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Thoracic Central Venous Intervention
Brandt C. Wible, MD; Scott M. Brannan, MD; Stephan Wicky, MD
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KEY FACTS

  • Terminology

    • Procedure

      • Post Procedure

        TERMINOLOGY

        • Synonyms

          • Brachiocephalic vein = innominate vein
            • Brachiocephalic vein is preferred term
            • Innominate veins bilateral, unlike innominate artery
        • Definitions

          • Thoracic central vein obstruction (TCVO)
            • Thoracic veins
              • Central (systemic): Intrathoracic segment of internal jugular veins (IJVs), subclavian veins (SCVs), brachiocephalic veins (BCVs), superior vena cava (SVC), and suprahepatic portion of inferior vena cava (IVC)
              • Somatic: Azygos/hemiazygos, paraspinal/epidural, superficial/body wall veins (collateral circulation in cases of systemic TCVO)
              • Visceral: Pulmonary veins, coronary sinus
            • Obstruction
              • Pathophysiologic venous luminal narrowing impeding blood flow
              • Can be partial (stenosis) or complete (occlusion)
            • Mechanism of obstruction
              • Extrinsic compression: Tumor, arterial, musculoskeletal, postoperative scarring/fibrosis
              • Venous wall thickening: Mural thrombus, fibrosis/hyperplasia secondary to stent/implanted cardiac device, tumor, infection, inflammation, intramural dissection/hematoma
              • Endoluminal occlusion: Thrombus, endoluminal medical implants, fibrin sheath/neointimal tissue
            • Clinical reporting guidelines have been suggested, including
              • Obstruction: Pattern and degree plus flow type
              • Symptoms: Swelling, pain, respiratory, and neurologic
              • Duration (of symptoms): 1-14 days (acute), 15-28 days (subacute) > 28 days (chronic)
              • Signs: Swelling, venous pressure, performance status
          • Axillosubclavian vein thrombosis (ASVT)
            • Primary ASVT
              • a.k.a. Paget-Schrötter syndrome, effort thrombosis, venous thoracic outlet syndrome
              • Thrombosis/stenosis/occlusion of axillary vein/SCV
              • Occurs in young adults following repetitive exercises (e.g., swimming, weight lifting) or prolonged shoulder abduction
                • M:F = 2:1
                • 15-35 years old
                • > 70% of cases involve dominant upper extremity
              • Associated with mechanical vein compression at costoclavicular junction
                • Hypertrophied/broad subclavius or anterior scalene muscle extrinsically compress vein
                • Repetitive trauma leads to thickening and fibrosis
                • Injured venous intima promotes thrombus formation
                • Clavicular and 1st-rib anomalies are rare
              • Clinical symptoms/physical findings
                • Sudden severe unilateral upper extremity swelling resulting from venous hypertension
                • Subocclusive thrombosis and resolution common before ultimate event
                • Prominent collateral veins on arm/shoulder/chest quickly develop on affected side
                • Acute symptoms of heaviness, aching, and swelling improve if ignored/untreated
                • Symptoms return with resumption of activity
                • 10% incidence of pulmonary embolism
            • Secondary ASVT
              • Much more common than primary form, accounting for 80% of ASVT
              • Etiology associated with inciting venous injury
                • Long-term central venous catheters/pacemakers primary cause (> 60%); increasing incidence
                • Malignancy/adenopathy (25-29%)
                • Trauma/surgery
                • Radiation therapy
              • May also involve brachiocephalic veins (BVs)
              • Affected population is older and less healthy
          • SVC obstruction (SVCO)
            • Malignant etiologies: Most common (60% of SVCO cases)
              • Most common etiologies: Bronchogenic carcinoma, small cell lung cancer, lymphoma, mediastinal metastasis
              • Symptoms
                • Typically worse than benign etiologies
                • Rapid onset limiting development of collaterals
                • Central location involving SVC just superior to right atrium, potentially involving azygous vein
            • Benign etiologies: Increasing as % of SVCO
              • Most common etiologies
                • Central venous catheters and pacemaker wires (30-40% of SVCO cases)
                • Fibrosing mediastinitis, aortic aneurysm (rare)
            • Clinical manifestations of SVCO
              • Neck swelling (100%), facial edema (48-82%), distended neck veins (63%), dyspnea (54-83%), cough (22-58%)
              • Reduced cardiac preload: Hypotension and syncope, especially during bending or coughing
              • Less common: Hoarseness, headache, dizziness, phrenic nerve paresis, chest pain
              • Grade 4 life-threatening SVC syndrome (5% of SVC syndrome presentations)
                • Significant laryngeal edema or cerebral edema or hemodynamic compromise
                • Requires emergent endovascular stent placement
                • Radiation therapy not indicated for 1st-line treatment if endovascular stenting is feasible
              • If thrombotic etiology (28% of SVC syndromes): Anticoagulation is 1st-line treatment, effective in 88% of cases when started within 5 days
              • If infectious etiology: Antibiotic coverage is 1st-line treatment
              • If benign fibrosing mediastinitis: Covered stents preferred over bare metal stents (BMS), demonstrating reduced restenosis and severity
        • Anatomy

          • Venous anatomy of involved veins
            • Upper extremity veins
              • Cephalic, basilic, median basilic
                • Basilic vein frequently joins brachial vein
                • Brachial vein terminates in axillary vein
              • Deep veins: Brachial (usually paired), axillary
              • Axillary vein starts at border of teres major muscle
                • Becomes subclavian vein at 1st rib outer edge
            • Mediastinal veins
              • Subclavian/internal jugular veins join to form brachiocephalic veins
              • Brachiocephalic veins join to form SVC
                • SVC posterior to manubrium/sternum on right
              • Collateral drainage with chronic SVC occlusion
                • Azygos system; unnamed neck/chest collaterals

        PREPROCEDURE

        • Indications

          • Contraindications

            • Preprocedure Imaging

              • Special Considerations

                • Getting Started

                  PROCEDURE

                  • Patient Position/Location

                    • Procedure Steps

                      • Alternative Procedures/Therapies

                        POST PROCEDURE

                        • Things to Do

                          OUTCOMES

                          • Complications

                            • Expected Outcome

                              Selected References

                              1. Azizi AH et al: Superior vena cava syndrome. JACC Cardiovasc Interv. 13(24):2896-910, 2020
                              2. Klein-Weigel PF et al: Superior vena cava syndrome. Vasa. 49(6):437-48, 2020
                              3. Dolmatch BL et al: Society of Interventional Radiology reporting standards for thoracic central vein obstruction: endorsed by the American Society of Diagnostic and Interventional Nephrology (ASDIN), British Society of Interventional Radiology (BSIR), Canadian Interventional Radiology Association (CIRA), Heart Rhythm Society (HRS), Indian Society of Vascular and Interventional Radiology (ISVIR), Vascular Access Society of the Americas (VASA), and Vascular Access Society of Britain and Ireland (VASBI). J Vasc Access. 20(2):114-22, 2019
                              4. Ratzon R et al: Thrombosis, anticoagulation and outcomes in malignant superior vena cava syndrome. J Thromb Thrombolysis. 47(1):121-8, 2019
                              5. Haddad MM et al: Is long-term anticoagulation required after stent placement for benign superior vena cava syndrome? J Vasc Interv Radiol. 29(12):1741-7, 2018
                              6. Shamimi-Noori SM et al: Venous stents: current status and future directions. Tech Vasc Interv Radiol. 21(2):113-6, 2018
                              7. Breault S et al: Percutaneous endovascular management of chronic superior vena cava syndrome of benign causes: long-term follow-up. Eur Radiol. 27(1):97-104, 2016
                              8. Agarwal AK: Endovascular interventions for central vein stenosis. Kidney Res Clin Pract. 34(4):228-32, 2015
                              9. Collin G et al: Central venous obstruction in the thorax. Clin Radiol. 70(6):654-60, 2015
                              10. Quaretti P et al: Dialysis catheter-related superior vena cava syndrome with patent vena cava: long term efficacy of unilateral Viatorr stent-graft avoiding catheter manipulation. Korean J Radiol. 15(3):364-9, 2014
                              11. Agarwal AK: Central vein stenosis. Am J Kidney Dis. 61(6):1001-15, 2013
                              12. Iafrati M et al: Radiofrequency thermal wire is a useful adjunct to treat chronic central venous occlusions. J Vasc Surg. 55(2):603-6, 2012
                              13. Canales JF et al: Single center experience with percutaneous endovascular repair of superior vena cava syndrome. Catheter Cardiovasc Interv. 77(5):733-9, 2011
                              14. Cho TH et al: The role of stenting the superior vena cava syndrome in patients with malignant disease. Angiology. 62(3):248-52, 2011
                              15. Lepper PM et al: Superior vena cava syndrome in thoracic malignancies. Respir Care. 56(5):653-66, 2011
                              16. Thompson JF et al: Venous thoracic outlet compression and the Paget-Schroetter syndrome: a review and recommendations for management. Cardiovasc Intervent Radiol. 34(5):903-10, 2011
                              17. Da Ines D et al: Cardiac tamponade after malignant superior vena cava stenting: two case reports and brief review of the literature. Acta Radiol. 51(3):256-9, 2010
                              18. Guzzo JL et al: Preoperative thrombolysis and venoplasty affords no benefit in patency following first rib resection and scalenectomy for subacute and chronic subclavian vein thrombosis. J Vasc Surg. 52(3):658-62, 2010
                              19. Illig KA et al: A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg. 51(6):1538-47, 2010
                              20. Greillier L et al: [Malignant superior vena cava syndrome: why refrain from vascular stenting?.] Rev Mal Respir. 26(7):719-21, 2009
                              21. Lanciego C et al: Endovascular stenting as the first step in the overall management of malignant superior vena cava syndrome. AJR Am J Roentgenol. 193(2):549-58, 2009
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Procedure

                                  • Post Procedure

                                    TERMINOLOGY

                                    • Synonyms

                                      • Brachiocephalic vein = innominate vein
                                        • Brachiocephalic vein is preferred term
                                        • Innominate veins bilateral, unlike innominate artery
                                    • Definitions

                                      • Thoracic central vein obstruction (TCVO)
                                        • Thoracic veins
                                          • Central (systemic): Intrathoracic segment of internal jugular veins (IJVs), subclavian veins (SCVs), brachiocephalic veins (BCVs), superior vena cava (SVC), and suprahepatic portion of inferior vena cava (IVC)
                                          • Somatic: Azygos/hemiazygos, paraspinal/epidural, superficial/body wall veins (collateral circulation in cases of systemic TCVO)
                                          • Visceral: Pulmonary veins, coronary sinus
                                        • Obstruction
                                          • Pathophysiologic venous luminal narrowing impeding blood flow
                                          • Can be partial (stenosis) or complete (occlusion)
                                        • Mechanism of obstruction
                                          • Extrinsic compression: Tumor, arterial, musculoskeletal, postoperative scarring/fibrosis
                                          • Venous wall thickening: Mural thrombus, fibrosis/hyperplasia secondary to stent/implanted cardiac device, tumor, infection, inflammation, intramural dissection/hematoma
                                          • Endoluminal occlusion: Thrombus, endoluminal medical implants, fibrin sheath/neointimal tissue
                                        • Clinical reporting guidelines have been suggested, including
                                          • Obstruction: Pattern and degree plus flow type
                                          • Symptoms: Swelling, pain, respiratory, and neurologic
                                          • Duration (of symptoms): 1-14 days (acute), 15-28 days (subacute) > 28 days (chronic)
                                          • Signs: Swelling, venous pressure, performance status
                                      • Axillosubclavian vein thrombosis (ASVT)
                                        • Primary ASVT
                                          • a.k.a. Paget-Schrötter syndrome, effort thrombosis, venous thoracic outlet syndrome
                                          • Thrombosis/stenosis/occlusion of axillary vein/SCV
                                          • Occurs in young adults following repetitive exercises (e.g., swimming, weight lifting) or prolonged shoulder abduction
                                            • M:F = 2:1
                                            • 15-35 years old
                                            • > 70% of cases involve dominant upper extremity
                                          • Associated with mechanical vein compression at costoclavicular junction
                                            • Hypertrophied/broad subclavius or anterior scalene muscle extrinsically compress vein
                                            • Repetitive trauma leads to thickening and fibrosis
                                            • Injured venous intima promotes thrombus formation
                                            • Clavicular and 1st-rib anomalies are rare
                                          • Clinical symptoms/physical findings
                                            • Sudden severe unilateral upper extremity swelling resulting from venous hypertension
                                            • Subocclusive thrombosis and resolution common before ultimate event
                                            • Prominent collateral veins on arm/shoulder/chest quickly develop on affected side
                                            • Acute symptoms of heaviness, aching, and swelling improve if ignored/untreated
                                            • Symptoms return with resumption of activity
                                            • 10% incidence of pulmonary embolism
                                        • Secondary ASVT
                                          • Much more common than primary form, accounting for 80% of ASVT
                                          • Etiology associated with inciting venous injury
                                            • Long-term central venous catheters/pacemakers primary cause (> 60%); increasing incidence
                                            • Malignancy/adenopathy (25-29%)
                                            • Trauma/surgery
                                            • Radiation therapy
                                          • May also involve brachiocephalic veins (BVs)
                                          • Affected population is older and less healthy
                                      • SVC obstruction (SVCO)
                                        • Malignant etiologies: Most common (60% of SVCO cases)
                                          • Most common etiologies: Bronchogenic carcinoma, small cell lung cancer, lymphoma, mediastinal metastasis
                                          • Symptoms
                                            • Typically worse than benign etiologies
                                            • Rapid onset limiting development of collaterals
                                            • Central location involving SVC just superior to right atrium, potentially involving azygous vein
                                        • Benign etiologies: Increasing as % of SVCO
                                          • Most common etiologies
                                            • Central venous catheters and pacemaker wires (30-40% of SVCO cases)
                                            • Fibrosing mediastinitis, aortic aneurysm (rare)
                                        • Clinical manifestations of SVCO
                                          • Neck swelling (100%), facial edema (48-82%), distended neck veins (63%), dyspnea (54-83%), cough (22-58%)
                                          • Reduced cardiac preload: Hypotension and syncope, especially during bending or coughing
                                          • Less common: Hoarseness, headache, dizziness, phrenic nerve paresis, chest pain
                                          • Grade 4 life-threatening SVC syndrome (5% of SVC syndrome presentations)
                                            • Significant laryngeal edema or cerebral edema or hemodynamic compromise
                                            • Requires emergent endovascular stent placement
                                            • Radiation therapy not indicated for 1st-line treatment if endovascular stenting is feasible
                                          • If thrombotic etiology (28% of SVC syndromes): Anticoagulation is 1st-line treatment, effective in 88% of cases when started within 5 days
                                          • If infectious etiology: Antibiotic coverage is 1st-line treatment
                                          • If benign fibrosing mediastinitis: Covered stents preferred over bare metal stents (BMS), demonstrating reduced restenosis and severity
                                    • Anatomy

                                      • Venous anatomy of involved veins
                                        • Upper extremity veins
                                          • Cephalic, basilic, median basilic
                                            • Basilic vein frequently joins brachial vein
                                            • Brachial vein terminates in axillary vein
                                          • Deep veins: Brachial (usually paired), axillary
                                          • Axillary vein starts at border of teres major muscle
                                            • Becomes subclavian vein at 1st rib outer edge
                                        • Mediastinal veins
                                          • Subclavian/internal jugular veins join to form brachiocephalic veins
                                          • Brachiocephalic veins join to form SVC
                                            • SVC posterior to manubrium/sternum on right
                                          • Collateral drainage with chronic SVC occlusion
                                            • Azygos system; unnamed neck/chest collaterals

                                    PREPROCEDURE

                                    • Indications

                                      • Contraindications

                                        • Preprocedure Imaging

                                          • Special Considerations

                                            • Getting Started

                                              PROCEDURE

                                              • Patient Position/Location

                                                • Procedure Steps

                                                  • Alternative Procedures/Therapies

                                                    POST PROCEDURE

                                                    • Things to Do

                                                      OUTCOMES

                                                      • Complications

                                                        • Expected Outcome

                                                          Selected References

                                                          1. Azizi AH et al: Superior vena cava syndrome. JACC Cardiovasc Interv. 13(24):2896-910, 2020
                                                          2. Klein-Weigel PF et al: Superior vena cava syndrome. Vasa. 49(6):437-48, 2020
                                                          3. Dolmatch BL et al: Society of Interventional Radiology reporting standards for thoracic central vein obstruction: endorsed by the American Society of Diagnostic and Interventional Nephrology (ASDIN), British Society of Interventional Radiology (BSIR), Canadian Interventional Radiology Association (CIRA), Heart Rhythm Society (HRS), Indian Society of Vascular and Interventional Radiology (ISVIR), Vascular Access Society of the Americas (VASA), and Vascular Access Society of Britain and Ireland (VASBI). J Vasc Access. 20(2):114-22, 2019
                                                          4. Ratzon R et al: Thrombosis, anticoagulation and outcomes in malignant superior vena cava syndrome. J Thromb Thrombolysis. 47(1):121-8, 2019
                                                          5. Haddad MM et al: Is long-term anticoagulation required after stent placement for benign superior vena cava syndrome? J Vasc Interv Radiol. 29(12):1741-7, 2018
                                                          6. Shamimi-Noori SM et al: Venous stents: current status and future directions. Tech Vasc Interv Radiol. 21(2):113-6, 2018
                                                          7. Breault S et al: Percutaneous endovascular management of chronic superior vena cava syndrome of benign causes: long-term follow-up. Eur Radiol. 27(1):97-104, 2016
                                                          8. Agarwal AK: Endovascular interventions for central vein stenosis. Kidney Res Clin Pract. 34(4):228-32, 2015
                                                          9. Collin G et al: Central venous obstruction in the thorax. Clin Radiol. 70(6):654-60, 2015
                                                          10. Quaretti P et al: Dialysis catheter-related superior vena cava syndrome with patent vena cava: long term efficacy of unilateral Viatorr stent-graft avoiding catheter manipulation. Korean J Radiol. 15(3):364-9, 2014
                                                          11. Agarwal AK: Central vein stenosis. Am J Kidney Dis. 61(6):1001-15, 2013
                                                          12. Iafrati M et al: Radiofrequency thermal wire is a useful adjunct to treat chronic central venous occlusions. J Vasc Surg. 55(2):603-6, 2012
                                                          13. Canales JF et al: Single center experience with percutaneous endovascular repair of superior vena cava syndrome. Catheter Cardiovasc Interv. 77(5):733-9, 2011
                                                          14. Cho TH et al: The role of stenting the superior vena cava syndrome in patients with malignant disease. Angiology. 62(3):248-52, 2011
                                                          15. Lepper PM et al: Superior vena cava syndrome in thoracic malignancies. Respir Care. 56(5):653-66, 2011
                                                          16. Thompson JF et al: Venous thoracic outlet compression and the Paget-Schroetter syndrome: a review and recommendations for management. Cardiovasc Intervent Radiol. 34(5):903-10, 2011
                                                          17. Da Ines D et al: Cardiac tamponade after malignant superior vena cava stenting: two case reports and brief review of the literature. Acta Radiol. 51(3):256-9, 2010
                                                          18. Guzzo JL et al: Preoperative thrombolysis and venoplasty affords no benefit in patency following first rib resection and scalenectomy for subacute and chronic subclavian vein thrombosis. J Vasc Surg. 52(3):658-62, 2010
                                                          19. Illig KA et al: A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg. 51(6):1538-47, 2010
                                                          20. Greillier L et al: [Malignant superior vena cava syndrome: why refrain from vascular stenting?.] Rev Mal Respir. 26(7):719-21, 2009
                                                          21. Lanciego C et al: Endovascular stenting as the first step in the overall management of malignant superior vena cava syndrome. AJR Am J Roentgenol. 193(2):549-58, 2009