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OBJECTIVES:
Thoracic aortic mural thrombus (TAMT) is a rare pathology and potential source of cerebral, visceral, and peripheral emboli. Due to the rarity, there are no treatment guidelines for or a consensus on the management of TAMT. We herein present a 62-year-old male in a hypercoagulable state due to primary polycythemia vera (PV) who developed TAMT followed by multiple catastrophic thromboembolic events despite aggressive anticoagulation and surgical therapy. To the best of our knowledge, this is the second reported case of TAMT occurring in a patient with PV.
METHODS:
Review of this case, as well as review basic principles regarding TAMT.
RESULTS:
A 62 year-old male with primary PV and systemic mastocytosis presented with right lower extremity ischemia and chest discomfort. Computed tomography (CT) scan of the chest, abdomen and pelvis revealed a focal filling defect in the distal aortic arch measuring 1.6 x 1.6 x 1.1 cm consistent with a focal thrombus, and a focal non-occlusive filling defect within the right common femoral artery. Systemic heparinization was initiated. He underwent a thrombectomy of the right lower extremity and thoracic aortic stent graft (SG) placement (GORE TAG 28mm x 15cm) for exclusion of the thrombus. Postoperative course was complicated by spinal cord ischemia followed by delayed catastrophic recurrent thromboembolisms including all extremities, mesenteric and cerebral ischemia. The patient eventually expired due to anoxic brain injury.
CONCLUSIONS:
Aggressive anticoagulation and surgical thrombectomy to solve peripheral ischemic complications seems to be an appropriate strategy. The endovascular approach is the emerging treatment of choice for TAMT with promising short-term outcomes. By contrast, aortic mural thrombus in the presence of underlying PV is exceedingly rare and may pose recurrent thromboembolic risk despite endovascular SG repair, possibly due to stimulation of coagulation cascade. We would recommend proceeding with extreme caution when performing endovascular exclusion of the thrombus as PV may be a prohibitive risk.