Superior vena cava syndrome (SVCS) is a partial or complete obstruction of the blood flow returning to the heart from the head, neck, upper thorax, and upper extremities. Because the superior vena cava (SVC) is located within the narrow space of the mediastinum, any intraluminal or extraluminal compression impairs venous drainage and results in venous congestion, with engorgement of the supplying veins.
As the congestion increases, so does venous pressure behind the compression, and blood may be shunted to adjacent collateral pathways to maintain blood return to the right atrium (Moore, 2005; Haapoja & Blendowski, 1999). The collateral circulation usually bypasses the obstructed SVC through the azygos, internal mammary, thoracic, vertebral, and subcutaneous veins. If any of these vessels are not patent or if the level of compression is below the azygos vein, compensation is challenged, and more acute symptoms result as blood is shunted through the inferior vena cava (Flounders, 2003; Yahalom, 2001; Haapoja & Blendowski, 1999).
Rising venous pressure and venous stasis in the head and upper torso result in third spacing of fluid into adjacent tissue. Fluid accumulation only adds to the vena caval compression and may compress other vital structures in the mediastinum, resulting in pleural or pericardial effusions. If the obstruction of venous blood flow is severe and the collateral circulation pathways are inadequate, cardiac filling and output decline, leading to impaired cerebral perfusion. These sequelae often are accompanied by thrombosis and both laryngeal and cerebral edema, which further place the patient at risk for acute respiratory, cardiovascular, and neurologic distress (Moore, 2005; Flounders, 2003; Myers, 2001; Haapoja & Blendowski, 1999).
EPIDEMIOLOGY AND ETIOLOGY
As an oncologic emergency, SVCS is relatively rare, affecting only 3% to 4% of cancer patients. Three basic types of occlusion can result in SVCS: (1) extraluminal compression caused by a mass, metastases, fibrosis, or one or more enlarged lymph nodes; (2) intraluminal obstruction caused by a mass invading the vessel wall or thrombosis; and (3) intraluminal response to infection or inflammation (Miaskowski, 1999; Hogan & Rosenthal, 1998; Joyce & Cunningham, 1998).
Approximately 15% to 22% of all cases of SVCS are associated with benign conditions, primarily infection or thrombosis; cancer accounts for 78% to 85% of SVCS cases (Drews, 2006). Lung cancer and lymphoma, especially with right-side perihilar adenopathy, account for most cancer-related cases of SVCS. Small cell and squamous cell lung cancers are the most common histological types, because they tend to develop centrally in the pulmonary bed (Drews, 2006; Hogan & Rosenthal, 1998). Because of the right lung’s proximity to the SVC, right-side lung cancer is four times more likely to be associated with SVCS than left-side lung cancer. Other cancers that may be associated with SVCS include Kaposi’s sarcoma, thymoma, germ cell tumors, and cancers in which nodal mediastinal metastasis is a potential risk (Drews, 2006; Tyson, 2004; Flounders, 2003; Joyce & Cunningham, 1998).
RISK PROFILE
• Age and gender (Haapoja & Blendowski, 1999):
• Average age is 40 to 70 years.
• SVCS is three times more to likely to occur in men.
• Benign conditions associated with SVCS (Drews, 2006; Krimsky et al., 2002; Miaskowski, 1999):
• Aortic aneurysm
• Infections
• Involving the structures in the mediastinum (i.e., mediastinitis, tuberculosis, actinomycosis, aspergillosis, and blastomycosis).
• Resulting from contagious spread from a lung, pleural, or skin infection (i.e., nocardiosis).
• Thyroid disorders (i.e., goiter).
• Sclerosing cholangitis.
• Sarcoidosis
• Vascular fibrosis after thoracic radiation.
• Central venous catheters or tunneled venous ports in patients with the following conditions (Rice et al., 2006; Moore, 2005; Tyson, 2004; Flounders, 2003; Haapoja & Blendowski, 1999):
• Hypercoagulable state.
• Damage to the intima of the SVC by the venous catheter.
• Pacemaker
• Venous stasis from extraluminal compression of the SVC.
• Inadequate flushing of the venous catheter or port.
• Malignant causes of SVCS (Drews, 2006; Flounders, 2003; Lonardi et al., 2002; Yahalom, 2001; Haapoja & Blendowski, 1999):
• Most common in men age 50 to 70 years with primary or metastatic tumors involving the mediastinum.
• Lung cancer:
• Small cell lung cancer (SCLC) is more common than non-small cell lung cancer (NSCLC).
• Non-small cell lung cancer of squamous cell histology
• Non-Hodgkin’s lymphoma:
• Diffuse large cell type (i.e., primarily large cell lymphoma with sclerosing).
• Lymphoblastic type.
• Metastases to the mediastinum from primary cancers (Joyce & Cunningham, 1998):
• Breast cancer
• Esophageal cancer
• Colon cancer
• Testicular cancer
• Kaposi’s sarcoma
• Thymoma
• Germ cell cancer
PROGNOSIS
Although SVCS can be accompanied by distressing symptoms, it rarely presents as a life-threatening emergency (Tyson, 2004). Most patients usually respond to treatment for SVCS, with relief of symptoms or tumor regression if cancer is present. Death from venous obstruction alone has not been described in the medical literature. Mortality comes from concurrent or advanced stages of disease, such as cancer in the mediastinum (Bierdrager, et al., 2005; Myers, 2001; Haapoja & Blendowski, 1999).
The prognosis depends on how rapidly the obstruction develops the degree of vena caval blockage, and compensation by collateral circulation. If venous return can be maintained and hypoxia prevented, heart failure and organ damage can be prevented even with acute onset. The overall prognosis also depends on the underlying disease and its histology, if malignant. Benign causes can be easily treated, and these patients have a favorable prognosis; likewise for patients with lymphoma and small-cell lung cancer. Patients with mediastinal metastases have limited long-term response. Although early diagnosis and treatment of SVCS may not bring long-term survival, quality of life can be improved with palliation (Drews, 2006; Moore, 2005; Lonardi et al., 2002; Myers, 2001; Yahalom, 2001).
PROFESSIONAL ASSESSMENT CRITERIA (PAC)
The range and severity of symptoms in SVCS depend on how rapidly the obstruction develops, its underlying cause, its location and whether it extends into the mediastinum, the presence of laryngeal edema, the availability of collateral circulation, and the amount of venous hypertension (Drews, 2006; Moore, 2005; Flounders, 2003; Yahalom, 2001; Haapoja & Blendowski, 1999).
Symptoms can arise suddenly or gradually. Because the course of SVCS can vary greatly from one patient to another, identifying a baseline assessment for each individual is important. Early recognition prompts early treatment and can often prevent symptoms from progressing to life-threatening airway obstruction, congestive heart failure, and cerebral hypoxia (Flounders, 2003; Haapoja & Blendowski, 1999).
Although debate persists about which symptoms present early in SVCS, the cluster of hallmark symptoms include dyspnea, facial edema or head fullness, cough, upper extremity edema, and upper torso vein engorgement. Most signs and symptoms worsen when the patient bends over or is in the supine position. Some patients may experience symptoms of SVCS only in the morning after rising (Drews, 2006; Moore, 2005; Flounders, 2003: Myers, 2001; Haapoja & Blendowski, 1999). Table 46-1 presents a more detailed list of some of the clinical signs and symptoms, and Table 46-2 lists the diagnostic and differential tests for SVCS.
| System | Subjective Assessment | Objective Assessment |
|---|---|---|
| General | C/O hoarseness, chest pain, pain at site of goiter or thrombosis | Enlarged, nontender lymph nodes |
| Skin | C/O ruddy complexion | Plethora (facial erythema) or skin tone change in upper torso, reddish palms and mucous membranes, “looks healthier” with fewer visible age lines and wrinkles |
| HEENT | C/O headache, visual changes, mood changes, difficulty swallowing, nasal stuffiness | Mood or behavior changes, enlarged lymph nodes in the neck, periorbital edema, edema of the conjunctivae |
| Respiratory | C/O dyspnea, “catching” breath, nonproductive cough, breathing hard or fast, fatigue, throat spasms | Acute dyspnea, SOB, stridor, crackles on auscultation, tachypnea, decreased lung expansion, dyspnea on exertion, decreased pulse oximetry level, cyanosis (nail bed first), paroxysmal nocturnal dyspnea (increased dyspnea when lying down), laryngospasms and sternal retractions on inspiration, right-side pleural effusion |
| Cardiovascular | C/O palpitations, dizziness, Stoke’s sign (tight collar, unable to button shirt at neck), neck and/or head fullness, rings on hands feel tight, ear fullness, chest or breast edema, orthopnea | Acute tachycardia; bounding pulse; initial hypertension then hypotension; possibly orthostatic blood pressures; BP may be higher in upper extremities than in lower extremities; increased jugular venous distension/pressure; edema in the face, neck, chest, and/or arms; early morning periorbital edema; decreased neck flexibility; epistaxis; difficulty with peripheral venipuncture; hand veins do not collapse when the hands are raised over the head; frequent IV infiltrations on right side |
| GI | C/O dysphagia, poor appetite, nausea, heartburn | Weight gain over 1 to 2 weeks |
| GU | C/O “not voiding much” | Urine output <30 mL/hr |
| Musculoskeletal | C/O upper body aches, stiffness, weakness, fatigue | Decreased range of motion in upper extremities, decreased ability to perform ADLs |
| Neurologic | Family reports intermittent confusion, C/O decreased ability to concentrate, headache, lethargy, visual changes | Mood swings, delirium, altered cognition, altered level of consciousness (can progress to stupor and coma), altered vision, Horner’s syndrome (unilateral ptosis with constricted pupil), seizures, death |
| Psychosocial | C/O restlessness, apprehension, anxiety, premonition of doom, irritability | Visibly restless, anxious |
| Test | Risk | Findings |
|---|---|---|
| CBC with diff | Minimal | Monitor for anemia or infection. WBC count may be elevated. Hgb and Hct may be low. |
| PT/PTT/INR | Minimal | Monitor for clotting times, pulmonary embolism, hypercoagulable state, DIC. Results may be elevated. Obtain baseline data for possible thrombolytic therapy. |
| Fibrin split products | Minimal | Assess for s/s of DVT, DIC, PE. May be positive test. Obtain baseline data. |
| Fibrinogen | Minimal | Monitor for DVT, DIC, PE. Obtain baseline data. |
| Chemistries | Minimal | Monitor for hypovolemia. BUN/creatinine may be elevated. Obtain baseline values for renal function. Monitor for hyponatremia, hypokalemia, hypo magnesia, hypokalemia. Obtain baseline data. |
| ABGs | Minimal | Monitor for degree of respiratory compromise. May indicate poor perfusion. Obtain baseline data. |
| Biopsy | Moderate to high risk, depending on degree of respiratory compromise and risk of bleeding | Obtain and assess biopsy results if a mass is present; may indicate cancer diagnosis. |
| ECG | Minimal | Monitor rhythms for tachycardia and atrial fibrillation or myocardial injury/ischemia. Obtain baseline data. |
| CT scan | Minimal | Differentiates internal or external compression; identifies mass and surrounding structures; localizes tumor area for biopsy. |
| CXR | Minimal | Identifies presence of mediastinal mass, if SVC is caused by a tumor or lymphadenopathy. Normal CXR may indicate cause is thrombus or radiation fibrosis. |
| CT-guided biopsy | Moderate to high risk, depending on degree of respiratory compromise and risk of bleeding | Obtain to evaluate the cancer diagnosis (histologic diagnosis). |
| MRI | Minimal | Multidimensional image of the vena cava and source of obstruction; noninvasive tool to identify vascular pathways and mediastinal structures. |
| Contrast venography | Moderate, depending on the risk of bleeding | Detects compromise in the circulation and collateral venous development. |
NURSING CARE AND TREATMENT
SVCS has many potential causes, therefore identification of its underlying etiology is important in directing treatment, especially because the syndrome may be the first sign of disease (Wudel & Nesbitt, 2001; Yahalom, 2001).
1. Vital signs: Assess for hypertension, tachycardia, tachypnea, and/or fever. Monitor every 15 minutes if the patient is in acute distress.
3. Obtain and interpret O2 saturation (signs of hypoxia include restlessness, dyspnea, anxiety, and cyanosis) and ABGs (hypoxia, acidosis).
4. Measure dyspnea on a 0 to 10 scale (0 = no dyspnea and 10 = severe dyspnea) at least every shift unless the patient is in acute distress.
5. Administer oxygen to treat hypoxia. Suction as needed for difficulty handling secretions. Have airway at bedside.
6. Assess for any laryngeal or tracheal edema, hoarseness, difficulty swallowing, stridor, headache, visual changes, changes in mood or orientation, delirium, palpitations, dizziness, epistaxis, nonproductive cough, crackles, adventitious breath sounds, decreased chest expansion, neck and chest vein engorgement, and edema (face, neck, upper torso, upper extremities).
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