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

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.

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

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

The Clown Prince of Jazz 9

Urschel’s Sign

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%

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

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

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

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

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)

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

Catheter - Induced DVT R L

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

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

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

Pacemaker - Induced DVT

Duplex Ultrasound Right Subclavian Vein

Duplex Ultrasound Right Subclavian Vein

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

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: 1301-1306

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

Duplex Ultrasound Right Subclavian Vein

Duplex Ultrasound of Right Subclavian Vein @ 3 - months

Paget - Schroëtter Syndrome Leopold-von-Schroëtter, Vienna (1837-1908) Sir James Paget, London (1814-1898)

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

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

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

Duplex Ultrasound

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

Orders to Dr. Charles Dotter !!!

Dr. Dotter’s “Rebuttal”

Venogram Right Axillary - Subclavian Vein

EKOS Catheter

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

Venogram (Post t - PA @ 4 hours)

Final Venogram

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

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

Venogram 6 Month Follow - up

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

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, 2008:254-260

Post - Lysis of Axillary - SCV

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

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

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. 2012 Jun;133 (6 Supp)