Upper Extremity Deep Venous Thrombosis: Etiologies, Diagnosis, and Updates in Therapeutic Strategies

Abstract

Upper extremity deep vein thrombosis (UEDVT) is responsible for 4 to 10% of all deep vein thrombosis (DVT). Untreated UEDVT can lead to significant disability secondary to the postthrombotic syndrome. To date, there are no randomized trials specifically comparing different therapeutic strategies. Ultimately, optimal management of UEDVT depends on the underlying etiology, patient symptoms, and degree of thrombosis, with supporting evidence primarily extrapolated from lower extremity DVT data. This article will review the classification, presentation, and diagnosis of both primary and secondary UEDVT. In addition, it will discuss updates in clinical guidelines, anticoagulation, endovascular and surgical treatment strategies.

Keywords

upper extremity deep vein thrombosis - postthrombotic syndrome - endovenous catheter-directed therapy - venous thoracic outlet syndrome

Publication History

Article published online:
20 December 2022

• References

• 1 Isma N, Svensson PJ, Gottsäter A, Lindblad B. Upper extremity deep venous thrombosis in the population-based Malmö thrombophilia study (MATS). Epidemiology, risk factors, recurrence risk, and mortality. Thromb Res 2010; 125 (06) e335-e338
• 2 Engelberger RP, Kucher N. Management of deep vein thrombosis of the upper extremity. Circulation 2012; 126 (06) 768-773
• 3 Modi BP, Chewning R, Kumar R. Venous thoracic outlet syndrome and Paget-Schroetter syndrome. Semin Pediatr Surg 2021; 30 (06) 151125
• 4 Hendler MF, Meschengieser SS, Blanco AN. et al. Primary upper-extremity deep vein thrombosis: high prevalence of thrombophilic defects. Am J Hematol 2004; 76 (04) 330-337
• 5 Kucher N. Clinical practice. Deep-vein thrombosis of the upper extremities. N Engl J Med 2011; 364 (09) 861-869
• 6 Winters JP, Callas PW, Cushman M, Repp AB, Zakai NA. Central venous catheters and upper extremity deep vein thrombosis in medical inpatients: the Medical Inpatients and Thrombosis (MITH) Study. J Thromb Haemost 2015; 13 (12) 2155-2160
• 7 Luciani A, Clement O, Halimi P. et al. Catheter-related upper extremity deep venous thrombosis in cancer patients: a prospective study based on Doppler US. Radiology 2001; 220 (03) 655-660
• 8 Duijzer D, de Winter MA, Nijkeuter M, Tuinenburg AE, Westerink J. Upper extremity deep vein thrombosis and asymptomatic vein occlusion in patients with transvenous leads: a systematic review and meta-analysis. Front Cardiovasc Med 2021; 8: 698336
• 9 Joffe HV, Kucher N, Tapson VF, Goldhaber SZ, Deep Vein Thrombosis FSC. Deep Vein Thrombosis (DVT) FREE Steering Committee. Upper-extremity deep vein thrombosis: a prospective registry of 592 patients. Circulation 2004; 110 (12) 1605-1611
• 10 Paauw JD, Borders H, Ingalls N. et al. The incidence of PICC line-associated thrombosis with and without the use of prophylactic anticoagulants. JPEN J Parenter Enteral Nutr 2008; 32 (04) 443-447
• 11 Sanders RJ, Hammond SL, Rao NM. Diagnosis of thoracic outlet syndrome. J Vasc Surg 2007; 46 (03) 601-604
• 12 Lee JB, Kurzweil A, Lahham S. A case report on Paget-Schroetter syndrome presenting as acute localized rhabdomyolysis. Clin Pract Cases Emerg Med 2020; 4 (03) 358-361
• 13 Muñoz FJ, Mismetti P, Poggio R. et al; RIETE Investigators. Clinical outcome of patients with upper-extremity deep vein thrombosis: results from the RIETE Registry. Chest 2008; 133 (01) 143-148
• 14 Levy MM, Albuquerque F, Pfeifer JD. Low incidence of pulmonary embolism associated with upper-extremity deep venous thrombosis. Ann Vasc Surg 2012; 26 (07) 964-972
• 15 Houghton DE, Billett HH, Gaddh M. et al. Risk of pulmonary emboli after removal of an upper extremity central catheter associated with a deep vein thrombosis. Blood Adv 2021; 5 (14) 2807-2812
• 16 Elman EE, Kahn SR. The post-thrombotic syndrome after upper extremity deep venous thrombosis in adults: a systematic review. Thromb Res 2006; 117 (06) 609-614
• 17 Thiyagarajah K, Ellingwood L, Endres K. et al. Post-thrombotic syndrome and recurrent thromboembolism in patients with upper extremity deep vein thrombosis: a systematic review and meta-analysis. Thromb Res 2019; 174: 34-39
• 18 Desjardins B, Hanley M, Steigner ML. et al; Expert Panel on Vascular Imaging. ACR Appropriateness Criteria® suspected upper extremity deep vein thrombosis. J Am Coll Radiol 2020; 17 (5S): S315-S322
• 19 van Dam LF, Dronkers CEA, Gautam G. et al; Theia Study Group. Magnetic resonance imaging for diagnosis of recurrent ipsilateral deep vein thrombosis. Blood 2020; 135 (16) 1377-1385
• 20 Kearon C, Akl EA, Ornelas J. et al. Antithrombotic therapy for VTE disease: CHEST Guideline and Expert Panel Report. Chest 2016; 149 (02) 315-352
• 21 Stevens SM, Woller SC, Kreuziger LB. et al. Antithrombotic therapy for VTE disease: second update of the CHEST Guideline and Expert Panel Report. Chest 2021; 160 (06) e545-e608
• 22 Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK. Paget-Schroetter syndrome: review of pathogenesis and treatment of effort thrombosis. West J Emerg Med 2010; 11 (04) 358-362
• 23 AbuRahma AF, Sadler D, Stuart P, Khan MZ, Boland JP. Conventional versus thrombolytic therapy in spontaneous (effort) axillary-subclavian vein thrombosis. Am J Surg 1991; 161 (04) 459-465
• 24 Aburahma AF, Sadler DL, Robinson PA. Axillary subclavian vein thrombosis. Changing patterns of etiology, diagnostic, and therapeutic modalities. Am Surg 1991; 57 (02) 101-107
• 25 Lee WA, Hill BB, Harris Jr EJ, Semba CP, Olcott C IV. Surgical intervention is not required for all patients with subclavian vein thrombosis. J Vasc Surg 2000; 32 (01) 57-67
• 26 Molina JE, Hunter DW, Dietz CA. Protocols for Paget-Schroetter syndrome and late treatment of chronic subclavian vein obstruction. Ann Thorac Surg 2009; 87 (02) 416-422
• 27 Edo Fleta G, Torres Blanco Á, Gómez Palonés F, Ortiz Monzón E. Combined non-surgical treatment for Paget-Schröetter syndrome: a case report. J Med Case Reports 2016; 10: 171
• 28 Haig Y, Enden T, Grøtta O. et al; CaVenT Study Group. Post-thrombotic syndrome after catheter-directed thrombolysis for deep vein thrombosis (CaVenT): 5-year follow-up results of an open-label, randomised controlled trial. Lancet Haematol 2016; 3 (02) e64-e71
• 29 Comerota AJ, Kearon C, Gu CS. et al; ATTRACT Trial Investigators. Endovascular thrombus removal for acute iliofemoral deep vein thrombosis. Circulation 2019; 139 (09) 1162-1173
• 30 Doyle A, Wolford HY, Davies MG. et al. Management of effort thrombosis of the subclavian vein: today's treatment. Ann Vasc Surg 2007; 21 (06) 723-729
• 31 Meier GH, Pollak JS, Rosenblatt M, Dickey KW, Gusberg RJ. Initial experience with venous stents in exertional axillary-subclavian vein thrombosis. J Vasc Surg 1996; 24 (06) 974-981 , discussion 981–983
• 32 Kärkkäinen JM, Nuutinen H, Riekkinen T. et al. Pharmacomechanical thrombectomy in Paget-Schroetter syndrome. Cardiovasc Intervent Radiol 2016; 39 (09) 1272-1279
• 33 Singh A, Kaur M, Singh A, Bhatti A, Patel R. Paget-Schroetter syndrome (PSS) and adjunctive treatment with mechanical aspiration thrombectomy and catheter directed thrombolysis: a case report and review of literature. Cureus 2022; 14 (04) e24437
• 34 Sýkora J, Zeleňák K, Vorčák M. et al. Percutaneous thrombectomy in the management of early rethrombosis in venous thoracic outlet syndrome: two case reports. CVIR Endovasc 2021; 4 (01) 61
• 35 Bayer O, Schummer C, Richter K, Fröber R, Schummer W. Implication of the anatomy of the pericardial reflection on positioning of central venous catheters. J Cardiothorac Vasc Anesth 2006; 20 (06) 777-780
• 36 Usoh F, Hingorani A, Ascher E. et al. Long-term follow-up for superior vena cava filter placement. Ann Vasc Surg 2009; 23 (03) 350-354
• 37 Owens CA, Bui JT, Knuttinen MG, Gaba RC, Carrillo TC. Pulmonary embolism from upper extremity deep vein thrombosis and the role of superior vena cava filters: a review of the literature. J Vasc Interv Radiol 2010; 21 (06) 779-787
• 38 Samoila G, Twine CP, Williams IM. The infraclavicular approach for Paget-Schroetter syndrome. Ann R Coll Surg Engl 2018; 100 (02) 83-91
• 39 Suzuki T, Kimura H, Matsumura N, Iwamoto T. Surgical approaches for thoracic outlet syndrome: a review of the literature. Journal of Hand Surgery Global Online 2022
• 40 Elixène JB, Sadaghianloo N, Mousnier A, Brizzi S, Declemy S, Hassen-Khodja R. Long-term functional outcomes and subclavian vein patency in patients undergoing thoracic outlet surgery for Paget-Schroetter syndrome. J Cardiovasc Surg (Torino) 2017; 58 (03) 451-457
• 41 de Kleijn RJCMF, Schropp L, Westerink J, de Borst GJ, Petri BJ. Timing of thoracic outlet decompression after thrombolysis for primary upper extremity deep venous thrombosis: a systematic review. Ann Vasc Surg 2020; 66: 654-661
• 42 Silverberg D, Fish M, Lubetsky A. et al. Long-term outcome after nonsurgical management of Paget-Schroetter syndrome. J Vasc Surg Venous Lymphat Disord 2021; 9 (01) 170-177
• 43 Peek J, Vos CG, Ünlü Ç, van de Pavoordt HDWM, van den Akker PJ, de Vries JPM. Outcome of surgical treatment for thoracic outlet syndrome: systematic review and meta-analysis. Ann Vasc Surg 2017; 40: 303-326
• 44 Lugo J, Tanious A, Armstrong P. et al. Acute Paget-Schroetter syndrome: does the first rib routinely need to be removed after thrombolysis?. Ann Vasc Surg 2015; 29 (06) 1073-1077
• 45 Carlon TA, Sudheendra D. Interventional therapy for upper extremity deep vein thrombosis. Semin Intervent Radiol 2017; 34 (01) 54-60
• 46 Harmon D, Dabaja W, Qaqi O. A novel interventional approach to upper extremity swelling. J Vasc Surg Cases Innov Tech 2020; 6 (02) 209-211