PAGET-SCHROETTER SYNDROME




PATIENT STORY



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A previously healthy 22-year-old man presents with a 3-day history of right upper extremity swelling and pain. He is a competitive cross fit athlete and performs vigorous upper body weight lifting several days per week. On examination the right forearm and brachium are diffusely swollen with an overlying erythrocyanotic appearance. A venous duplex ultrasound identifies acute thrombosis within the axillary and subclavian veins. In preparation for catheter-directed thrombolysis, a right upper extremity venogram is performed. Figures 56-1A and B venographically illustrate diffuse intraluminal filling defects consistent with acute brachial and axillosubclavian deep vein thrombosis (DVT).




FIGURE 56-1A


Right upper extremity venogram demonstrating acute right brachial deep venous thrombosis (DVT).






FIGURE 56-1B


Right upper extremity venogram demonstrating acute right axillosubclavian vein deep venous thrombosis (DVT).






EPIDEMIOLOGY



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  • Primary effort-related thrombosis of the subclavian vein caused by compression within the thoracic outlet (Figure 56-2A).



  • Provocative arm positioning includes repetitive hyperabduction and external rotation of the upper extremity or posterior and inferior shoulder rotation.



  • Absence of other causes, such as extrinsic mass or indwelling catheter.



  • Male-to-female ratio is approximately 2:1.1



  • Mostly in third and fourth decades of life.1,2



  • Young, healthy athletes after vigorous upper extremity exercise and in patients who do frequent overhead maneuvers (painters, construction workers, wood choppers, auto repair workers).1



  • Involves the dominant arm in 80% of cases.





FIGURE 56-2A


Schematic of subclavian vein deep venous thrombosis (DVT) caused by compression from abnormal costoclavicular ligament.






ANATOMY AND PATHOPHYSIOLOGY



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  • The subclavian vein passes through the thoracic outlet bounded by the clavicle and subclavius muscle anteriorly, the anterior scalene muscle laterally, the first rib posterior-inferiorly, and the costoclavicular ligament medially (Figure 56-2B).



  • Extrinsic compression of the subclavian vein by the first rib and the clavicle.1,2, and 3



  • Hypertrophy of the anterior scalene muscle and/or the subclavius muscle can decrease the size of the outlet and compress the subclavian vein.1



  • Multifactorial pathophysiology that fulfills the Virchow triad including (1) anatomic changes listed above leading to stasis; (2) hypercoagulability in association with exercise-associated stress; (3) intimal tears within the vein wall in association with repetitive shoulder-arm movement.





FIGURE 56-2B


Schematic of normal anatomy of the thoracic outlet.




Jan 13, 2019 | Posted by in CARDIOLOGY | Comments Off on PAGET-SCHROETTER SYNDROME

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