1. Paget - Schroetter Syndrome: Diagnosis and Treatment
Robert M Schainfeld, DO
Associate Director, Vascular Medicine
Massachusetts General Hospital

2. Disclosure Statement of Financial Interest
I, Robert Schainfeld, DO NOT have a financial interest/arrangement or affiliation with one or more organizations that could be perceived as a real or apparent conflict of interest in the context of the subject of this presentation.

4. Upper Extremity Venous Anatomy
Superficial veins
- cephalic
- median antecubital branches
- basilic
Deep veins
- ulnar, radial and brachial (paired)
- axillary and subclavian
- brachiocephalic and superior vena cava

8. Presentation of Upper Extremity Venous Thrombosis (UEDVT)
2% - 4% of all venous thromboses
US - incidence of 50,000 cases annually
11 cases / 100,000 hospital admissions
Involves brachial, axillary and subclavian veins
Signs / Symptoms
Swelling / discoloration
Pain / discomfort in arm, shoulder, neck
Prominent superficial veins

9. The Clown Prince of Jazz9

11. Urschel’s Sign

12. Clinical Sequelae Pulmonary embolus ~ 12% symptomatic and up to
~ 36% may remain asymptomatic
Venous hypertension  PTS (severe 13%)
Loss of future vascular access or SVC syndrom
Recurrence after Tx ~ 2% - 8%

13. Etiologies of Upper Extremity Venous Thrombosis
Primary axillo - subclavian vein thrombosis (idiopathic or Paget – Schroetter syndrome)
No associated disease or trauma
Exertion - related
Secondary axillo - subclavian vein thrombosis
Recognized cause
2o to central venous catheters (CVC), ICD, pacemakers
Systemic due to malignancy, thrombophilia, trauma

14. Characteristics of Upper Extremity Venous Thrombosis (UEDVT)
Younger, non - white, lower BMI
More likely admitted for non - VTE Dx
Recent CVC
Infection
Malignancy
ICU discharge
Less likely recurrent DVT

15. Risk Factors for UEDVT Malignancy Central venous catheters
Oral contraceptive Rx (2 - 6 fold increased risk)
Hormone replacement Rx
Immobilization
Obesity
Pregnancy
Ovarian hyperstimulation

16. Diagnosis of Upper Extremity DVT (Duplex Ultrasonography)
Symptomatic patients
- Sensitivity / Specificity = 82% - 100%
Asymptomatic patients
- Sensitivity = 35% *
* pediatrics w / non - occlusive central vein thrombus

17. Advantages and Disadvantages of Imaging Modalities in Dx of UEDVT
Ultrasound Inexpensive Noninvasive Reproducible CT scan Detect central thrombus Detect extrinsic vein compression MRI Detect central thrombus Detailed info about flow and collaterals
May not detect central thrombus below clavicle Contrast dye Claustrophobia Not suited if metal (stents, ICD, PPM)

18. AGW
51 year old male admitted with H1N1 respiratory failure, and multi - system organ failure
PICC placed in right basilic vein for pressor support
Stabilizes and transferred to medical floor from ICU
3 days after PICC removed, swelling noted in right arm

19. Catheter - Induced DVT R L

20. Treatment Options for UEDVT
Limb elevation
Graduated compression sleeve
Anticoagulation
- UFH warfarin
- LMWH warfarin
- LMWH as monotherapy
Catheter - directed lysis
PTA / thrombectomy
Surgical thrombectomy
TOS decompression
SVC Filter

21. Management of Central Venous Catheter - Induced DVT
Removal of CVC if feasible - ideal solution
AC for minimum of 3 – months
If catheter is necessity, AC until catheter removal,
continue for total of 3 – 6 months as tolerated
Favorable clinical outcomes in small patient series

22. Management of Asymptomatic UEDVT
Treatment is ill - defined
Risk / benefit analysis of treatment not yet known
Absence of data, thus asymptomatic subclavian vein thrombi should be treated expectantly
Risk of AC probably outweighs its ??? benefits

23. Pacemaker - Induced DVT

24. Duplex Ultrasound Right Subclavian Vein

25. Duplex Ultrasound Right Subclavian Vein

26. Risk Factors for Severe Venous Stenosis / Occlusions
Multiple pacemaker leads
Hormone therapy
Personal history of VTE
Temporary wire before PPM implantation
Presence of PPM (ICD upgrade)
Use of dual coil leads

27. Do Carmo Da Costa, SS, et al. PACE 2002; 25: 1301-1306
Conclusions
Frequent venous lesions (64%)
Independent risk factors for venous lesions
- previous temporary PM and LVEF < 40%
Paucity of symptomatic patients (5.2%)
Do Carmo Da Costa, SS, et al. PACE 2002; 25:

28. Management of PPM / ICD Venous Thrombosis
Anticoagulation
Thrombolysis
Laser removal of old leads
PTA
Stenting (Wallstents / Nitinol)
Surgery
- reserved for endovascular treatment failures or
unfavorable anatomy for endovenous Rx

29. Duplex Ultrasound Right Subclavian Vein

30. Duplex Ultrasound of Right Subclavian Vein @ 3 - months

31. Paget - Schroëtter Syndrome
Leopold-von-Schroëtter, Vienna
( )
Sir James Paget,
London
( )

32. Paget - Schroëtter Syndrome
2 - 4% of DVT involve upper limb
Often secondary to repetitive upper extremity activity in the presence of a mechanical abnormality at the thoracic inlet
Arm abduction, cervical extension & shoulder depression
e.g. Weight lifting, baseball throwing, rowing, lacrosse, lobster fishing
Repetitive compression results in fibrous tissue formation that permanently strangles the vein
Most patients present after vigorous physical activity
? Micro-trauma → activation of coagulation cascade

33. Common anomalies
- Young athlete with hypertrophied muscle
- First or clavicular rib
- Musculofascial bands
- Cervical ribs

34. Ms SM 48 yo F presented with right upper extremity swelling HPI
Previously well & active
Woke - up with pain & gross swelling of arm
Heavy lifting & mammogram few days prior
Multiple presentations to OSH
Rx as cellulitis
PMH
HTN
Smoker
No VTE - DVT or PE

35. Duplex Ultrasound

36. You’re on one of the few arteries and Veins we can’t unclog.

37. Orders to Dr. Charles Dotter !!!

38. Dr. Dotter’s “Rebuttal”

39. Venogram Right Axillary - Subclavian Vein

40. EKOS Catheter

41. Ultrasound Accelerated Catheter-Directed Lysis
5F EndoWave® Peripheral System
Multi Side Port Infusion Catheter
Ultrasound Core Wire
2mg/hr x 4 hrs
Heparin (PTT 40-50)

42. Venogram (Post t - PA @ 4 hours)

43. Final Venogram

44. Venogram @ 3 - weeks (Post -1st rib resection)

45. Venogram @ 3 - weeks (Post -1st rib resection)

46. Venogram 6 Month Follow - up

47. Fate of Contralateral Vein
UCLA series: 61% with compression of contralateral vein on venography
If normal in neutral pos’n – stress (TOS)
Role of surgery if Asx ???
- elective repair if compression of vein in dominant arm and occupation exposes patient at increased risk for thrombosis

48. Treatment of Primary ASDVT Results
Largest retrospective series (50 - years)
626 limbs / 608 patients
Best results in 511 / 548 patients < 6 weeks & prompt surgery
24 / 42 limbs > 6 weeks all remained sx
36 patients, no lysis
10 - ASX
25 (PTS) - despite first rib resection
Urschel et al., Ann Thorac Surg, 2008:

49. Post - Lysis of Axillary - SCV

50. Algorithm for the Management of 1o ASDVT
Venography
Thrombosis
Compression/Stricture
Lysis
Anticoagulation
Evaluation
Symptomatic
Asymptomatic
Abnormality
Asymptomatic
No Abnormality
1st Rib Resection
(early vs. delayed?)
Residual Stenosis
Normal Vein
PTA +/- Stent
Urschel et al., Ann Thorac Surg, 2002:69

52. Complications of UEDVT
Brachial plexopathy
Chronic venous insufficiency
Loss of vascular access
Pulmonary embolus (PE)
Septic thrombophlebitis
SVC syndrome
Thoracic duct obstruction
Venous gangrene

53. Treatment of Upper - Extremity DVT
LMWH, UFH or fondaparinux at therapeutic doses (Grade 1C)
Most patients do not warrant thrombolytic therapy (Grade 1C)
Selected patients w / low bleeding risk or severe sxs of recent onset (14 d) – CDT appropriate if expertise and resources available (Grade 2C)
Chest Jun;133 (6 Supp)