Venous Thrombosis of the Upper Extremities Agne s Y. Y. Le e, MD, FRCP( C) Je ffre y S. Ginsbe rg, MD, FRCP( C) Address Division of Hematology and Thromboembolism, Department of Medicine, Hamilton Health Sciences Corporation, Henderson Site, 711 Concession Street, Hamilton, Ontario, Canada L8V 1C3. E-mail: alee@ thrombosis.hhscr.org Current Treatment Options in Cardiovascular Medicine 2001, 3:207–214 Current Science Inc. ISSN 1092-8464 Copyright © 2001 by Current Science Inc. Introduction Spontaneous or effort-related upper-extremity deep vein thrombosis (UEDVT) was first described independently by Paget and von Schroetter in the 1800s. Although the original case reports involved idiopathic (unprovoked) thrombosis of the axillary-subclavian venous system, UEDVT is now commonly associated with secondary causes such as central venous catheterization. Over 75% of cases of UEDVT are associated with indwelling central catheters, and they represent approximately 2% of all cases of deep vein thrombosis [1,2]. Primary UEDVT occurs primarily in young, healthy individuals in the second or third decade, with a slightly higher prevalence in males. About 60% of cases occur in the dominant arm [1,2]. Some cases of UEDVT in patients are associated with strenuous or repetitive arm exercise or prolonged abduction, such as rowing or weight lifting, whereas other patients have anatomical abnormalities, such as thoracic outlet syndrome. The latter predisposes the subclavian and axillary veins to mechanical injury and intimal damage, which allegedly can lead to recurrent thrombosis. Unfortunately, despite a lack of evidence linking UEDVT to direct venous compression and vessel injury, the belief in this hypothesis has led to the frequent practice of surgical thoracic outlet decompression in many centers. Based upon documented case series, the long-term prognosis of Paget-von Schroetter syndrome is considered to be poor, with up to 70% of untreated patients and about Opinion statement The goals of treating patients with upper-extremity deep vein thrombosis (UEDVT) are to relieve acute symptoms of venous occlusion, prevent pulmonary embolism, reduce the likelihood of recurrent thrombosis, and avoid the development of postphlebitic syndrome. Although the details of management differ, depending on the underlying cause and precipitating factors, anticoagulant therapy should be the first-line treat- ment of choice in all cases. For patients with primary or idiopathic UEDVT (Paget- von Schroetter syndrome), aggressive measures including catheter-directed thrombo- lysis, vascular procedures ( eg , balloon angioplasty, stenting, filter), and surgical maneuvers ( eg , first rib resection) have been advocated by some surgeons, but none of these high-risk interventions has been evaluated properly in prospective controlled trials. In contrast, for patients with catheter-associated central venous thrombosis (CACVT), or other secondary cases of UEDVT, many clinicians simply withdraw the catheter and avoid anticoagulant therapy. Because well-designed clinical trials are lacking, recommendations about the management of UEDVT are derived from descrip- tive studies and case series. Until further research identifies the natural history and optimum management of UEDVT, it seems reasonable to base treatment on antico- agulant regimens with proven effectiveness in lower-extremity deep vein thrombosis (LEDVT). The use of additional intervention(s) should be reserved for carefully selected patients.