Background In the absence of diffuse atherosclerosis or aneurysms, TAMT are exceedingly rare Cerebral, visceral and peripheral arterial emboli are a common and debilitating clinical presentation The rate of repeat embolisation is unknown, but seemingly high Indication and timing of thoracic aortic thrombectomy are controversial Paucity of data describing this disorder

OBJECTIVES  To describe the clinical presentation, treatment and outcome of 13 patients with TAMT  To define a treatment strategy for patients with TAMT

Patient Population Between 2/96 and 7/09, 13 patients were treated with TAMT Mean age 52 ± 13 years (8 females) Hypercoagulable disorder/ + family history n=6 Peripheral embolectomy/thrombectomyn=5 Diagnosis: CTA (n=11); TEE (n=12); angiography (n=1) Intravenous heparin/ASAn=13 Thoracic Aortic Thrombectomyn=7 Medical Treatmentn=5

PatientAgePresenting embolic event Coagulation DisorderRecurrent Embolism Location in aortaTreatmentStatus 152Blue toe syndrome, Upper Extremity, TIA Arch (2)MedicalA 264Stroke ArchSurgeryA 346Bilateral LE, Embolectomy/Thrombectomy BLE, Rt BKA Protein S Deficiency, + FHYDistal Arch, DTAMedicalD 442Renal-Splenicpositive FHYThoraco-AbdominalSurgeryA 550Bilateral LEThrombocytopenia, Homocysteinemia, + FH Thoraco-AbdominalMedicalA 660Blue toe syndromepositive FH Proximal DTASurgeryA 784Mesenteric, stroke Arch (2)MedicalD 848Blue toe syndrome YProximal DTASurgeryA 936spleen, Rt LE, Lt Upper extremities Homocysteinemia + FHYProximal DTASurgeryA 1042splenic Thoraco-Abdominal SurgeryA 1169Stroke ArchMedicalA 1251Mesenteric, stroke, Bilateral LE positive FHYDTAMedicalD 1342Stroke, Blue Toe Syndrome Ascending,Distal Arch (2) Surgery- Medical A PATIENT CHARACTERISTICS

Location Ascending aorta and archn=5 Descending aorta n=6 Descending and abdominaln=3 Localized defect in aortic wall n=3 (isolated ulcer in 2 and aortic fossette in one) Highly mobile (pedunculated)n=11 Pathology

Clinical Scenario 46 year-old female with a strong history of hypercoagulable disorder presented with flank pain and hematuria. A CT angio showed a pedunculated TAMT (fig 1) and evidence of renal and splenic infarcts (fig 2). Intravenous heparin was used for 5 days followed by left thoraco-laparotomy with removal of a large aortic thrombus (clamp and sew) (fig 3). She recovered well and discharged home (warfarin/ASA) on day 7. She is alive at one year with no recurrence. Figure 1Figure 2Figure 3

Surgical Procedures 1.Thoracic aortic thrombectomiesn=7 Left thoracotomy (atrial-femoral bypass)n=4 Median sternotomy with cardiopulmonary bypassn=2 (hypothermic circulatory arrest in 1) Left thoraco-laparotomy (clamp and sew)n=1 2.Procedures for complications*n=7 Lower extremity embolectomy/thrombectomyn=2 Femoro-popliteal artery bypass n=1 Mesenteric artery embolectomy/bowel resectionn=1 Lower extremity amputationn=1 Upper extremity embolectomy/thrombectomyn=1 Celiac artery embolectomyn=1 * Pre-post and/or during thoracic aortic procedure

Clinical Outcomes 1.Surgeryn=7 Operative mortality 0% Recurrence n=1 (8 mm suture line thrombus that resolved with anticoagulation) All patients alive at mean follow-up of 24 ± 16 mo 2.Medical treatmentn=6 1 patient died at presentation with stroke/mesenteric ischemia 6 patients at mean follow-up of 14 ± 11 mo (1 patient = 2 thrombus) 2 patients had a fatal recurrent embolic event within 6 weeks 2 patients had resolution of thrombus and within 4 weeks 2 patients had a stable thrombus

Reference^^YearCasesDiagnosisTreatment Recurrence (Thrombectomy) Recurrence (Anticoagulation) Laperche et al.^199723TEEOperative/Medical 1/10 4/15 Lau et al.19975TEEMedical 1/4 Goueffic et al.^200238TEE/CTA/MRIOperative4/38NA Choukron et al.20019TEE/CTAOperative/Medical 1/50/4 Bowdisk et al.20025TEE/CTAMedical NA 0/5 Pagni et al TEE/CTA/AngioOperative/Medical 1/7 3/6 * LITERATURE *1 patient had a thrombus in the ascending aortic (surgery) and one in the descending aorta (medical treatment) ^ Multi-center study ^^Only series reporting 5 or more patients

Therapeutic Strategy All patients are given aspirin and intravenous heparin at diagnosis Peripheral or visceral embolectomy if indicated Work-up for malignancy and pro- coagulable disorders If the patient is viable after initial embolic event thoracic thrombectomy within 2 weeks if no resolution of thrombus If initial embolic event is too morbid or surgical risk too high oral anticoagulation and CTA follow-up If thrombus is pedunculated (highly mobile) Early Thrombectomy

SUMMARY TAMT is a rare and often debilitating clinical condition Thoracic aortic thrombectomy can be performed at low risk Early intervention may prevent fatal recurrent embolic events Larger series are needed to better define the role of surgery and the embolic risk of these lesions