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Clinical Investigations: PULMONARY EMBOLISM |

Thromboembolic Disease Involving the Superior Vena Cava and Brachiocephalic Veins* FREE TO VIEW

Todd R. Otten, MD; Paul D. Stein, MD, FCCP; Kalpesh C. Patel, MD; Syed Mustafa, MD; Allen Silbergleit, MD, PhD
Author and Funding Information

*From St. Joseph Mercy Oakland, Pontiac, MI.

Correspondence to: Paul D. Stein, MD, FCCP, St. Joseph Mercy Oakland, 44555 Woodward Ave, Suite 107, Pontiac, MI 48341-2985; e-mail: steinp@trinity-health.org



Chest. 2003;123(3):809-812. doi:10.1378/chest.123.3.809
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Published online

Purpose: To evaluate the frequency of diagnosis and the characteristics of brachiocephalic vein and superior vena cava (SVC) thromboembolic disease.

Background: Thromboembolic disease of the brachiocephalic veins or SVC rarely has been reported. In view of the frequent use of central venous access lines, it would seem that the percentage of hospitalized patients with thromboembolic disease of the brachiocephalic veins or SVC should be higher than is generally recognized.

Methods: A retrospective search for thromboembolic disease involving the brachiocephalic veins and SVC was made of patients who were hospitalized over a 2-year period.

Results: Thromboembolic disease of the brachiocephalic veins or SVC was diagnosed in 23 of 34,567 hospitalized adults (0.06%) who were ≥ 20 years old. Two of 23 patients (8.7%) had pulmonary embolism. Cancer was present in 17 of 23 patients (74%), and 15 of 23 patients (65%) had central venous access lines. Edema of the arm, face, or neck was present in 21 of 23 patients (91%). Pain or discomfort was present 15 of 23 patients (65%).

Conclusion: Isolated brachiocephalic vein and SVC thrombosis occur in a sufficient number of hospitalized patients to merit consideration of the diagnosis in patients who have cancer, central venous access lines, or both. The signs and symptoms of brachiocephalic vein thrombosis have features in common with SVC syndrome as well as with upper extremity deep venous thrombosis. In a patient with appropriate clinical findings, venography or other imaging may be indicated.

Thrombosis of the superior vena cava (SVC) is an uncommon cause of SVC syndrome.1Rarely, pulmonary embolism (PE) with the SVC syndrome has been observed at autopsy.23 We are aware of only two reported cases in living patients.45 In addition, we are aware of only one case of PE due to brachiocephalic vein thrombosis.6In view of the frequent use of central venous access lines, and the known association of such instrumentation with thrombosis,79 it would seem that thrombotic involvement of the brachiocephalic veins and SVC should be diagnosed more frequently than is generally reported. Therefore, we evaluated the frequency of diagnosis and the characteristics of thromboembolic disease involving the brachiocephalic vein and SVC in a community/teaching hospital to test the hypothesis that such disease merits consideration, particularly in patients with cancer and in those with central venous access lines.

We used two sources for the identification of patients with thrombosis of the brachiocephalic (innominate) veins or SVC at St. Joseph Mercy Oakland (Pontiac, MI) during a 2-year period from July 1, 1998, through June 30, 2000. One source of the identification of patients was hospital discharge diagnostic codes using the International Classification of Disease, ninth revision, clinical modification (ICD-9-CM) diagnostic codes.10 The other was computer-generated lists of patients who had undergone contrast venography, contrast-enhanced spiral CT scanning, and magnetic resonance angiography of the upper extremity and SVC.

A computer search was made of all patients who had ICD-9-CM discharge diagnostic codes 453.2 (venous embolism and thrombosis of the vena cava), 453.8 (venous thrombosis of other specified sites), 453.9 (venous thrombosis of unspecified sites), and 444.21 (arterial embolism and thrombosis of upper extremity). Confirmation of the diagnosis of thrombosis of the brachiocephalic veins or SVC was made by reviewing the medical records of each patient. A report of a diagnostic venogram, spiral CT scan, or magnetic resonance angiogram was required for inclusion in the study.

We searched computer-generated lists of all patients who had undergone imaging of the upper extremity or SVC. This included contrast venography (194 patients), spiral CT scanning (285 patients), and magnetic resonance angiography (11 patients). The reports of each patient were reviewed, and positive cases were included.

We searched for coincident upper extremity deep venous thrombosis (DVT). Patients were identified by using the ICD-9-CM discharge codes 451.83 (phlebitis and thrombophlebitis of deep veins of upper extremities [brachial, radial, and ulnar veins]), 451.84 (phlebitis and thrombophlebitis of upper extremities unspecified), and 451.89 (phlebitis and thrombophlebitis other [axillary, jugular, subclavian, and breast]). The results of computer-generated lists of compression ultrasound, venography, and magnetic resonance angiography of the distal upper extremity also were evaluated. An objective verification of the diagnosis was required in all patients.

We searched for coincident DVT of the lower extremities. All patients with a hospital discharge diagnosis of DVT based on the following codes were evaluated: 451 (phlebitis and thrombophlebitis); 451.0 (femoropopliteal vein and saphenous vein); 451.1 (deep vessels of lower extremities); 451.11 (femoral vein deep); 451.19 (popliteal vein, tibial vein, and other); 451.2 (lower extremities); 451.8 (other sites); 451.81 (iliac vein); 453.0 (hepatic vein thrombosis); 453.1 (thrombophlebitis migrans); 453.2 (vena cava); 453.8 (other specified sites); 671.3 (DVT antepartum); and 671.4 (DVT postpartum). All diagnoses of DVT of the lower extremities were confirmed by venous ultrasound.

The frequency of diagnosing PE during this period at the same hospital has been reported, and the method of identification of PE has been described.11 We used this database to determine whether any of the patients with brachiocephalic vein thrombosis or SVC thrombosis had PE.

St. Joseph Mercy Oakland is a general hospital with a published sample census of 269 patients.12 It is designated by the American College of Surgeons as a teaching hospital category cancer center. It is also a trauma center, but it is not a burn center.

Venous thromboembolic disease of the brachiocephalic veins and SVC in hospitalized adults who were ≥ 20 years old was diagnosed in 23 of 34,567 patients (0.06%). The patients ranged in age from 37 to 80 years. Seven patients (30%) were men. The diagnosis was made by contrast venography in 21 patients, and by contrast-enhanced spiral CT scanning in 2 patients.

Brachiocephalic vein thrombosis was observed in 22 patients, and SVC thrombosis was observed in 6 patients. The number of patients with brachiocephalic vein thrombosis alone or in combination with SVC thrombosis or with thrombosis of the subclavian or axillary veins is shown in Table 1 . All six patients with SVC thrombosis showed incomplete obstruction of the SVC. Among the 22 patients with brachiocephalic vein involvement, 13 showed total occlusion or occlusion sufficient to produce collateral veins.

Two of 23 patients (8.7%) had nonfatal PEs, both of which were diagnosed by high-probability ventilation-perfusion lung scans. One patient had brachiocephalic vein thrombosis and, in addition, bilateral thrombosis of the axillary, subclavian, and internal jugular veins. The PE occurred before the venous thrombosis was diagnosed and treated. The other patient had brachiocephalic vein thrombosis with no other upper body DVT, but in addition had proximal lower extremity DVT. The PE occurred while the patient was receiving treatment for lower extremity DVT. This was the only patient with coincident DVT of the lower extremities.

Predisposing factors are shown in Table 2 . Among patients who had central venous access lines, 10 had Groshong catheters, 4 had Infusaports, and 1 had a peripherally inserted central catheter (all from Bard Access Systems; Salt Lake City, UT).

Three patients with brachiocephalic vein thrombosis had neither cancer nor central venous lines, but they had arteriovenous shunts for renal dialysis. There was no evidence of thrombosis of the shunts. One of these patients had subclavian vein thrombosis in addition to thrombosis of the brachiocephalic vein. The other two patients had no evidence of thrombosis at other sites. The patient who had SVC thrombosis alone had no clear cause for the thrombosis. She was taking estrogen replacement therapy following menopause.

The signs and symptoms observed in all patients, and in nine patients who showed involvement only of a brachiocephalic vein, are given in Table 3 . In the latter group, the thrombosis caused partial occlusion in six of nine patients (67%). Three of those patients with partial occlusions showed collateral vessels.

Twenty-three patients with thrombosis of a brachiocephalic vein or SVC were identified. Two patients had objectively diagnosed symptomatic PE (8.7%). The source of the PE in one patient, however, may have been from proximal DVT of the lower extremity.

Thrombosis of the SVC is a recognized cause of the SVC syndrome.1,1314 The association of clinically recognized PE with SVC thrombosis is limited to case reports45 and occasional patients observed at autopsy.23 It was suggested, however, that SVC thrombosis may pose a significant risk for PE.15 Brachiocephalic vein thrombosis has been identified in a case report.6 That patient also had PE.

Whether the frequency of diagnosing brachiocephalic vein thrombosis or SVC thrombosis that we observed among hospitalized patients is representative of other hospitals is uncertain. There is no literature with which to compare them. However, the frequency of diagnosing PE from all sources of DVT at this hospital was well within the range reported elsewhere.11

The frequency of isolated thrombosis of the SVC or brachiocephalic veins that we report probably grossly underestimates the true incidence, because the test that is usually performed in symptomatic patients, color duplex Doppler ultrasonography, cannot image the SVC and proximal segment of the brachiocephalic veins.1617 Contrast venography is the most conclusive test.16,1821 Helical CT phlebography,22 magnetic resonance angiography,2021 and gadolinium-enhanced magnetic resonance venography23 may be useful. If a patient has upper extremity signs and symptoms (eg, arm edema and discomfort) and has had a central venous access line and/or cancer, one of these diagnostic tests may be indicated if the ultrasonography finding is negative.

As the use of a central venous line has increased, its role in the etiology of SVC thrombosis3,8,2425 and in thrombosis of the great veins of the thorax9 became apparent. In our study, 65% of the patients had central venous access lines. An association of malignancy with thrombosis is also well-established.26Among our patients, 74% had malignancies. A hypercoagulable state also may be associated with thrombosis,27 but our patients generally were not evaluated for this. A patent arteriovenous shunt was present in three patients with thrombosis of a brachiocephalic vein. Whether the arteriovenous shunt contributed to the thrombosis is unclear.

The key features that we observed are that isolated brachiocephalic vein thrombosis and SVC thrombosis are not rare. It seems that PE may result, but the patients with PE that we observed also had upper extremity DVT or proximal lower extremity DVT. The signs and symptoms of brachiocephalic vein thrombosis have features in common with the SVC syndrome as well as upper extremity DVT. The etiology is usually malignancy, a central venous access line, or a combination of the two. In a patient with appropriate clinical findings, evaluation by venography, contrast-enhanced CT scanning, or magnetic resonance angiography may be indicated.

Abbreviations: DVT = deep venous thrombosis; ICD-9-CM = International Classification of Disease, ninth revision, clinical modification; PE = pulmonary embolism; SVC = superior vena cava

Table Graphic Jump Location
Table 1. Vessels Showing Thrombosis
* 

One of these patients also had DVT of a lower extremity.

Table Graphic Jump Location
Table 2. Predisposing Factors Among Patients With Brachiocephalic or SVC Thrombosis
Table Graphic Jump Location
Table 3. Signs and Symptoms
* 

Patients with accompanying thrombosis of axillary, subclavian, or internal jugular vein were excluded.

Gucalp, R, Dutcher, J (2001) Oncologic emergencies. Braunwald, E Fauci, AS Kasper, DLet al eds.Harrison's principles of internal medicine 15th ed. ,642-650 McGraw-Hill. New York, NY:
 
Maddox, A-M, Valdivieso, M, Lukeman, J, et al Superior vena cava obstruction in small cell bronchogenic carcinoma: clinical parameters and survival.Cancer1983;52,2165-2172. [PubMed] [CrossRef]
 
Ryan, JA, Abel, RM, Abbott, WM, et al Catheter complications in total parenteral nutrition: a prospective study of 200 consecutive patients.N Engl J Med1974;290,757-761. [PubMed]
 
Kwong, T, Leonidas, JC, Ilowite, NT Asymptomatic superior vena cava thrombosis and pulmonary embolism in an adolescent with SLE and antiphospholipid antibodies.Clin Exp Rheum1994;12,215-217
 
Goldstein, MF, Nestico, P, Olshan, AR, et al Superior vena cava thrombosis and pulmonary embolus: association with right atrial mural thrombus.Arch Intern Med1982;142,1726-1728. [PubMed]
 
Black, MD, French, GJ, Rasuli, P, et al Upper extremity deep venous thrombosis: underdiagnosed and potentially lethal.Chest1993;103,1887-1890. [PubMed]
 
Prandoni, P, Polistena, P, Bernardi, E, et al Upper-extremity deep vein thrombosis: risk factors, diagnosis, and complications.Arch Intern Med1997;157,57-62. [PubMed]
 
Torosian, MH, Meranze, S, Mullen, JL, et al Central venous access with occlusive superior central venous thrombosis.Ann Surg1986;203,30-33. [PubMed]
 
Haire, WD, Lieberman, RP, Edney, J, et al Hickman catheter- induced thoracic vein thrombosis: frequency and long-term sequelae in patients recovering from high-dose chemotherapy and marrow transplantation.Cancer1990;66,900-908. [PubMed]
 
Jones, ML Brouch, K Allen, MMet al eds. St. Anthony's ICD-9-CM code book. 1991; St. Anthony Publishers. Alexandria, VA:.
 
Stein, PD, Patel, KC, Kalra, NJ, et al Estimated incidence of acute pulmonary embolism in a community/teaching general hospital.Chest2002;121,802-805. [PubMed]
 
American Hospital Association. AHA guide to the health field 2000–2001 edition: health forum.2000,A221 American Hospital Association. Chicago, IL:
 
Abner, A Approach to the patient who presents with superior vena cava obstruction.Chest1993;103(suppl),394S-397S
 
Salsali, M, Cliffton, EE Superior vena caval obstruction with lung cancer.Ann Thorac Surg1968;6,437-442. [PubMed]
 
Adelstein, DJ, Hines, JD, Carter, SG, et al Thromboembolic events in patients with malignant superior vena cava syndrome and the role of anticoagulation.Cancer1988;62,2258-2262. [PubMed]
 
Knudson, GJ, Wiedmeyer, DA, Erickson, SJ, et al Color Doppler sonographic imaging in the assessment of upper-extremity deep venous thrombosis.AJR Am J Roentgenol1990;154,399-403. [PubMed]
 
Falk, RL, Smith, DF Thrombosis of upper extremity thoracic inlet veins: diagnosis with duplex Doppler sonography.AJR Am J Roentgenol1987;149,677-682. [PubMed]
 
Gooding, GAW, Hightower, DR, Moore, EH, et al Obstruction of the superior vena cava or subclavian veins: sonographic diagnosis.Radiology1986;159,663-665. [PubMed]
 
Schwartz, EE, Goodman, LR, Haskin, ME Role of CT scanning in the superior vena cava syndrome.Am J Clin Oncol1986;9,71-78. [PubMed]
 
Hartnell, GG, Hughes, LA, Finn, JP, et al Magnetic resonance angiography of the central chest veins.Chest1995;107,1053-1057. [PubMed]
 
Finn, JP, Zisk, JHS, Edelman, RR, et al Central venous occlusion: MR angiography.Radiology1993;187,245-251. [PubMed]
 
Qanadli, SD, Hajjam, ME, Bruckert, F, et al Helical CT phlebography of the superior vena cava: diagnosis and evaluation of venous obstruction.AJR Am J Roentgenol1999;172,1327-1333. [PubMed]
 
Kroencke, TJ, Taupitz, M, Arnold, R, et al Three-dimensional gadolinium-enhanced magnetic resonance venography in suspected thrombo-occlusive disease of the central chest veins.Chest2001;120,1570-1576. [PubMed]
 
Gore, JM, Matsumoto, AH, Layden, JJ, et al Superior vena cava syndrome: its association with indwelling balloon-tipped pulmonary artery catheter.Arch Intern Med1984;144,506-508. [PubMed]
 
Dollery, CM, Sullivan, ID, Bauraind, O, et al Thrombosis and embolism in long-term central venous access for parenteral nutrition.Lancet1994;344,1043-1045. [PubMed]
 
Ghosh, BC, Cliffton, EE Malignant tumors with superior vena cava obstruction.N Y State J Med1973;73,283-289. [PubMed]
 
Martinelli, I, Cattaneo, M, Panzeri, D, et al Risk factors for deep venous thrombosis of the upper extremities.Ann Intern Med1997;126,707-711. [PubMed]
 

Figures

Tables

Table Graphic Jump Location
Table 1. Vessels Showing Thrombosis
* 

One of these patients also had DVT of a lower extremity.

Table Graphic Jump Location
Table 2. Predisposing Factors Among Patients With Brachiocephalic or SVC Thrombosis
Table Graphic Jump Location
Table 3. Signs and Symptoms
* 

Patients with accompanying thrombosis of axillary, subclavian, or internal jugular vein were excluded.

References

Gucalp, R, Dutcher, J (2001) Oncologic emergencies. Braunwald, E Fauci, AS Kasper, DLet al eds.Harrison's principles of internal medicine 15th ed. ,642-650 McGraw-Hill. New York, NY:
 
Maddox, A-M, Valdivieso, M, Lukeman, J, et al Superior vena cava obstruction in small cell bronchogenic carcinoma: clinical parameters and survival.Cancer1983;52,2165-2172. [PubMed] [CrossRef]
 
Ryan, JA, Abel, RM, Abbott, WM, et al Catheter complications in total parenteral nutrition: a prospective study of 200 consecutive patients.N Engl J Med1974;290,757-761. [PubMed]
 
Kwong, T, Leonidas, JC, Ilowite, NT Asymptomatic superior vena cava thrombosis and pulmonary embolism in an adolescent with SLE and antiphospholipid antibodies.Clin Exp Rheum1994;12,215-217
 
Goldstein, MF, Nestico, P, Olshan, AR, et al Superior vena cava thrombosis and pulmonary embolus: association with right atrial mural thrombus.Arch Intern Med1982;142,1726-1728. [PubMed]
 
Black, MD, French, GJ, Rasuli, P, et al Upper extremity deep venous thrombosis: underdiagnosed and potentially lethal.Chest1993;103,1887-1890. [PubMed]
 
Prandoni, P, Polistena, P, Bernardi, E, et al Upper-extremity deep vein thrombosis: risk factors, diagnosis, and complications.Arch Intern Med1997;157,57-62. [PubMed]
 
Torosian, MH, Meranze, S, Mullen, JL, et al Central venous access with occlusive superior central venous thrombosis.Ann Surg1986;203,30-33. [PubMed]
 
Haire, WD, Lieberman, RP, Edney, J, et al Hickman catheter- induced thoracic vein thrombosis: frequency and long-term sequelae in patients recovering from high-dose chemotherapy and marrow transplantation.Cancer1990;66,900-908. [PubMed]
 
Jones, ML Brouch, K Allen, MMet al eds. St. Anthony's ICD-9-CM code book. 1991; St. Anthony Publishers. Alexandria, VA:.
 
Stein, PD, Patel, KC, Kalra, NJ, et al Estimated incidence of acute pulmonary embolism in a community/teaching general hospital.Chest2002;121,802-805. [PubMed]
 
American Hospital Association. AHA guide to the health field 2000–2001 edition: health forum.2000,A221 American Hospital Association. Chicago, IL:
 
Abner, A Approach to the patient who presents with superior vena cava obstruction.Chest1993;103(suppl),394S-397S
 
Salsali, M, Cliffton, EE Superior vena caval obstruction with lung cancer.Ann Thorac Surg1968;6,437-442. [PubMed]
 
Adelstein, DJ, Hines, JD, Carter, SG, et al Thromboembolic events in patients with malignant superior vena cava syndrome and the role of anticoagulation.Cancer1988;62,2258-2262. [PubMed]
 
Knudson, GJ, Wiedmeyer, DA, Erickson, SJ, et al Color Doppler sonographic imaging in the assessment of upper-extremity deep venous thrombosis.AJR Am J Roentgenol1990;154,399-403. [PubMed]
 
Falk, RL, Smith, DF Thrombosis of upper extremity thoracic inlet veins: diagnosis with duplex Doppler sonography.AJR Am J Roentgenol1987;149,677-682. [PubMed]
 
Gooding, GAW, Hightower, DR, Moore, EH, et al Obstruction of the superior vena cava or subclavian veins: sonographic diagnosis.Radiology1986;159,663-665. [PubMed]
 
Schwartz, EE, Goodman, LR, Haskin, ME Role of CT scanning in the superior vena cava syndrome.Am J Clin Oncol1986;9,71-78. [PubMed]
 
Hartnell, GG, Hughes, LA, Finn, JP, et al Magnetic resonance angiography of the central chest veins.Chest1995;107,1053-1057. [PubMed]
 
Finn, JP, Zisk, JHS, Edelman, RR, et al Central venous occlusion: MR angiography.Radiology1993;187,245-251. [PubMed]
 
Qanadli, SD, Hajjam, ME, Bruckert, F, et al Helical CT phlebography of the superior vena cava: diagnosis and evaluation of venous obstruction.AJR Am J Roentgenol1999;172,1327-1333. [PubMed]
 
Kroencke, TJ, Taupitz, M, Arnold, R, et al Three-dimensional gadolinium-enhanced magnetic resonance venography in suspected thrombo-occlusive disease of the central chest veins.Chest2001;120,1570-1576. [PubMed]
 
Gore, JM, Matsumoto, AH, Layden, JJ, et al Superior vena cava syndrome: its association with indwelling balloon-tipped pulmonary artery catheter.Arch Intern Med1984;144,506-508. [PubMed]
 
Dollery, CM, Sullivan, ID, Bauraind, O, et al Thrombosis and embolism in long-term central venous access for parenteral nutrition.Lancet1994;344,1043-1045. [PubMed]
 
Ghosh, BC, Cliffton, EE Malignant tumors with superior vena cava obstruction.N Y State J Med1973;73,283-289. [PubMed]
 
Martinelli, I, Cattaneo, M, Panzeri, D, et al Risk factors for deep venous thrombosis of the upper extremities.Ann Intern Med1997;126,707-711. [PubMed]
 
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