Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis

Abstract

Effort thrombosis, or Paget-Schroetter Syndrome, refers to axillary-subclavian vein thrombosis associated with strenuous and repetitive activity of the upper extremities. Anatomical abnormalities at the thoracic outlet and repetitive trauma to the endothelium of the subclavian vein are key factors in its initiation and progression. The role of hereditary and acquired thrombophilias is unclear. The pathogenesis of effort thrombosis is thus distinct from other venous thromboembolic disorders. Doppler ultrasonography is the preferred initial test, while contrast venography remains the gold standard for diagnosis. Computed tomographic venography and magnetic resonance venography are comparable to conventional venography and are being increasingly used. Conservative management with anticoagulation alone is inadequate and leads to significant residual disability. An aggressive multimodal treatment strategy consisting of catheter-directed thrombolysis, with or without early thoracic outlet decompression, is essential for optimizing outcomes. Despite excellent insights into its pathogenesis and advances in treatment, a significant number of patients with effort thrombosis continue to be treated suboptimally. Hence, there is an urgent need for increasing physician awareness about risk factors, etiology and the management of this unique and relatively infrequent disorder.

Figure 1.

Normal anatomy of the thoracic outlet. Foot note: Reproduced with permission from Urschel et al, Ann Thorac Surg 2008;86:254–60 Copyright Elsevier Inc, 2008.

Figure 2.

Abnormal lateral insertion of the costoclavicular ligament in Paget-Schroetter syndrome. Foot note: Reproduced with permission from Urschel et al, Ann Thorac Surg 2008;86:254–60 Copyright Elsevier Inc, 2008.

Figure 3.

Diagnostic and management algorithm for patients with suspected effort thrombosis. TOD, thoracic outlet decompression