Medicine, Society of Laparoendoscopic Surgeons, Southeastern Surgical Congress, and Southern Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

LaMar O Mack, MD  Resident Physician, Department of Surgery, University of Tennessee Medical Center

LaMar O Mack, MD is a member of the following medical societies: American Urological Association, National Medical Association, and Student National Medical Association

Disclosure: Nothing to disclose.

Andre M Kallab, MD  Clinical Associate Professor of Oncology, Medical College of Georgia; Consulting Staff, Department of Oncology, Northeast Georgia Diagnostic Clinic

Andre M Kallab, MD is a member of the following medical societies: American College of Physicians, American Medical Association, and American Society of Hematology

Disclosure: Nothing to disclose.

Allan Bernard Dunlap, MD  Fellow in Trauma/Surgical Critical Care, University of Tennessee Health Science Center College of Medicine

Allan Bernard Dunlap, MD is a member of the following medical societies: American College of Surgeons and Society for Vascular Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard M Stillman†, MD, FACS  Honorary Medical Staff, Northwest Medical Center; Former Chief of Staff and Medical Director, Wound Healing Center, Department of Surgery, Northwest Medical Center

Richard M Stillman†, MD, FACS is a member of the following medical societies: American College of Angiology, American College of Surgeons, Association for Academic Surgery, and Society of University Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Vincent Lopez Rowe, MD  Associate Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

Paolo Zamboni, MD  Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Vincent Lopez Rowe, MD  Associate Professor of Surgery, Department of Surgery, Division of Vascular Surgery, University of Southern California Medical Center

Vincent Lopez Rowe, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, Pacific Coast Surgical Association, Peripheral Vascular Surgery Society, Society for Clinical Vascular Surgery, Society for Vascular Surgery, and Western Vascular Surgical Society

Disclosure: Nothing to disclose.

References

  1. National Cancer Institute. Cardiopulmonary syndromes. 2005;[Full Text].

  2. Schechter MM. The superior vena cava syndrome. Am J Med Sci. Jan 1954;227(1):46-56. [Medline].

  3. Flounders J. Superor vena cava syndrome. Oncol Nurs Forum. 2003;30(4):E84-8.

  4. Ahmann FR. A reassessment of the clinical implications of the superior vena caval syndrome. J Clin Oncol. Aug 1984;2(8):961-9. [Medline].

  5. Hassikou H, Bono W, Bahiri R, et al. Vascular involvement in Behçet's disease. Two case reports. Joint Bone Spine. Jun 2002;69(4):416-8. [Medline].

  6. Hunter W. The history of an aneurysm of the aorta with some remarks on aneurysms in general. Med Obs Enq. 1757;1:323-357.

  7. Armstrong BA, Perez CA, Simpson JR, et al. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys. Apr 1987;13(4):531-9. [Medline].

  8. Houman M, Ksontini I, Ben Ghorbel I, et al. Association of right heart thrombosis, endomyocardial fibrosis, and pulmonary artery aneurysm in Behçet's disease. Eur J Intern Med. Oct 2002;13(7):455. [Medline].

  9. Salsali M, Cliffton EE. Superior vena caval obstruction in carcinoma of lung. N Y State J Med. Nov 15 1969;69(22):2875-80. [Medline].

  10. Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore). Jan 2006;85(1):37-42. [Medline].

  11. Blalock A, Cunningham RS, Robinson CS. Experimental production of chylothorax by occlusion of the superior vena cava. Ann Surg. Sep 1936;104(3):359-64. [Medline].

  12. Escalante CP. Causes and management of superior vena cava syndrome. Oncology (Williston Park). Jun 1993;7(6):61-8; discussion 71-2, 75-7. [Medline].

  13. Schraufnagel DE, Hill R, Leech JA, et al. Superior vena caval obstruction. Is it a medical emergency?. Am J Med. Jun 1981;70(6):1169-74. [Medline].

  14. Klassen KP, Andrews NC, Curtis GM. Diagnosis and treatment of superior-vena-cava obstruction. AMA Arch Surg. Sep 1951;63(3):311-25. [Medline].

  15. Sakura M, Tsujii T, Yamauchi A, et al. Superior vena cava syndrome caused by supraclavicular lymph node metastasis of renal cell carcinoma. Int J Clin Oncol. Oct 2007;12(5):382-4. [Medline].

  16. Nieto AF, Doty DB. Superior vena cava obstruction: clinical syndrome, etiology, and treatment. Curr Probl Cancer. Sep 1986;10(9):441-84. [Medline].

  17. Lochridge SK, Knibbe WP, Doty DB. Obstruction of the superior vena cava. Surgery. Jan 1979;85(1):14-24. [Medline].

  18. Bigsby R, Greengrass R, Unruh H. Diagnostic algorithm for acute superior vena caval obstruction (SVCO). J Cardiovasc Surg (Torino). Aug 1993;34(4):347-50. [Medline].

  19. Parish JM, Marschke RF Jr, Dines DE, et al. Etiologic considerations in superior vena cava syndrome. Mayo Clin Proc. Jul 1981;56(7):407-13. [Medline].

  20. Marcy PY, Magne N, Bentolila F, et al. Superior vena cava obstruction: is stenting necessary?. Support Care Cancer. Mar 2001;9(2):103-7. [Medline].

  21. Akoglu H, Yilmaz R, Peynircioglu B, et al. A rare complication of hemodialysis catheters: superior vena cava syndrome. Hemodial Int. Oct 2007;11(4):385-91. [Medline].

  22. Scarantino C, Salazar OM, Rubin P, et al. The optimum radiation schedule in treatment of superior vena caval obstruction: importance of 99mTc scintiangiograms. Int J Radiat Oncol Biol Phys. Nov-Dec 1979;5(11-12):1987-95. [Medline].

  23. Abner A. Approach to the patient who presents with superior vena cava obstruction. Chest. Apr 1993;103(4 Suppl):394S-397S. [Medline].

  24. Dosios T, Theakos N, Chatziantoniou C. Cervical mediastinoscopy and anterior mediastinotomy in superior vena cava obstruction. Chest. Sep 2005;128(3):1551-6. [Medline].

  25. Rowell NP, Gleeson FV. Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus: a systematic review. Clin Oncol (R Coll Radiol). Oct 2002;14(5):338-51. [Medline].

  26. Urban T, Lebeau B, Chastang C, et al. Superior vena cava syndrome in small-cell lung cancer. Arch Intern Med. Feb 8 1993;153(3):384-7. [Medline].

  27. Maddox AM, Valdivieso M, Lukeman J, et al. Superior vena cava obstruction in small cell bronchogenic carcinoma. Clinical parameters and survival. Cancer. Dec 1 1983;52(11):2165-72. [Medline].

  28. Perez-Soler R, McLaughlin P, Velasquez WS, et al. Clinical features and results of management of superior vena cava syndrome secondary to lymphoma. J Clin Oncol. Apr 1984;2(4):260-6. [Medline].

  29. Adelstein DJ, Hines JD, Carter SG, et al. Thromboembolic events in patients with malignant superior vena cava syndrome and the role of anticoagulation. Cancer. Nov 15 1988;62(10):2258-62. [Medline].

  30. Watkinson AF, Yeow TN, Fraser C. Endovascular stenting to treat obstruction of the superior vena cava. BMJ. Jun 21 2008;336(7658):1434-7. [Medline].

  31. Garcia Monaco R, Bertoni H, Pallota G, et al. Use of self-expanding vascular endoprostheses in superior vena cava syndrome. Eur J Cardiothorac Surg. Aug 2003;24(2):208-11. [Medline].

  32. Smayra T, Otal P, Chabbert V, et al. Long-term results of endovascular stent placement in the superior caval venous system. Cardiovasc Intervent Radiol. Nov-Dec 2001;24(6):388-94. [Medline].

  33. Thony F, Moro D, Witmeyer P, et al. Endovascular treatment of superior vena cava obstruction in patients with malignancies. Eur Radiol. 1999;9(5):965-71. [Medline].

  34. Yim CD, Sane SS, Bjarnason H. Superior vena cava stenting. Radiol Clin North Am. Mar 2000;38(2):409-24. [Medline].

  35. Garlitski AC, Swingle JD, Aizer A, et al. Percutaneous treatment of the superior vena cava syndrome via an excimer laser sheath in a patient with a single chamber atrial pacemaker. J Interv Card Electrophysiol. Sep 2006;16(3):203-6. [Medline].

  36. Nunnelee JD. Superior vena cava syndrome. J Vasc Nurs. Mar 2007;25(1):2-5; quiz 6. [Medline].

  37. Rice TW. Pleural effusions in superior vena cava syndrome: prevalence, characteristics, and proposed pathophysiology. Curr Opin Pulm Med. Jul 2007;13(4):324-7. [Medline].

  38. Vogelzang R, Schindler N. Superor vena cava syndrome: endovascular therapy. In: Gloviczki P, Yoa J, eds. Handbook of venous disorders. (2nd ed). London: Arnold; 2001:401-7.

 
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Superior vena cava syndrome (case 1). The patient was a 35-year-old man with a 3-year history of progressive upper-extremity and fascial swelling. The patient had undergone treatment for histoplasmosis in the past. CT scan shows a narrowed superior vena cava with adjacent calcified lymph nodes and posterior soft tissue thickening.
Superior vena cava syndrome (case 1, cont'd). Sonogram shows markedly damped venous waveform with complete loss of normal venous pulsatility and minimal respiratory variation.
Superior vena cava syndrome (case 1, cont'd). Venogram shows almost complete occlusion of the superior vena cava with dramatic collateral drainage through the left superior intercostal vein.
Superior vena cava syndrome (case 1, cont'd). A Palmaz P308 stent mounted on a 12-mm balloon was deployed in the superior vena cava after it was predilated to 8 mm. The stent was subsequently dilated to 14 mm.
Superior vena cava syndrome (case 1, cont'd). Venogram obtained after stenting shows a widely patent superior vena cava with no collateral drainage. Pressure measurements after stenting showed a 1- to 2-mm residual gradient.
Superior vena cava syndrome (case 1, cont'd). Sonogram obtained 1 year after stenting shows near-normal venous pulsatility and respiratory phasicity. The patient experienced a complete resolution of symptoms.
 
 
 
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