Risk factors: UEDVT is further categorized as either primary or secondary, depending upon the cause. First described in the late 1800s, spontaneous primary thrombosis of the upper extremity, or Paget-Schroetter syndrome, accounts for approximately 20% of UEDVT.9 Primary UEDVT includes both idiopathic and “effort-related” thrombosis. Effort-related thrombosis usually develops among young people after strenuous or repetitive exercise, such as pitching a baseball. Some hypothesize that effort-related thrombosis is related to a hypercoaguable state or anatomic abnormalities, although a specific cause, such as thoracic outlet syndrome, is found in only 5% of these cases.10,11
Secondary UEDVT characterizes thrombosis in which an endogenous or exogenous risk factor is present. Endogenous risk factors include coagulation abnormalities, such as antithrombin, protein C and protein S deficiencies; factor V Leiden gene mutation; hyperhomocysteinemia; and antiphospholipid antibody syndrome. Exogenous risk factors include CVC pacemakers, intracardiac defibrillators, malignancy, previous or concurrent LEDVT, oral contraceptives, some artificial reproductive technologies (women can develop ovarian hyperstimulation syndrome, which is associated with increased hypercoaguability), trauma, and IV drug use (especially cocaine).5,12-14
Clinical presentation and diagnosis: Swelling (80% of patients) and pain (40% of patients) are the most common UEDVT symptoms at presentation.2 Other clinical features include new, prominent veins throughout the shoulder girdle, erythema, increased warmth, functional impairment, parathesias, and non-specific feelings of arm heaviness or discomfort. Symptoms typically worsen with arm use and improve with rest and elevation.15 Patients with UEDVT related to CVC are more likely to be asymptomatic and may present only with PE.16 The differential diagnosis includes superficial phlebitis, lymphatic edema, hematoma, contusions, venous compression, and muscle tears.17
Contrast venography is the gold standard for the UEDVT diagnosis. However, it is more expensive and invasive than ultrasound, and thus serial compression ultrasound is now the standard test in UEDVT evaluation. Then again, contrast venography remains the test of choice in patients with high pre-test probability and negative ultrasound results.18,19
Prevention: Nearly 70% of secondary UEDVT is associated with a CVC.5 Further, CVC use is the most powerful predictor of UEDVT (adjusted odds ratio (OR), 9.7; 95% CI, 7.8 to 12.2).2 Despite the association between CVCs and UEDVT, anticoagulant prophylaxis is not recommended. Studies evaluating the results of 1-mg warfarin conflict and include small populations. Warfarin’s potential interaction with antibiotics and dosing variance based on nutritional intake logically prompted studies on the potential benefit of low-weight molecular heparain (LWMH); however, these studies have failed to show benefit.20,21
Colorado Rockies pitcher Aaron Cook had a pulmonary embolism during a 2004 game that was attributed to effort-related thrombosis of his right arm. He needed two surgeries and 12 months of rehab before returning to the mound.
Treatment: Recent ACCP guidelines recommend treating UEDVT patients with unfractionated heparin (UFH) or LMWH and warfarin, with an INR goal of 2 to 3 for at least three months depending upon the overall clinical scenario. Two small studies evaluating catheter-related thrombosis (15 patients in each trial) reported no subsequent embolic phenomenon.22,23 Some authors interpreted this data to mean UEDVT was not as morbid as LEDVT and, subsequently, that catheter-related UEDVTs require only one month of therapy. Since the small studies were published, the increasing incidence and relevance of UEDVT have become more widely recognized, and most authors are recommending three months of treatment.
Still, it’s important to note that there aren’t any published data directly comparing the one-month and the three-month anticoagulation therapies. The RIETE registry, which is the largest ongoing published registry of patients with confirmed DVT or PE, reports similar three-month clinical outcomes between those with UEDVT and LEDVT.
Small, single-center trials have shown that active intervention, such as thrombolysis, surgery, or multi-staged approaches are associated with increased vein patency and decreased rates of post-thrombotic syndrome.24,25 However, ACCP has withheld general recommendations for these interventions based on a lack of sufficient data to comment on their overall safety and efficacy, as well as comparable rates of post-thrombotic syndrome (15% to 50%) in studies that directly compared surgical and medical intervention. In fact, the ACCP recommends against interventional treatments unless the patient has failed anticoagulation therapy, has severe symptoms, and expertise is available.4