see also:

• deep venous thrombosis (DVT)
• DVT prophylaxis
• Mx of lower limb DVT

• ~10% of all cases of deep venous thrombosis (DVT) involve the upper extremity resulting in an annual incidence in USA of 0.4-1 cases per 10,000 people
• cases have become more common with use of central venous catheters, cardiac pacemakers and defibrillators
• compared to patients with lower limb DVT, patients with upper limb DVT are:
  • more likely to be younger and leaner
  • more likely to have underlying cancer (in a recent prospective study, 38% had cancer and 3 month mortality was 11% with only 0.5% dying from PE)
  • less likely to have underlying acquired or hereditary thrombophilia

• accounts for 20% of cases

• venous thoracic outlet syndrome
  • DVT results from repetitive microtrauma to the subclavian vein and its surrounding structures, often due to anatomical anomalies of the costoclavicular junction.
• Paget-Schroetter syndrome
  • 2/3rds of patients with primary upper limb DVT (mostly young men), report strenuous activity involving force (pitching a baseball, playing badminton, swimming, lifting weights, rowing, wrestling) or abduction (overhead activities such as painting, car repairs, etc) of the dominant arm prior to development of the DVT.
• idiopathic

• 80% of cases

• catheter-associated
• cancer-associated
• surgery or trauma of the arm or shoulder - compression or venous stasis due to immobilisation or plaster cast
• pregnancy, OCP or ovarian hyperstimulation syndrome

• often subclinical - thrombosis is present in up to 2/3rds of patients with central venous catheters on routine screening without clinical signs.
• discomfort, pain, paraesthesiae and weakness of the arm
• swelling, oedema, discoloration and visible collaterals of the arm are typical
• superior vena cava syndrome is usually caused by caval tumour infiltration:
  • facial swelling, headache, nausea and SOB

• pulmonary embolism (PE) is less common than in lower limb DVT - 6% vs 15-32% for lower limb DVT
  • 3 month mortality from PE
• recurrence of DVT is less common than in lower limb DVT - 2-5% vs 10% for lower limb DVT
• post-thrombotic syndrome is less common than in lower limb DVT - 5% vs 56% for lower limb DVT
  • risk is higher for thrombosis of the axillary subclavian veins compared with other upper limb locations
  • risk is higher with residual thrombosis at 6 months
  • risk is lower for catheter-associated thrombosis

• D-Dimer has a 100% sensitivity but given that a significant proportion of patients will have cancer or catheter, the D-Dimer is likely to be elevated anyway and specificity is very low at ~14% and thus this is NOT as useful a test as it is for pulmonary embolism (PE), and it's use is best reserved for those with low to intermediate pre-test probability in which case a negative D-Dimer is an accurate means of excluding it.
• compression ultrasonography and duplex ultrasonography:
  • the preferred Ix but may miss proximal subclavian and brachiocephalic thromboses as these are difficult to visualise due to overlying bone.
• CT angiography or MR angiography
  • may be useful if ultrasound is not diagnostic, and has the advantage of visualising potential associated conditions such as neoplasm, adenopathy or venous thoracic outlet anomalies.

• removal of central venous catheter is only recommended if either:
  • no further need for it
  • no expected difficulties in gaining iv access
  • malfunction of catheter
  • infection
  • contraindication to anticoagulation
  • persisting symptoms or signs despite anticoagulation Rx
• anticoagulation Rx:
  • usual recommendation is for initial 5 day Rx with heparin or LMW heparin¹⁾, if not contra-indicated, then re-assessment:
    • only mild symptoms ⇒ change to at least 3-6 months anticoagulation with warfarin or a LMW heparin (the latter is preferred in those with underlying cancer).
    • severe symptoms/signs, then consider:
      • catheter-directed thrombolysis if bleeding risk is low
      • catheter or surgical interventions if failure of thrombolysis and anticoagulation
• investigation of underlying cause:
  • this should be guided based upon individual patient circumstances
  • consider thoracic CT scan