Pacemaker-associated Phlegmasia Cerulea Dolens Treated with Catheter-directed Thrombolysis

Abstract

Phlegmasia cerulea dolens (PCD) is a rare and severe form of deep venous thrombosis that is classically associated with the lower extremities. We report a case of upper extremity PCD developing abruptly in a 37-year-old female with an indwelling cardiac pacemaker who presented to the emergency department complaining of pain and paresthesias in her left arm, adjoining left chest wall, and inferior neck. Her condition was promptly diagnosed and successfully treated with intravenous unfractionated heparin and balloon venoplasty with catheter-directed thrombolysis without any known residual signs or symptoms at hospital discharge.

A comparative analysis of the efficacy and safety of therapeutic interventions in phlegmasia cerulea dolens

“…The use of CDT as a single treatment approach in PCD was reported in many scenarios including a patient with pacemaker-associated upper extremity PCD, 12 a patient with history of ankylosing spondylitis, 13 two patients with gynecologic malignancies, 14 a pregnant woman, 15 a patient with simultaneous arterial thrombosis, 16 and a patient with bilateral lower extremities PCD due to IVC agenesis. 17 The only patient in our review who was found to have IVC agenesis was successfully treated with a combination of CDT and PMCDT but developed post-thrombotic syndrome afterwards.…”

Upper limb phlegmasia cerulea dolens treated with single-session suction thrombectomy: a case report

“…PCD is characterized by four cardinal signs: oedema, pain, limb cyanosis and extreme venous outflow obstruction. In contrast to the insidious onset of symptomatic DVT, PCD symptoms evolve rapidly over hours [ 1–3 ]. Untreated PCD is fatal in 25-40% of cases and the reported rate of amputation in survivors is 20–50% [ 4 , 5 ].…”

“…Acute upper limb deep vein thrombosis (DVT) is an uncommon clinical presentation, whilst upper limb phlegmasia cerulea dolens (PCD) is even rarer [ 1 ]. PCD describes a clinical syndrome characterized by a triad of acute limb swelling, pain and cyanosis (or purple discolouration) of the affected limb.…”

“…An interplay of Virchow’s triad, risk factors for PCD include hypercoagulable states such as malignancy, venous stasis and trauma. It is a potentially critical condition with complications including venous gangrene, limb loss and death [ 1–3 ]. Prompt recognition and institution of mainstay therapies is paramount to the alleviation of thrombus burden and overall improvement in patient outcomes.…”

Multimodal approach of venous recanalization in patients with a critical limb ischemia due to phlegmasia cerulea dolens: A case series of 17 patients in a single center

“…1,7,10 Thus, advanced PCD has a high mortality and morbidity as amputation rates of 20–50% and mortality rates of 22% have been reported. 4,5,15 Underlying malignancy should be one of the most important risk factors. 4,7 There have been stated mortality rates of 55% in patients with an advanced PCD and underlying malignancy.…”