Successful endovascular repair of acute type B aortic dissection in undiagnosed Ehlers-Danlos syndrome type IV.

A 61-year-old man presented with an acute type B aortic dissection for which a stent-graft was introduced. He remains complication-free 4 years onwards and has since been diagnosed with Ehlers-Danlos syndrome type IV (EDS IV). His particular mutation is predicted to result in lesser levels of normal collagen and may explain his favourable outcome from endovascular intervention. Understanding the genotype-phenotype correlation may influence the choice of therapy offered to patients with EDS IV.

Management of spontaneous and iatrogenic retroperitoneal haemorrhage: conservative management, endovascular intervention or open surgery?

BACKGROUND: Retroperitoneal haematoma is a rare clinical entity with variable aetiology, which is increasing in incidence mainly due to complications related to interventional procedures. There is no general consensus as to the best management plan for patients with retroperitoneal haematoma. METHODS: A literature review was undertaken using MEDLINE, all relevant papers on retroperitoneal haemorrhage or haematoma were used. RESULTS: The diagnosis is often delayed as symptoms are nonspecific. Retroperitoneal haematoma should be suspected in patients with significant groin, flank, abdominal, back pain or haemodynamic instability following an interventional procedure. Spontaneous haemorrhage usually occurs in patients who are anticoagulated. Multi-slice CT and arteriography are important for diagnosis. Most haemodynamically stable patients can be managed with fluid resuscitation, correction of coagulopathy and blood transfusion. Endovascular treatment involving selective intra-arterial embolisation or the deployment of stent-grafts over the punctured vessel is attaining an increasingly important role. Open repair of retroperitoneal bleeding vessels should be reserved for cases when there is failure of conservative or endovascular measures to control the bleeding. Open repair is also required if endovascular facilities or expertise is unavailable and in cases where the patient is unstable. If treated inappropriately, the mortality of patients with retroperitoneal haematoma remains high. CONCLUSION: There is a lack of level I evidence for the best management plans for retroperitoneal haematoma, and evidence is based on small cohort series or isolated case reports. Conservative management should only be reserved for patients who are stable. Interventional radiology with intra-arterial embolisation or stent-grafting is the treatment of choice. Open surgery is now rarely required.

Endoluminal repair of distal aortic arch aneurysms causing aorto-vocal syndrome.

PURPOSE: We have evaluated the efficacy of endovascular repair of distal aortic arch aneurysms (DAAA) causing recurrent laryngeal nerve palsy. MATERIAL AND METHODS: Eight patients (five male and three female) with median age of 72 years (range: 59-80) presented with left recurrent laryngeal nerve palsy associated with DAAA. All patients were considered unfit for open surgery. The median aneurysm size was 5.9 cm (range: 5-7.3). Thirteen stents were deployed: eight Gore, four Endofit and one Talent. Epidural anaesthesia was used in all patients. The left subclavian artery was covered in all and the left common carotid in three who had a preliminary right to left carotid-carotid bypass. Routine follow-up (FU) was with computed tomography (CT) at 3-6 months and yearly thereafter. RESULTS: Exclusion of the aneurysm sac was achieved in all patients. Thirty-day mortality was 0%, with no paraplegia or stroke. Early complications included: rupture of the external iliac artery (one) and common femoral artery thrombectomy (one). One patient died of unknown cause at 17 months. The mean FU in the remaining seven patients was 21 months (range: 6-51). Aneurysm size decreased in five, was unchanged in one and increased in one. Three patients had improvement in voice quality postoperatively. One patient had a recurrent type 1 endoleak which was restented twice. No late deaths have occurred. CONCLUSION: Though technically the procedures involved were more complicated, endovascular repair of DAAA causing aorto-vocal syndrome is safe and offers a realistic alternative to open surgery. Hoarseness of the voice can improve postoperatively and is associated with reduction in aortic sac diameter.

Neurological complications following endoluminal repair of thoracic aortic disease.

Open surgery for thoracic aortic disease is associated with significant morbidity and the reported rates for paraplegia and stroke are 3%-19% and 6%-11%, respectively. Spinal cord ischemia and stroke have also been reported following endoluminal repair. This study reviews the incidence of paraplegia and stroke in a series of 186 patients treated with thoracic stent grafts. From July 1997 to September 2006, 186 patients (125 men) underwent endoluminal repair of thoracic aortic pathology. Mean age was 71 years (range, 17-90 years). One hundred twenty-eight patients were treated electively and 58 patients had urgent procedures. Anesthesia was epidural in 131, general in 50, and local in 5 patients. Seven patients developed paraplegia (3.8%; two urgent and five elective). All occurred in-hospital apart from one associated with severe hypotension after a myocardial infarction at 3 weeks. Four of these recovered with cerebrospinal fluid (CSF) drainage. One patient with paraplegia died and two had permanent neurological deficit. The rate of permanent paraplegia and death was 1.6%. There were seven strokes (3.8%; four urgent and three elective). Three patients made a complete recovery, one had permanent expressive dysphasia, and three died. The rate of permanent stroke and death was 2.1%. Endoluminal treatment of thoracic aortic disease is an attractive alternative to open surgery; however, there is still a risk of paraplegia and stroke. Permanent neurological deficits and death occurred in 3.7% of the patients in this series. We conclude that prompt recognition of paraplegia and immediate insertion of a CSF drain can be an effective way of recovering spinal cord function and improving the prognosis.

Hybrid repair of type B dissecting aneurysm with associated Kommerell's diverticulum.

Endovascular therapy has revolutionised vascular surgery. Complex open surgical procedures may be replaced with a combination of extra-anatomical reconstruction and endovascular treatment. This minimally invasive approach is associated with a lower peri-operative morbidity and mortality than open repair. We describe a novel 2-stage treatment in a patient with Type B thoracic aortic dissection with subsequent aneurysmal dilatation and the added difficulty of a concomitant aneurysmal aberrant right subclavian artery (Kommerell's diverticulum).

Large infra-renal abdominal aortic aneurysms: endovascular vs. open repair--single centre experience.

Endovascular aneurysm repair (EVAR) has become an established alternative to open repair (OR). We present a consecutive series of 486 elective patients with large infra-renal aortic abdominal aneurysm, comparing OR with EVAR. Prospective data collected during an 8-year period from January 1997 to October 2005 was reviewed. Statistical analysis performed using SPSS data editor with chi(2) tests and Mann-Whitney U-tests. There were 486 patients with 329 OR (293 males, 36 females) with median age of 72 years with median diameter 6.3 cm and 157 EVAR (148 males, 9 females) with median age 75 years with median diameter 6.1 cm. Mortality was 13 (4%) for OR and 5 (3.2%) for EVAR (three of whom were in the UK EVAR 2 trial). Blood loss was significantly less for EVAR 500 ml vs. 1500 ml for OR. Sixty-five (19.8%) patients with OR had significantly more peri-operative complications compared with 14 (8.9%) with EVAR. The length of stay in hospital was significantly less for EVAR. This non-randomised study shows that although EVAR does not have a statistically significantly lower mortality, it does have statistically significantly lower complication rates compared with OR. EVAR can be achieved with good primary success, but long-term follow-up is essential to assess durability.

Treatment of mycotic aortic aneurysms with endoluminal grafts.

PURPOSE: To report the benefit of endoluminal repair of mycotic aortic aneurysms and highlight the need for a registry. METHODS: Nine patients (five female) were identified over 5 years (1998-2003) as having presumed mycotic aortic aneurysms (12 in total) suitable for endoluminal grafting. A total of nine thoracic and three abdominal were grafted and followed up for a median of 36 months. RESULTS: Six of the aneurysms have resolved and one was converted to an open repair. There was one early death from rupture of a second undiagnosed aneurysm and two late deaths from rupture due to persistent inflammation. Long-term antibiotics have not been mandatory to ensure survival. CONCLUSIONS: Mycotic aortic aneurysms of the thoracic and abdominal aorta do benefit from endoluminal repair, particularly when arising in previously normal aortic tissue. Endoluminal grafting also has a role in the palliation of secondarily infected aortas and so to prove its efficacy in the treatment of all these rare cases a registry is required.

Endovascular treatment of acute type B aortic dissection.

Intervention is currently reserved in acute aortic dissection for Stanford Type A and for complications of Type B. Endovascular techniques such as fenestration of the intimal flap and stenting of vessel origins have been used to alleviate end-organ ischaemia due to compromised branches. The introduction of stent grafts has offered a realistic alternative to surgery for Type B dissections. Closure of the primary entry tear encourages thrombosis of the false lumen, which is associated with good long-term outcome. Many questions remain unanswered and randomised controlled trials need to be performed to establish the role of stent grafts in uncomplicated Type B dissections, and the use of bare stents to encourage thrombosis of the more distal false lumen. Improvements in the design and engineering of stent grafts may help to establish endoluminal repair as the first line treatment of aortic dissection.

Comparison of extra-anatomic bypass grafting with angioplasty for atherosclerotic disease of the supra-aortic trunks.

BACKGROUND: Symptomatic stenosis of the supra-aortic trunks (subclavian, innominate and common carotid arteries) can be treated by angioplasty/stenting or surgical bypass. The aim of this study was to compare the initial success and outcome of these two types of treatment. METHODS: A prospective database was used to collect information on the presentation, initial success, complications and outcome in 76 patients treated in a single centre between 1983 and 2003. RESULTS: Thirty-five surgical extra-anatomic bypasses were performed, 13 carotid to carotid, 14 carotid to subclavian, two carotid to axillary, three axillary to axillary, one subclavian to axillary and two subclavian to subclavian. One graft occluded after 19 years. No limbs were amputated and no patient had a stroke. The secondary patency rate was 97 per cent at a mean follow-up of 5 years. Forty-one angioplasties were attempted, 34 of the left subclavian, six of the right subclavian and one of the innominate artery. Angioplasty for six subclavian occlusions was unsuccessful. Twenty-seven of 33 arteries remained patent at a mean follow-up of 4 years after a successful endovascular procedure. CONCLUSION: Extra-anatomic bypass for supra-aortic trunk disease has a better patency than angioplasty, with a comparable complication rate.

Mid-term results for second-generation thoracic stent grafts.

BACKGROUND: Thoracic stent grafts offer an alternative to open surgery for thoracic aortic disease, but their long-term durability is unknown. This report includes mid-term follow-up for commercially available thoracic devices. METHODS: Data were collected prospectively for a series of endoluminal grafts used to treat thoracic aortic pathology. RESULTS: Between July 1997 and October 2002, 67 patients received thoracic stent grafts. Elective procedures incurred a 30-day mortality rate of 2 per cent (one of 42 patients) and urgent repair 16 per cent (four of 25). Paraplegia affected three (4 per cent) of 67 patients and three patients had a stroke. The median follow-up was 17 (range 2-64) months; four patients were lost. There were six late deaths, two from aneurysm rupture (rupture of a mycotic aneurysm at 5 months and stent migration at 28 months). Other device-related complications comprised three proximal endoleaks, one of which required open surgical correction with removal of the stent graft, and two distal endoleaks, which were successfully treated with distal extension cuffs. CONCLUSION: In the mid term, endoluminal repair of thoracic aortic pathology appears to be a safe alternative to open surgery, but continued surveillance is essential.

Results of urgent and emergency thoracic procedures treated by endoluminal repair.

INTRODUCTION: emergency surgery on the thoracic aorta is associated with a high mortality. Endovascular treatment for these patients may offer a realistic alternative to open surgery. METHOD: between 1997 and 2002 data was collected prospectively on all patients who underwent urgent or emergency endoluminal repair for thoracic aortic pathology. All patients had ruptured or were at risk of rupture, and had been assessed as high risk for open surgery. RESULTS: twenty-four patients required urgent/emergency stent grafts. The median age was 74 (range 17-90). Indications included: trauma (transection in 3 and traumatic dissection in 1), acute symptomatic type B dissection (4), symptomatic degenerative aneurysms (7), false aneurysms associated with infection (6), Takayasu's vasculitis causing rupture of the descending thoracic aorta (1), symptomatic aneurysm associated with chronic dissection (1) and a secondary aorto-oesophageal fistula (1). The 30-day survival was 83.3% (20/24) and the survival at 1 year was 70.8% (17/24). The median follow-up was 13.5 months (range 2-57). The complications included: transient paraplegia (1), non-disabling stroke (1), distal endoleak treated with an extension cuff (1) and a proximal endoleak (1) which required removal of the graft at open surgery. CONCLUSION: endoluminal repair of thoracic aortic disease requiring urgent/emergency treatment has encouraging results with low morbidity and mortality rates compared with open surgery. Long-term follow-up is required to assess the durability of the grafts.

Thoracic aortic stent grafts--early experience from two centres using commercially available devices.

OBJECTIVES: open surgical intervention for aneurysms of the distal arch and descending thoracic aorta is associated with high morbidity and mortality. Stent grafts offer an attractive alternative treatment for these aneurysms. The aim of this study was to assess the morbidity and mortality of endovascular treatment for these aneurysms with stent grafts. DESIGN, PATIENTS AND METHODS: a prospective observational study was performed of 37 consecutive patients treated from July 1997 to October 2000 (30 at Guy's and St. Thomas' and 7 at Sheffield). Indications included degenerative aneurysms (n=18), false aneurysm (5), acute dissection (4), aortic transection (4), aneurysm related to previous surgery for coarctation (3), chronic dissection (2) and traumatic dissection (1). Nineteen were performed as elective and 18 as non-elective procedures. RESULTS: three non-elective patients died in hospital (in-hospital and 30-day mortality 8%) and one suffered a stroke with spontaneous full recovery. No elective patient died. One patient with a persistent proximal endoleak required conversion to open repair at 6 weeks. Two patients with persistent flow into the sac at 24 h spontaneously thrombosed at subsequent 3 month follow-up. Two further patients developed new distal endoleaks at 3 months and required distal extension cuffs. One patient died at 28 months of aortic rupture. Serial CT scans had shown prolapse of the stent graft into the aneurysm sac and the patient died just before planned endovascular repair. No patient suffered paraplegia or renal failure. Intensive care facilities were only required for patients who needed them preoperatively. CONCLUSIONS: thoracic stent grafts can be performed with low morbidity and mortality. They offer a realistic alternative to open surgery. Long term follow up is required to assess their durability.