Superior Vena Cava Syndrome. Etiology and management

Superior vena cava (SVC) syndrome is a collection of clinical signs and symptoms resulting from either partial or complete obstruction of blood flow through the SVC.
This obstruction is most commonly a result of thrombus formation or tumor infiltration of the vessel wall.

1. Superior vena cava syndrome(SVCS) PRESENTER: ROMANUS MAPUNDA MSC. ONCOLOGY NURSING

2. Superior vena cava syndrome(SVCS) OUTLINE Introduction Pathophysiology Anatomy Epidemiology Etiology/Risk factors Clinical manifestation Diagnostic work up Treatment Prevention and control approach

3. INTRODUCTION Superior vena cava (SVC) syndrome is a collection of clinical signs and symptoms resulting from either partial or complete obstruction of blood flow through the SVC . This obstruction is most commonly a result of thrombus formation or tumor infiltration of the vessel wall.

4. INTRODUCTION Today , this syndrome is most commonly seen secondary to malignancy, although there has been a more recent rise in benign etiologies . Increased upper body venous pressures results to venous congestion.

5. INTRODUCTION Malignant diseases causing SVCO are Lung cancer (>SCLC and SCC histology cases) which accounts for nearly 85% of all cases F ollowed by Lymphomas mainly NHL 10-15%, with less than 2% occurs in patients with Thymomas and mediastinal Germ cell tumours .

6. INTRODUCTION Non malignancy causes of SVCO includes retrosternal goitre , sarcoidosis , tuberculosis, mediastinal post Irradiation, Idiopathic fibrosis. There is also an increase of SVCO in cancer patents with long term central venous catheters

7. Pathophysiology Mechanisms has divided into three categories which are compromised vessel anatomy, impaired venous flow, and diminished vessel wall integrity (can core exist in PT with SVC syndrome) Extrinsic compression and obstruction of the SVC by a mass in the mediastinum is the most common cause of SVC syndrome

8. Pathophysiology This is often associated with malignancy; however, there are a variety of nonmalignant masses. A growing proportion of SVC syndromes are now associated with occlusive venous thrombus formation that compromises venous flow back to the heart.

9. Pathophysiology The increasing use of indwelling intravascular devices such as catheters and pacemakers leads have played a major role in this growth . Resultant venous wall inflammation, fibrosis, and eventual thrombus lead to stenosis of the vessel itself

10. Anatomy The SVC is the major drainage vessel for venous blood( 2cm and a length of approximately 6-7cm) from the head, neck, upper extremities, and upper thorax. It arises from the union of the left and right brachiocephalic veins, posterior to the first right costal cartilage. It descends vertically through the superior mediastinum, behind the intercostal spaces and to the right of the aorta and trachea. At the level of the second costal cartilage, the SVC enters the middle mediastinum and becomes surrounded by the fibrous pericardium. It terminates by emptying into the superior aspect of the right atrium at the level of the third costal cartilage . It is a thin-walled, low-pressure, vascular structure. This wall is easily compressed as it traverses the right side of the mediastinum.

11. EPIDEMIOLOGY United States statistics The incidence of SVCS within the United States is roughly 15,000 cases per year SVCS develops in 5-10% of patients with a right-side malignant intrathoracic mass lesion. In 1969, Salsali and Cliffton observed SVCS in 4.2% of 4960 patients with lung cancer; In 1987, Armstrong and Perez found SVCS in 1.9% of 952 patients with lymphoma.

12. EPIDEMIOLOGY Age and sex demographics Malignant causes of SVCS are predominantly observed in individuals aged 40-60 years. Benign causes (aged 30-40 years). SVC in the pediatric age group is rare Malignant causes of SVCS are most commonly observed in males ( WHY ) In contrast, cases related to benign causes show no sex-related differences in frequency.

13. ETIOLOGY / RISK FACTORS The most common etiology of SVCS is malignancy. I nfectious causes ( syphilis , tuberculosis, and fungus The most common cause of malignancy-related SVCS is bronchogenic carcinoma, which accounts for nearly 80% of cases. Lymphoma accounts for approximately 15% of cases . dialysis catheters and pacemaker are associated with SVCS. ( WHY )

14. A STUDY

15. Clinical manifestation

16. CLINICAL MANIFESTATION

17. Other less common symptoms of SVC syndrome Stridor Hoarseness Dysphagia P leural effusion H eadache , nausea Lightheadedness Syncope C hange in vision, A ltered mental status U pper body edema Cyanosis and coma Horner’s syndrome, symptoms on one side of face Some rare but serious clinical consequences reported in SVC syndrome include cerebral edema and upper respiratory compromise secondary to edema of the larynx and pharynx.

18. PHYSICAL EXAMINATION The characteristic physical findings of SVCS include venous distention of the neck and chest wall, facial edema, upper-extremity edema, mental changes, cyanosis, papilledema, stupor, and even coma. Bending forward or lying down may aggravate the symptoms and signs.

19. Diagnostic work up Chest X-ray. CT and MRI Invasive contrast venography. Sputum cytology. Thoracentesis. Bronchoscopy. Needle aspiration of a peripheral lymph node, or mediastinoscopy

20. Treatment / MANAGEMENT E levation of the patient’s head as a simple maneuver with the goal of decreasing venous pressure and supplemental oxygen if indicated. For patients with thrombus related to an indwelling intravascular device, removal should be considered along with anticoagulation ( Heparin or Warfarin) therapy and catheter-directed thrombolysis

21. Treatment / MANAGEMENT Emergency treatment is indicated when brain edema, decreased cardiac output, or upper airway edema is present Steroids can be started ( Dexamethasone) Diuretics

22. Treatment / MANAGEMENT Radiation therapy (standard treatment for most patients with SVCS) Dose - 20Gy/ 5 #s or 30Gys/10#s or 37.5Gys/15#s The radiation dose depends on tumor size and radioresponsiveness. The radiation portal should include a 2-cm margin around the tumor.

23. Treatment / MANAGEMENT When SVCS is due to thrombus around a central venous catheter P atients may be treated with thrombolytics or anticoagulants ( eg , heparin or oral anticoagulants). Chemotherapy For chemo sensitive tumors.  

24. Surgical care: Surgical bypass May be a useful way to palliate symptoms( ie , after failure of radiation therapy and chemotherapy) 2. Stenting Percutaneous transluminal angioplasty (PTA), thrombolysis, or some combination  In most patients with SVCS, stenting of the SVC provides rapid symptomatic relief within few days

25. Differential Diagnosis Cardiac tamponade Mediastinitis Thoracic aortic aneurysm Tuberculosis

26. COMPLICATION Complications of SVCS may include the following: Laryngeal edema Cerebral edema Decreased cardiac output with hypotension Pulmonary embolism (when an associated thrombus is present)

27. Nursing care consideration Major considerations in the nursing care of patients with SVCS include R ecognition of high-risk patients F acilitation and coordination of diagnostic procedures A ssessment of respiratory, cardiac and neurologic systems A dministration of therapy P rovision of emotional and psychosocial support, and Patient education.

28. References 1. Azizi AH, Shafi I, Shah N, Rosenfield K, Schainfeld R, Sista A, et al. Superior Vena Cava Syndrome.  JACC Cardiovasc Interv . 2020 Dec 28 . 2. Klein- Weigel PF, Elitok S, Ruttloff A, Reinhold S, Nielitz J, Steindl J, et al. Superior vena cava syndrome.  Vasa . 2020 Oct. 3. Flounders JA. Oncology emergency modules: superior vena cava syndrome.  Oncol Nurs Forum . 2003 Jul-Aug. 4. Hassikou H, Bono W, Bahiri R, Abir S, Benomar M, Hassouni NH. Vascular involvement in Behçet's disease. Two case reports.  Joint Bone Spine . 2002 Jun. 5. Aung EY, Khan M, Williams N, Raja U, Hamady M. Endovascular Stenting in Superior Vena Cava Syndrome: A Systematic Review and Meta-analysis.  Cardiovasc Intervent Radiol . 2022 Sep. 6. Superior Vena Cava Syndrome (2024) https :// emedicine.medscape.com/article/460865-differential 7. Superior Vena Cava Syndrome (2024) https ://www.ncbi.nlm.nih.gov/books/NBK441981 /