Hybrid Repair of a Thoraco-abdominal Aortic Aneurysm Associated with Loeys-Dietz Syndrome

Introduction: Loeys-Dietz syndrome (LDS) is a genetic syndrome caused by mutations in transforming growth factor beta receptors (TGFBR) 1 and 2. It can manifest with craniofacial, musculoskeletal, cognitive abnormalities, and vascular pathologies including early onset aortic root aneurysms, extensive aortic dissections, and TAAA. Open repair is considered the gold standard treatment but carries morbidity risks, especially in patients with multiple previous aortic procedures. Endovascular treatment is associated with treatment failure when used in the native aorta, because of inherent wall weakness precluding seal. This case report adds to the available literature on hybrid treatment of LDS associated aortic pathologies. Report: This is the report of staged hybrid TAAA treatment in a 24 year old male patient with multiple previous aortic procedures via sternotomy and thoracotomy. Retrograde infrarenal aortic visceral debranching was performed using 14 mm by 7 mm bifurcated Dacron grafts. These emerged from the limbs of an 18 mm by 9 mm bifurcated Dacron graft in an aortobi-iliac reconstruction. This was followed by staged thoracic endovascular aortic repair (TEVAR) seven days later using three endografts (26 mm–22 mm × 150 mm distal, 30 mm × 200 mm bridging, then 32 mm × 100 mm proximal). The endograft landed in an old thoracic aortic graft proximally and the new infrarenal aortic graft distally. Follow up at 11 months showed patency and no sac expansion. Conclusion: Hybrid TAAA repair was a valid treatment option in this patient with LDS and multiple previous aortic procedures. It minimised the morbidity of revision surgery and mitigated potential treatment failure by achieving an endovascular seal in surgical grafts. Short term surveillance showed no complications. Limitations to making recommendations include lack of long term follow up

Heparin-induced thrombocytopenia causing graft thrombosis and bowel ischemia postendovascular aneurysm repair

Heparin-induced thrombocytopenia (HIT) is an immune-mediated thrombocytopenia resulting from prior heparin exposure. It can be associated with limb- or life-threatening thrombotic events. Patients undergoing any vascular procedures including endovascular procedures that require heparin administration are at risk. There is very little reported in the literature with regards to thrombosis associated with HIT after endovascular aortic aneurysm repair. All reported cases of HIT thrombosis presented as acute arterial lower limb ischemia or deep vein thrombosis. In this report, we present a case of HIT complicated by stent graft thrombosis and bowel ischemia

Paraplegia prevention branches: A new adjunct for preventing or treating spinal cord injury after endovascular repair of thoracoabdominal aneurysms

In this report, we describe a technique that could potentially be used for both prevention and treatment of spinal cord ischemia (SCI) in endovascular repair of thoracoabdominal aneurysms. This technique involves using a specially designed endograft with side branches (paraplegia prevention branches [PPBs]), which are left patent to perfuse the aneurysmal sac and any associated lumbar or intercostal arteries in the early postoperative period. The use of PPBs with this technique is feasible and allows for a temporary controlled endoleak that may be useful for preventing or reversing spinal cord injury. This technique may be considered as an adjunct to the more standard perioperative physiological manipulations such as permissive hypertension and spinal fluid drainage