A combined vascular surgical and clinical genetics approach to diffuse aneurysmal disease.

We report two patients who presented with extensive aneurysmal disease, in association with minimal external physical signs. Patient 1 remained genetically undiagnosed despite multiple structural, biochemical and genetic investigations. He made a good recovery following surgery for popliteal and left axillary artery aneurysms. Patient 2 was diagnosed with vascular type Ehlers-Danlos syndrome, associated with a high degree of tissue and blood vessel fragility, and is being managed conservatively. Early multidisciplinary assessment of such patients facilitates accurate diagnosis and management.

Recent advances in thoraco-abdominal aortic aneurysm repair.

Thoraco-abdominal aortic aneurysm repair remains a formidable challenge to vascular surgeons. The traditional repair of thoraco-laparotomy with aortic cross-clamping is associated with a high morbidity and mortality despite significant advances in perioperative critical care, anaesthetic and surgical techniques. The advent of the endovascular revolution has shown a marked paradigm in the approach to all aneurysm repairs. As a logical progression from the open repair, the St Mary's visceral hybrid repair combines traditional open techniques (retrograde visceral and renal revascularisation via mid-line laparotomy) with endovascular stent grafting, thereby avoiding the need for thoracotomy and aortic cross-clamping. In specialist centres, the results have been encouraging and easily comparable to the open repair. The technique has been used in several centres around the world and represents a robust, transferrable method of repairing thoraco-abdominal aortic aneurysms. Stent-grafting technologies have reached a point of sophistication that wholly endovascular methods of repairing thoraco-abdominal aortic aneurysms are being performed in several centres around the world. Although these stent grafts have to be customised to the individual patient and are only suitable for certain types of aneurysmal anatomies, they represent the future of thoraco-abdominal aortic aneurysm repair. We review the history of thoraco-abdominal aortic aneurysm repair, the exciting advances in their treatment and discuss our approach to the management of thoraco-abdominal aortic aneurysms in the 21st century.

Recombinant activated factor VII: a solution to refractory haemorrhage in vascular surgery?

OBJECTIVES: Post-operative haemorrhage is a recognised complication and independent predictor of outcome in complex vascular surgery. The off-license administration of activated Recombinant Factor VII (rFVIIa) to treat haemorrhage in other surgical settings has been investigated, but concerns over potential adverse events have limited its use in vascular surgery. This article reports rFVIIa's method of action and systematically reviews rFVIIa's role in complex vascular surgery. METHODS: A systematic literature search identified articles reporting on rFVIIa administration within vascular surgery patients. Patient-specific data regarding transfusion requirements was extracted and pooled statistical analysis performed. RESULTS: 15 articles reporting 43 patients were identified. RFVIIa has been administered in open and endovascular procedures and in both elective and emergency settings. Major aortic surgery accounted for 75% of cases. The range of rFVIIa administered as a cumulative dose was large, as was the variation in initial dose. Transfusion data from 9 patients was pooled and analysed. Significant differences were found between pre- and post- rFVIIa for packed red cell transfusions (mean 29.2 vs. 8.2, p=0.015). Intra-arterial thrombosis was reported in 3 cases. CONCLUSIONS: RFVIIa may reduce haemorrhage in selected vascular surgical patients. Randomized controlled trials are justified to definitively investigate its role within this setting.