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A thoracic aortic aneurysm (TAA) is a potentially life-threatening condition with structural weakness of the aortic wall, which can progress to arterial dilatation and rupture. Today, both an increasing awareness of vascular disease and the access to tomographic imaging facilitate the diagnosis of TAA even in an asymptomatic stage. The risk of rupture for untreated aneurysms beyond a diameter of 5.6 cm ranges from 46% to 74% and the two-year mortality rate is greater than 70%, with most deaths resulting from rupture. Treatment options include surgical and non-surgical repair to prevent aneurysm enlargement and rupture. While most cases of ascending aortic involvement are subject to surgical repair (partially with valve-preserving techniques), aneurysm of the distal arch and descending thoracic aorta are amenable to emerging endovascular techniques as an alternative to classic open repair or to a hybrid approach (combining debranching surgery with stent grafting) in an attempt to improve outcomes.

While thoracic endovascular aortic repair is an effective treatment option for descending thoracic aorta pathology, it does have limitations. The main limitation is related to the anatomical difficulties when disease involves the aortic arch. A fenestrated, branched aortic stent graft and hybrid operation has been introduced to overcome this limitation, but it is a custom-made device and is time consuming to manufacture. Furthermore, these devices cannot be used in an emergency setting. We report two patients with massive descending thoracic aortic aneurysm and ruptured aortic dissection very near the aortic arch who underwent a procedure which we named the modified chimney technique. The modified chimney technique can be used as a treatment option in such an emergency situation or as a rescue procedure when aortic pathology is involved near the supra-aortic vessels.

A 71-year-old patient was admitted for synchronous aneurysms of the aortic arch, brachiocephalic trunk, and juxtarenal abdominal aorta involving the iliac arteries. The patient first underwent open surgical repair of the juxtarenal abdominal aortic aneurysm by means of aorto-bifemoral bypass. Three months later, he underwent off-pump surgical repair of the aneurysm of the brachiocephalic trunk and bypass grafting from the ascending aorta to the brachiocephalic trunk and the left common carotid artery, followed by successful exclusion of the aneurysm of the aortic arch by deployment of a Zenith TX1 custom-made endograft, inserted through a limb of the aorto-bifemoral graft.

Combined endovascular and open surgical treatment is an appealing new alternative to open surgical repair for complex aortic diseases. Debranching of the aortic arch enables endovascular grafting in this area, thereby avoiding cardiopulmonary bypass and circulatory arrest. Staged and simultaneous procedures should be considered for the treatment of complex aortic diseases even in poor-risk patients; however, due to the investigative characteristics of these procedures, patient selection and postoperative follow-up should be carried out with utmost attention.

We describe a new surgical technique adopted for the repair of Stanford type A aortic dissection. In order to minimize the risk of malperfusion caused by retrograde flow during cardiopulmonary bypass, we avoid femoral artery cannulation. On the hypothesis that it is best not to interfere with the hemodynamics of the dissection, we cannulate the dissected ascending aorta, in either the true or false lumen. We here report 2 cases of successful surgical treatment of Stanford type A aortic dissection. In both cases, the false lumen was cannulated under deep hypothermic circulatory arrest, without clamping the aorta. While the patient was cooling, a 10-mm GORE-TEX side arm was sutured to a Dacron graft prosthesis. Repair of the aortic arch was carried out 1st. The aortic cannula was inserted into the GORE-TEX side arm, the tubular prosthesis was cross-clamped, and cardiopulmonary bypass was reinstituted. After this, the aortic bulb was repaired as usual and the tubular prosthesis was sutured to the bulb. No postoperative cerebral complication occurred. Our experience must be confirmed by more cases and a longer follow up.

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Aortic rupture has a high mortality rate and can be considered a medical emergency. The standard treatment for aortic rupture is surgical repair. An aortic stent graft for a ruptured descending aorta is considered an effective alternative treatment. However, an aortic stent graft is difficult when the aortic aneurysm is in the aortic arch due to supra-aortic vessels. We report on a patient with a ruptured aortic arch aneurysm treated with a hybrid procedure, which involved a carotid to carotid bypass operation and an aortic stent graft. A 71-year-old male patient visited our cardiovascular center suffering from hemoptysis. The chest CT and aortography showed a 9 cm sized aortic arch aneurysm 0.5 cm distal to the left subclavian artery and a hemothorax in the left lung. The patient refused to undergo a full open operation. We performed a carotid to carotid bypass in advance, and two pieces of aortic stent grafts were placed across the left carotid artery and left subclavian artery. The follow up CT showed the aortic stent grafts, no endoleaks and no thrombus in the aortic arch aneurysm. The patient was discharged from the hospital without complication.

INTRODUCTION:

A case of thoracic-abdominal dissection after open surgical exclusion of an infrarenal aortic aneurysm is presented.

CASE PRESENTATION:

A 62-year-old woman was diagnosed with an infrarenal abdominal aortic aneurysm with a rapid increase in maximal diameter. She underwent surgery for aneurysm exclusion by an end-to-end aortoaortic bypass with Dacron collagen (Intervascular; WL Gore & Associates Inc, USA). After 15 days, she was admitted to the emergency department with intense epigastric and lumbar pain. Computed tomography angiography with contrast revealed an aortic dissection with origin in the proximal bypass anastomosis and cranial extension to the thoracic aorta. The true lumen at the level of the eighth thoracic vertebra was practically collapsed by the false lumen. The celiac trunk, and the mesenteric and renal arteries were perfused by the true lumen. After the acute phase of the aortic dissection, surgical repair was planned. Two paths of false lumen were found – one at the thoracic aorta and the second in the proximal bypass anastomosis. Surgical repair comprised two approaches. First, a Valiant Thoracic stent graft (Medtronic Inc, UK) was implanted distal from the left subclavian artery, expanding the collapsed true lumen and covering the false and dissected lumen. Second, an infrarenal Endurant abdominal stent graft (Medtronic Inc) was implanted. This second device was complemented with an aortic infrarenal extension using a Talent abdominal stent graft (Medtronic Inc) in the infrarenal aortic neck to achieve a hermetic seal. The postoperative clinical course was uneventful, and her symptoms were completely resolved in six months.

CONCLUSION:

Arteritis must be taken into account in young patients with high inflammatory markers. Covered stents and endoprosthetic devices seem to be effective methods to seal the dissected lumen.

We present the case of a 57-year-old woman who had an intramural hematoma of the ascending aorta and aortic arch. After initial blood pressure control and imaging studies, the patient underwent limited surgical repair that consisted of ascending aortic replacement. One week postoperatively, the aortic arch hematoma progressed to a full dissection that extended into the proximal descending aorta. Emergent aortic arch replacement was required.

Current world medical literature regarding thoracic aortic intramural hematoma is presented. This case supports the treatment of intramural hematomas of the ascending aorta and arch by surgical replacement of both segments with a Dacron graft, with the patient under deep hypothermic circulatory arrest. (Tex Heart Inst J 2003;30:325–7)

Introduction

Current strategies for repair of descending thoracic aortic aneurysms consist of open repair with surgical graft replacement or thoracic endovascular aortic repair. We review and update our overall experience in aortic thoracic diseases and specifically analyzed our outcomes with thoracic endovascular aortic repair in patients with descending thoracic aortic aneurysms.

Methods

From 1993 to present a total of 1144 patients were treated in our Center for pathology involving the thoracic aorta. Since 1998, 322 patients underwent thoracic endovascular aortic repair, and among this group, in 188 cases the descending aorta was involved. In 74% of patients treated for a descending thoracic aortic lesion, a degenerative aneurysm was observed.

Results

In patients with descending thoracic aortic aneurysms receiving thoracic endovascular aortic repair, our technical success rate, i.e. deployment of endograft with complete exclusion of the lesion/minimal endoleak, was 99.5% (one case required emergent open conversion) with a perioperative mortality of 2.6% (five patients). The rate of spinal cord ischemia, manifesting either as paraplegia or paraparesis, was 4.7%. Delayed onset spinal cord ischemia ameliorated with adequate arterial pressure and cerebrospinal fluid drainage.

Conclusions

Our experience of selected patients undergoing thoracic endovascular aortic repair of descending thoracic aorta aneurysms is satisfactory with very low mortality and morbidity. A large use of thoracic endovascular aortic repair is foreseen in the next future.

Introduction

Current strategies for operative treatment of a thoracic aortic aneurysm consist of open repair with surgical graft replacement or endovascular exclusion. To reduce mortality and morbidity of open repair, a multimodal approach has gradually evolved by maximizing organ protection.

Methods

On a total of 1108 patients treated in our Center from 1993 for pathology involving the thoracic aorta, we reviewed the prospectively collected data of 194 consecutive patients who underwent open thoracic aortic aneurysm repair, 104 (54%) for degenerative aneurysms, 65 (34%) for dissections, 25 (12%) for other pattern of disease. Left Heart Bypass was used in 82% of cases, clamp and sew technique in 16%, hypotermic circulatory arrest in 2%.

Results

Overall perioperative mortality was 4.1%. The rate of pulmonary complications was 8.8%. The rate of cardiac complications, i.e. new onset myocardial necrosis demonstrated by positive blood tests, was 6.2%. The rate of renal complications was 7.2%. Cerebrovascular accident, defined as a new neurologic deficit lasting more than 24 hours confirmed by imaging, occurred in 2.0% of patients. The rate of spinal cord ischemia, manifesting either as paraplegia or paraparesis, was 4.6%.

Conclusions

Mortality and morbidity rates of open thoracic aortic aneurysm repair are currently satisfactory especially in fit patients. In order to define surgical indications and the role of endovascular repair, consideration of age of the patient, comorbidity, symptoms, life expectancy, likely quality of life (if asymptomatic), aortic diameter, aneurysm morphology, aneurysm extent, suitability of landing zones, and operator experience are all distinctly relevant.

A 73-year-old woman with a history of chronic hypertension and severe chronic obstructive pulmonary disease, presented to a district general hospital with thoracic pain in a profound state of shock. She was diagnosed with cardiac tamponade, severe mitral regurgitation, and Stanford type A (Debakey type I) intramural hematoma. Her ascending aorta was of a significant size and therefore emergent repair was done to replace the ascending aorta and mitral valve. After 6 months, an increased aneurysmal size of 6.0 cm was observed in a follow up contrast-enhanced computed tomography angiography. The patient was successfully treated by a staged hybrid procedure involving initial supra-aortic reconstruction.

We report a case of ruptured mycotic aneurysm involving innominate artery requiring an urgent surgical treatment. A 62-yr-old woman presented with fever and dyspnea. Previously, she was diagnosed with colon cancer and received right hemicolectomy and one cycle of adjuvant chemotherapy. On echocardiogram, pericardial effusion was noted and emergency pericardiocentesis was performed. CT scan revealed aortic aneurysm involving ascending aorta and innominate artery, and thrombi surrounding those structures. Patch repair of the defect in the ascending aorta and ringed Goretex graft to bypass the innominate and ascending aorta were performed. We believe that this is the first case of ruptured mycotic aneurysm involving innominate artery.

Twenty-four cases of acute type-A aortic dissection with aortic valvular insufficiency were treated in our institution by means of an emergency operation in which the aortic valve, ascending aorta, and aortic arch were resected and replaced with a valved conduit that had been lengthened with a tubular Dacron graft. The procedure included the use of deep hypothermia for cerebral protection, as well as extracorporeal circulation. Aortic resection was performed from the aortic valve to the origin of the descending thoracic aorta; the aortic graft was anastomosed proximally to the valve annulus and distally to the descending aorta. The carotid orifices were connected to the side of the graft in a single tissue button. The coronary arteries were then reconnected by means of double venous bypass grafts to the innominate artery, to allow for inclusion of the graft. Within 1 month after operation, four patients died of the consequences of dissection. Six months postoperatively, one patient succumbed to an infarction. Six months to 5 years after operation, the remaining 19 patients are still alive. On the basis of this experience, we believe that acute type-A aortic dissection with aortic valvular insufficiency should be treated during the first hours after the onset of symptoms. The above-described procedure proved effective in the control of bleeding, which is the major risk in emergency operations of this type. (Texas Heart Institute Journal 1987; 14:418-421)

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Acute type A aortic dissection can be complicated by visceral malperfusion and is associated with a significant surgical morbidity and mortality. We describe a case of successful management of a complex acute type A dissection with mesenteric and lower limb ischemia treated with endovascular thoracic stenting and femoro-femoral crossover bypass grafting followed by aortic arch repair. To accomplish this, we applied a staged therapeutic approach using serial lactate measurements to assess the adequacy of peripheral perfusion and metabolic status prior to surgical repair of the proximal dissection.

Ruptured aortic arch aneurysm is a life threatening disease. Surgical repair has an high perioperative mortality rate and totally endovascular treatment is a challenge. Hybrid repair has been proposed as a valuable approach. We report the case of a patient with a contained rupture of aortic arch aneurysm. We treated him with a debranching of supraortic vessels with carotid-carotid and carotid-subclavian bypass and deployment of two enodgrafts in two different times. We consider hybrid treatment for arch and hemiarch a feasible option for aortic arch aneurysms in non emergent and in an emergency setting with an improvement in perioperative morbidity and mortality.

Management of extensive, chronic, dissecting aortic aneurysms after prior repair of the ascending aorta presents a technical challenge for surgeons. A symptomatic 64-year-old patient was admitted for elective surgical repair of an aortic annular dilatation, causing severe aortic regurgitation, and a Crawford type II extended thoracoabdominal aneurysm, 4 years after he underwent primary repair of an acute aortic dissection. The aorta was diffusely dilated, and there were no sites beyond the distal aortic arch where anastomosis could be performed. We successfully performed total aortic replacement with a 2-stage strategy, using an arch translocation technique and an intra-arch elephant-trunk technique.

Executive Summary

Objective

To conduct an assessment on endovascular repair of descending thoracic aortic aneurysm (TAA).

Clinical Need

Aneurysm is the most common condition of the thoracic aorta requiring surgery. Aortic aneurysm is defined as a localized dilatation of the aorta. Most aneurysms of the thoracic aorta are asymptomatic and incidentally discovered. However, TAA tends to enlarge progressively and compress surrounding structures causing symptoms such as chest or back pain, dysphagia (difficulty swallowing), dyspnea (shortness of breath), cough, stridor (a harsh, high-pitched breath sound), and hoarseness. Significant aortic regurgitation causes symptoms of congestive heart failure. Embolization of the thrombus to the distal arterial circulation may occur and cause related symptoms. The aneurysm may eventually rupture and create a life-threatening condition.

The overall incidence rate of TAA is about 10 per 100,000 person-years. The descending aorta is involved in about 30% to 40% of these cases.

The prognosis of large untreated TAAs is poor, with a 3-year survival rate as low as 25%. Intervention is strongly recommended for any symptomatic TAA or any TAA that exceeds twice the diameter of a normal aorta or is 6 cm or larger. Open surgical treatment of TAA involves left thoracotomy and aortic graft replacement. Surgical treatment has been found to improve survival when compared with medical therapy. However, despite dramatic advances in surgical techniques for performing such complex operations, operative mortality from centres of excellence are between 8% and 20% for elective cases, and up to 50% in patients requiring emergency operations. In addition, survivors of open surgical repair of TAAs may suffer from severe complications. Postoperative or postprocedural complications of descending TAA repair include paraplegia, myocardial infarction, stroke, respiratory failure, renal failure, and intestinal ischemia.

The Technology

Endovascular aortic aneurysm repair (EVAR) using a stent graft, a procedure called endovascular stent-graft (ESG) placement, is a new alternative to the traditional surgical approach. It is less invasive, and initial results from several studies suggest that it may reduce mortality and morbidity associated with the repair of descending TAAs.

The goal in endovascular repair is to exclude the aneurysm from the systemic circulation and prevent it from rupturing, which is life-threatening. The endovascular placement of a stent graft eliminates the systemic pressure acting on the weakened wall of the aneurysm that may lead to the rupture. However, ESG placement has some specific complications, including endovascular leak (endoleak), graft migration, stent fracture, and mechanical damage to the access artery and aortic wall.

The Talent stent graft (manufactured by Medtronic Inc., Minneapolis, MN) is licensed in Canada for the treatment of patients with TAA (Class 4; licence 36552). The design of this device has evolved since its clinical introduction. The current version has a more flexible delivery catheter than did the original system. The prosthesis is composed of nitinol stents between thin layers of polyester graft material. Each stent is secured with oversewn sutures to prevent migration.

Review Strategy

Objectives

To compare the effectiveness and cost-effectiveness of ESG placement in the treatment of TAAs with a conventional surgical approach

To summarize the safety profile and effectiveness of ESG placement in the treatment of descending TAAs

Measures of Effectiveness

Primary Outcome

Mortality rates (30-day and longer term)

Secondary Outcomes

Technical success rate of introducing a stent graft and exclusion of the aneurysm sac from systemic circulation

Rate of reintervention (through surgical or endovascular approach)

Measures of Safety

Complications were categorized into 2 classes:

Those specific to the ESG procedure, including rates of aneurysm rupture, endoleak, graft migration, stent fracture, and kinking; and

Those due to the intervention, either surgical or endovascular. These include paraplegia, stroke, cardiovascular events, respiratory failure, real insufficiency, and intestinal ischemia.

Inclusion Criteria

Studies comparing the clinical outcomes of ESG treatment with surgical approaches

Studies reporting on the safety and effectiveness of the ESG procedure for the treatment of descending TAAs

Exclusion Criteria

Studies investigating the clinical effectiveness of ESG placement for other conditions such as aortic dissection, aortic ulcer, and traumatic injuries of the thoracic aorta

Studies investigating the aneurysms of the ascending and the arch of the aorta

Studies using custom-made grafts

Literature Search

The Medical Advisory Secretariat searched The International Network of Agencies for Health Technology Assessment and the Cochrane Database of Systematic Reviews for health technology assessments. It also searched MEDLINE, EMBASE, Medline In-Process & Other Non-Indexed Citations, and Cochrane CENTRAL from January 1, 2000 to July 11, 2005 for studies on ESG procedures. The search was limited to English-language articles and human studies.

One health technology assessment from the United Kingdom was identified. This systematic review included all pathologies of the thoracic aorta; therefore, it did not match the inclusion criteria. The search yielded 435 citations; of these, 9 studies met inclusion criteria.

Summary of Findings

Mortality

The results of a comparative study found that in-hospital mortality was not significantly different between ESG placement and surgery patients (2 [4.8%] for ESG vs. 6 [11.3%] for surgery).

Pooled data from case series with a mean follow-up ranging from 12 to 38 months showed a 30-day mortality and late mortality rate of 3.9% and 5.5%, respectively. These rates are lower than are those reported in the literature for surgical repair of TAA.

Case series showed that the most common cause of early death in patients undergoing endovascular repair is aortic rupture, and the most common causes of late death are cardiac events and aortoesophageal or aortobronchial fistula.

Technical Success Rate

Technical success rates reported by case series are 55% to 100% (100% and 94.4% in 2 studies with all elective cases, 89% in a study with 5% emergent cases, and 55% in a study with 42% emergent cases).

Surgical Reintervention

In the comparative study, 3 (7.1%) patients in the ESG group and 14 (26.5%) patients in the surgery group required surgical reintervention. In the ESG group, the reasons for surgical intervention were postoperative bleeding at the access site, paraplegia, and type 1 endoleak. In the surgical group, the reasons for surgery were duodenal perforation, persistent thoracic duct leakage, false aneurysm, and 11 cases of postoperative bleeding.

Pooled data from case series show that 9 (2.6%) patients required surgical intervention. The reasons for surgical intervention were endoleak (3 cases), aneurysm enlargement and suspected infection (1 case), aortic dissection (1 case), pseudoaneurysm of common femoral artery (1 case), evacuation of hematoma (1 case), graft migration (1 case), and injury to the access site (1 case).

Endovascular Revision

In the comparative study, 3 (7.1%) patients required endovascular revision due to persistent endoleak.

Pooled data from case series show that 19 (5.3%) patients required endovascular revision due to persistent endoleak.

Graft Migration

Two case series reported graft migration. In one study, 3 proximal and 4 component migrations were noted at 2-year follow-up (total of 5%). Another study reported 1 (3.7%) case of graft migration. Overall, the incidence of graft migration was 2.6%.

Aortic Rupture

In the comparative study, aortic rupture due to bare stent occurred in 1 case (2%). The pooled incidence of aortic rupture or dissection reported by case series was 1.4%.

Postprocedural Complications

In the comparative study, there were no statistically significant differences between the ESG and surgery groups in postprocedural complications, except for pneumonia. The rate of pneumonia was 9% for those who received an ESG and 28% for those who had surgery (P = .02). There were no cases of paraplegia in either group. The rate of other complications for ESG and surgery including stroke, cardiac, respiratory, and intestinal ischemia were all 5.1% for ESG placement and 10% for surgery. The rate for mild renal failure was 16% in the ESG group and 30% in the surgery group. The rate for severe renal failure was 11% for ESG placement and 10% for surgery.

Pooled data from case series show the following postprocedural complication rates in the ESG placement group: paraplegia (2.2%), stroke (3.9%), cardiac (2.9%), respiratory (8.7%), renal failure (2.8%), and intestinal ischemia (1%).

Time-Related Outcomes

The results of the comparative study show statistically significant differences between the ESG and surgery group for mean operative time (ESG, 2.7 hours; surgery, 5 hours), mean duration of intensive care unit stay (ESG, 11 days; surgery, 14 days), and mean length of hospital stay (ESG, 10 days; surgery, 30 days).

The mean duration of intensive care unit stay and hospital stay derived from case series is 1.6 and 7.8 days, respectively.

Ontario-Based Economic Analysis

In Ontario, the annual treatment figures for fiscal year 2004 include 17 cases of descending TAA repair procedures (source: Provincial Health Planning Database). Fourteen of these have been identified as “not ruptured” with a mean hospital length of stay of 9.23 days, and 3 cases have been identified as “ruptured,” with a mean hospital length of stay of 28 days. However, because one Canadian Classification of Health Interventions code was used for both procedures, it is not possible to determine how many were repaired with an EVAR procedure or with an open surgical procedure.

Hospitalization Costs

The current fiscal year forecast of in-hospital direct treatment costs for all in-province procedures of repair of descending TAAs is about $560,000 (Cdn). The forecast in-hospital total cost per year for in-province procedures is about $720,000 (Cdn). These costs include the device cost when the procedure is EVAR (source: Ontario Case Costing Initiative).

Professional (Ontario Health Insurance Plan) Costs

Professional costs per treated patient were calculated and include 2 preoperative thoracic surgery or EVAR consultations.

The professional costs of an EVAR include the fees paid to the surgeons, anesthetist, and surgical assistant (source: fee service codes). The procedure was calculated to take about 150 minutes.

The professional costs of an open surgical repair include the fees of the surgeon, anesthetist, and surgical assistant. Open surgical repair was estimated to take about 300 minutes.

Services provided by professionals in intensive care units were also taken into consideration, as were the costs of 2 postoperative consultations that the patients receive on average once they are discharged from the hospital. Therefore, total Ontario Health Insurance Plan costs per treated patient treated with EVAR are on average $2,956 (ruptured or not ruptured), as opposed to $5,824 for open surgical repair and $6,157 for open surgical repair when the aneurysm is ruptured.

Conclusions

Endovascular stent graft placement is a less invasive procedure for repair of TAA than is open surgical repair.

There is no high-quality evidence with long-term follow-up data to support the use of EVAR as the first choice of treatment for patients with TAA that are suitable candidates for surgical intervention.

However, short- and medium-term outcomes of ESG placement reported by several studies are satisfactory and comparable to surgical intervention; therefore, for patients at high risk of surgery, it is a practical option to consider. Short- and medium-term results show that the benefit of ESG placement over the surgical approach is a lower 30-day mortality and paraplegia rate; and shorter operative time, ICU stay, and hospital stay.

ABSTRACT

The emergence of endovascular repair of the thoracic aorta (TEVAR) quickly followed the development of technology for the exclusion of infrarenal abdominal aortic aneurysms. Stent grafts comprised of metal struts covered with fabric made of Dacron/polyester or polytetrafluoroethylene were developed for the purpose of achieving an adequate seal at the proximal and distal aspects of thoracic aneurysms, thus excluding sac flow. The recognition of the decreased morbidity of this approach compared with open repair was readily apparent, as it avoided left thoracotomy, aortic cross-clamping, and left heart bypass. Since then, TEVAR is increasingly being used for other aortic pathologies such as complicated type B dissection, traumatic aortic transection, and aneurysmal disease extending into the arch or visceral segment, requiring debranching procedures.

Background

A 24-year-old man presented with previously diagnosed Marfan’s syndrome. Since the age of 9 years, he had undergone eight cardiovascular procedures to treat rapidly progressive aneurysms, dissection and tortuous vascular disease involving the aortic root and arch, the thoracoabdominal aorta, and brachiocephalic, vertebral, internal thoracic and superior mesenteric arteries. Throughout this extensive series of cardiovascular surgical repairs, he recovered without stroke, paraplegia or renal impairment.

Investigations

CT scans, arteriogram, genetic mutation screening of transforming growth factor β receptors 1 and 2.

Diagnosis

Diffuse and rapidly progressing vascular disease in a patient who met the diagnostic criteria for Marfan’s syndrome, but was later rediagnosed with Loeys–Dietz syndrome. Genetic testing also revealed a de novo mutation in transforming growth factor β receptor 2.

Management

Regular cardiovascular surveillance for aneurysms and dissections, and aggressive surgical treatment of vascular disease.

A 50-year-old man, who 9 months earlier had undergone emergency operation for acute type I aortic dissection, was readmitted to our hospital with the diagnosis of an enlarging aneurysm of the false lumen involving the transverse arch and the proximal third of the descending thoracic aorta, due principally to redissection at the distal suture line of the ascending aortic graft. Replacement of the aortic arch and proximal descending thoracic aorta was performed by using the retrograde "pull-through" technique with hypothermic circulatory arrest and retrograde cerebral perfusion. Although circulatory arrest lasted 110 minutes, the patient was extubated on the 2nd postoperative day and had no central or peripheral neurologic damage. Mild, transitory renal dysfunction was observed in the 1st postoperative week, and the patient was discharged on the 18th postoperative day. He is asymtomatic at 15 postoperative months. Deep hypothermia and retrograde cerebral perfusion proved effective despite prolonged circulatory arrest. The retrograde "pull-through" technique is an effective method of replacing the entire thoracic aorta and should probably be considered for single-stage repair of acute type I aortic dissection with multiple intimal tears.

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Sixty eight patients with aneurysms of the thoracic aorta were studied. Forty one had aortic dissection, 24 had dilatation only, and three had transverse aortic rupture. Sixteen had Marfan's syndrome; 17 had hypertension; and in eight there were other causal factors. In 17 the cause of the aneurysm was unknown. Histological examination did not help to establish the cause of aneurysm. Echocardiography failed to detect dissection of the ascending aorta in four (21%) out of 19 cases studied. The mortality rate in the whole series was 26%. Early (operative and hospital) and late deaths occurred in 20% and 6% of patients respectively. The early mortality rate was 40% in the 24 emergency cases of dissection of the ascending aorta, 9% in patients operated on for dilatation of the ascending and transverse aorta without dissection, and 8% in patients with chronic dissection of the ascending aorta who had elective operation. Early and late mortality rates were no higher in patients with Marfan's disease than in any of the other groups. It is suggested that contrast enhanced computer tomography should be performed in all patients with pronounced aortic root dilatation and in patients with Marfan's disease with symptoms which suggest dissection, even if they have only slight aortic root dilatation. Preventive replacement of the ascending aorta should be considered in more patients to reduce the number of emergency operations, in which the mortality rate is high. There is no definite limit of aortic root dilatation above which preventive replacement of the ascending aorta should be routinely considered.

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After traditional treatment of acute type I aortic dissection, the possible persistence of residual false lumen in the untouched distal aorta can increase the risk of death.

This case report presents an example of single-stage complete hybrid repair of acute type I aortic dissection via surgical interposition of an ascending aortic tube-graft and reconstruction of the supra-aortic branches to enable circulatory inflow from the ascending aortic graft, which was followed by endovascular stenting of the arch and of the descending and thoracic aortic segments. This procedure was performed with partial sternotomy and without circulatory arrest, to improve early and late outcomes.

Unfortunately, there is no extensive experience with application of the technique described here, and we are contributing a report of only a single case. Nevertheless, we hope that this description of a single-stage complete repair of aortic dissection might lead to further application and eventually to fewer deaths in patients with acute type I aortic dissection. We suggest this approach for use especially in high-risk patients.

OBJECTIVE

Thoracic, arch, and proximal descending thoracic aorta diseases are still considered an enormous challenge. The hybrid approach developed in recent years (supra-aortic trunks debranching and thoracic endovascular repair aortic repair; TEVAR) may improve the morbidity and mortality of the population at risk. The aim of this study was to analyze retrospectively our experience in the hybrid treatment of aortic-arch aneurysms and dissections.

METHOD

We carried out a retrospective review of 27 patients who required a surgical debranching of the supra-aortic trunks and a TEVAR in the management of the aortic arch and proximal descending thoracic aortic disease. The aortic lesions included 18 degenerative arch-aortic aneurysms, four complicated aortic dissections, two subclavian artery aneurysms, and three penetrating atherosclerotic ulcers. Technical success was achieved in all patients.

RESULTS

The 30-day mortality rate was 11.1% (3/27). Mean follow-up was 16.7 months (range, 1–56), and the survival rate was 77.8%. The endoleaks’ rate was 3.7% (1/27), due to a stent-graft migration.

CONCLUSION

Hybrid approaches may represent an alternative option in the treatment of complex aortic lesions involving the arch and the proximal descending thoracic aorta in high-risk patients and emergency cases. However, the promising early results need to be confirmed by longer follow-up and larger comparative series.

Endovascular repair of inflammatory aortic aneurysms has been reported as an alternative to open surgical treatment. In selective cases, adjunctive bypass surgery may be required to provide an adequate landing zone. We report a case of endovascular repair of an inflammatory aortic aneurysm in a patient with Behçet's disease using a carotid-carotid bypass graft to provide an adequate landing zone. A 45-yr-old man with a voice change was referred to our hospital with the diagnosis of saccular aneurysm of the distal aortic arch resulting from vasculitis. Computed tomography showed a thoracic aortic aneurysm with thrombosis. Right to left carotid-carotid bypass grafting was performed. After 8 days, the patient underwent an endovascular stent graft placement distal to the origin of the innominate artery. The patient was discharged with medication and without postoperative complications after 5 days. Hybrid endovascular treatment may be suitable a complementary modality for repairing inflammatory aortic aneurysms.

Background

Aortic pathology progression and/or procedure related complications following endovascular repair should always be considered mostly in older patients. We herein describe a hybrid procedure for treatment of rapidly expanding thoracoabdominal aneurysm following endovascular treatment of a descending thoracic aortic aneurysm in an older patient.

Case presentation

A 82-year-old man at 18 months after endovascular surgery for a contained rupture of descending thoracic aortic aneurysm revealed a type IV thoracoabdominal aneurysm with significant increase of the aortic diameters at superior mesenteric and renal artery levels. A hybrid approach consisting of preventive visceral vessel revascularization and endovascular repair of entire abdominal aorta was performed. Under general anaesthesia and by xyphopubic laparotomy, the infrarenal aneurysmatic aorta and common iliac arteries were replaced by a bifurcated woven prosthetic graf. From each of the prosthetic branches two reverse 14x7 mm bifurcated PTFE prosthetic grafts were anastomized to both renal arteries and to the celiac axis and superior mesenteric artery, respectively. Vessel ischemia was restricted to the time required for anastomosis. Three 10 cm Gore endovascular stent-grafts for a total length of 15 cm, were used. The overlapping of the stent-grafts was carried out from the bottom upwards, starting from the aorto-iliac prosthetic body up to the healthy segment of thoracic aorta, 40 mm from the previous stent-grafts.

The patient was discharged on the 9th postoperative day.

Conclusion

This technique offers the advantage of a less invasive treatment, reducing the risk of paraplegia, visceral ischaemia and pulmonary complications, mostly in older patients.

Aortic aneurysm is one several well-known cardiovascular complications in patients with autosomal dominant polycystic kidney disease (ADPCKD). Commonly affected site of aortic aneurysm and its related dissection in ADPCKD is abdominal aorta. Long standing hypertension, haemodialysis, old age are closely related with discovering of aortic aneurysm and dissection in ADPCKD. However, thoracic aortic aneurysms and its related severe aortic regurgitations (ARs) are rare in younger patients suffering from ADPCKD, especially ones who have normal renal function. Here, we report a case involving a 27-year-old Asian male patient with severe AR due to an ascending aneurysm of the thoracic aorta associated with ADPCKD. The patient had normal renal function without Marfan's habitus. The AR and thoracic aortic aneurysm were corrected surgically.