覆膜血管内支架置入治疗B型主动脉夹层的临床应用研究

目的:评价覆膜血管内支架置入治疗B型主动脉夹层的疗效。方法:180例B型主动脉夹层,男158例,女22例,年龄(50．4±10．9)岁。术前行计算机体层摄影术(CT)增强扫描检查。在数字减影血管造影(DSA)监测下选用覆膜血管内支架封堵夹层原发破口。置入后即刻重复DSA检查。随访行胸部X线平片与CT增强扫描检查。结果:技术成功率99．4％(179／180),1例支架位置过高,行外科升主动脉-左颈总动脉-左锁骨下动脉搭桥术。术后即刻造影160例无内漏,18例少量内漏,2例中量内漏近段加短袖套支架(Cuff)后内漏消失;降主动脉真腔明显扩大,腹主动脉及分支供血均有不同程度的改善。术后随访(18．6±7．2)个月,18例少量内漏12例6个月内消失、6例无加重;21例支架段假腔消失,余者近端假腔内均有血栓形成;1例支架远端形成假性动脉瘤破裂死亡。结论:覆膜血管内支架置入治疗B型主动脉夹层安全有效,近、中期疗效满意,远期疗效有待于进一步观察。     

JOTEC E-vita覆膜支架在胸主动脉腔内治疗中的应用

目的：总结JOTEC E-vita覆膜支架在胸主动脉疾病腔内治疗中的应用经验.方法：自2009年2月至2012年9月,共有70例患者采用JOTEC E-vita覆膜支架行胸主动脉腔内治疗,男性67例,女性3例,年龄32～ 82岁,平均（57.3±10.8）岁.39例为Standford B型主动脉夹层,25例为主动脉穿通性溃疡并壁间血肿,2例为主动脉假性动脉瘤,4例为胸主动脉瘤.65例采用外科切开股动脉置入,5例为完全穿刺下置入.11例因近端锚定区不足覆盖左锁骨下动脉,其中9例采用“烟囱”技术.30例覆膜支架覆盖主动脉的长度＜230mm,定义为A组;40例覆膜支架覆盖主动脉长度≥230mm,定义为B组.术后1,3,6及12个月,之后每年均行主动脉CTA复查,观察有无内漏、病变是否隔绝完全.比较两组围手术期及随访期病死率、内漏发生率及围手术期均无脊髓缺血发生.结果：所有患者均成功的置入覆膜支架,技术成功率100％.除5例置入2枚覆膜支架外,其余均仅置入1枚覆膜支架,共置入75枚覆膜支架.支架锥形头撤除困难2例;支架打折12例;术后即刻内漏17例,内漏发生率为24.3％（17/70）.A、B两组患者围手术期均无脊髓缺血发生.随访2～40个月,随访期间病死率为1.43％ （1/70）,死因为再发Standford A型主动脉夹层.结论：长段胸主动脉病变,尤其是主动脉穿通性溃疡伴（或不伴）壁间血肿,为应用JOTEC E-vita覆膜支架的最佳适应症,并不增加脊髓缺血的发生率.     

腹主动脉病变腔内修复并发症及治疗

目的:总结腹主动脉病变腔内治疗的疗效,探讨并发症发生原因及防治措施。方法:2012年1月至2014年12月,连续性覆膜支架腔内修复术的腹主动脉病变患者224例,其中腹主动脉真性动脉瘤205例,腹主动脉假性动脉瘤5例,腹主动脉瘤破裂4例,腹主动脉穿通性溃疡4例,孤立性腹主动脉夹层2例,腹主动脉腔内修复(EVAR)术后二次介入治疗4例;男性206例,女性18例;年龄36~90岁,平均(69.0±9.2)岁。患者出院前,术后1、6、12个月及每年行主动脉CTA检查。统计不同时间段的并发症,计算其发生率及病死率。结果:术中无死亡,技术成功率100%。围手术期病死率及主要并发症率分别为0.9%(2/224)和10.3%(23/224)。围手术期主要并发症包括内漏13例、支架移位2例,瘤颈医源性夹层1例,脊髓缺血1例,入路血管损伤2例,外周血管血栓形成3例,严重低血红蛋白血症1例。随访12~48个月,随访期间病死率及并发症率分别为8.9%(20/224)和4.5%(10/224)。随访期间主要并发症包括内漏4例、间歇性跛行3例、外周血管血栓形成或闭塞2例及腹主动脉假性动脉瘤感染复发1例。结论:采用覆膜支架置入腔内修复术治疗腹主动脉瘤安全可靠,但严重并发症不客忽视。严格把握适应证、术前精确测量及术中细致操作可在一定程度上降低并发症发生。此外,应重视术后定期复查以及时发现并处理相关并发症。     

覆膜支架腔内隔绝术治疗高龄胸腹主动脉瘤的疗效

目的：探讨主动脉腔内隔绝术治疗高龄患者胸腹动脉瘤的临床效果。方法：回顾性分析我院2004年2月至2011年7月25例实行覆膜支架腔内隔绝术的高龄胸腹主动脉瘤患者（年龄均≥65岁）的临床资料,总结手术效果,术后并发症和死亡率。结果：24例（96％）康复出院,术后并发症发生率24％（6／25）,病死率4％（1／25）,平均住院时间为（14．36±2．46）d,术后住院时间为（9．50±1．58）d。结论：覆膜支架腔内隔绝术可作为治疗高龄主动脉瘤首选方式,但须严格把握手术指征。     

同期行降主动脉和腹主动脉腔内修复术

目的：讨论病变同时累及降主动脉和腹主动脉的主动脉疾患,同期行胸主动脉和腹主动脉腔内修复术对脊髓缺血的影响.方法：回顾性分析2009年2月至2013年1月间,本中心收住院的病变同时累及降主动脉和腹主动脉的18例患者,其中男性17例,女性1例,年龄50 ～ 78岁,平均（61.13±7.25）岁,其中胸主动脉瘤伴腹主动脉瘤10例,胸主动脉穿通溃疡伴腹主动脉瘤5例,胸主动脉及腹主动脉均为穿通溃疡2例,胸主动脉夹层伴腹主动脉瘤1例,全部采用双侧股动脉切开行主动脉腔内修复术,其中胸主动脉根据病变范围置入1枚或2枚覆膜支架（部分重叠）,腹主动脉置入分体式或一体式支架.结果：术后1例脊髓供血障碍（5.6％）,经脱水及神经营养性治疗后痊愈,随访3～24个月,无支架移位或内漏等并发症发生.结论：应用覆膜支架同时覆盖降主动脉和腹主动脉处理主动脉病变是安全、有效的.个别可引起脊髓缺血,应进行积极预防和及时处理.     

腔内隔绝术治疗112例Stanford B型主动脉夹层动脉瘤患者疗效观察

目的评价主动脉夹层腔内隔绝术治疗Stanford B型主动脉夹层动脉瘤的院内及长期临床疗效。方法选择行主动脉腔内隔绝术治疗Stanford B型主动脉夹层患者112例。经股动脉切开置入覆膜支架封堵胸主动脉破裂口,置入后冠状动脉造影检查证实疗效,术后随访平均(39±18)个月。分析其临床特点及疗效。结果手术成功112例,共置入覆膜支架119枚。左锁骨下动脉完全被封闭8例,合并严重狭窄病变的冠心病患者完成PCI 16例,主动脉腔内隔绝术后综合征19例,术后1个月内夹层破裂死亡3例。随访3个月,所有患者内膜破裂口封闭,胸降主动脉和腹主动脉真腔扩大,假腔内血栓形成,支架位置、形态正常。术后明显残余内漏10例,3个月自行封闭。术后6个月,再发升主动脉夹层3例,其中行升主动脉外科手术1例,截瘫1例,胃癌多器官转移死亡1例。术后1年迟发性内漏1例。结论腔内隔绝术治疗Stanford B型主动脉夹层动脉瘤的院内及长期疗效满意。     

腔内隔绝术治疗胸主动脉假性动脉瘤16例临床分析

目的探讨主动脉假性动脉瘤的临床特征,评价腔内隔绝术治疗主动脉假性动脉瘤的疗效及安全性。方法 2008年7月至2013年9月于沈阳军区总医院行主动脉腔内隔绝术治疗主动脉假性动脉瘤的患者16例。男13例,女3例,年龄(61.2±11.5)岁。经股动脉切开植入覆膜支架封堵主动脉假性动脉瘤破口,主动脉造影确认疗效;合并严重冠状动脉狭窄的患者,于腔内隔绝术后3~7 d完成经皮冠状动脉介入治疗(PCI)。观察主动脉疾病患者介入治疗的疗效。结果主动脉腔内隔绝术操作成功率为100%,共植入14枚主体覆膜支架及4枚短体覆膜支架,16例患者均无残余内漏。1例患者部分封闭左锁骨下动脉开口,左上肢血供无缺血症状。2例冠状动脉严重病变患者,1例行冠状动脉旁路移植术,1例行植入支架治疗。围术期无出血、心肌梗死、死亡等并发症。术后平均随访(68±29)个月,随访率为87.5%(14/16)。1例患者术后3个月发生脑出血,1例患者术后6个月发现胃癌化疗治疗病情稳定,于术后12个月发生截瘫,目前已失去行动能力。冠心病患者无心绞痛发作,无复查冠状动脉造影患者,无MACE发生。随访期间共死亡患者4例(25%),明确主动脉血管破裂死亡2例(12.5%)。结论主动脉腔内隔绝术治疗主动脉假性动脉瘤,创伤小、近期及长期疗效好,并发症低。合并冠心病患者择期二次行PCI安全可行。     

覆膜支架腔内治疗急性胸主动脉综合征

目的 评价覆膜支架腔内治疗急性胸主动脉综合征的有效性和安全性。方法 2001年5月至2005年12月应用覆膜支架治疗57例急性胸主动脉综合征患者,其中急性主动脉B型夹层45例,穿透性粥样硬化性溃疡（PAU）或假性动脉瘤9例,创伤性胸主动脉瘤3例。建立数据库,分析其临床特点、疗效及随访结果。结果 57例患者覆膜支架置入技术成功率100％。5例有近端内漏,1例术中出现升主动脉夹层,未予特殊处理,随访结果良好;1例术后7天出现升主动脉夹层并发心包填塞死亡。5例PAU或主动脉夹层合并冠心病患者,在应用覆膜支架成功完全封闭破口后立即行冠状动脉介入治疗成功。1例出现术后一过性双下肢无力,经静脉滴注山莨菪碱和甘露醇2天后痊愈。1例支架覆盖左锁骨下动脉开口导致左椎动脉缺血,嗜睡2天后自行好转。术后重症监护病房时间1～8（平均3．5）天,术后平均住院10天。术后30天内死亡2例,1例死于升主动脉夹层破裂,1例死于急性肾衰竭。术后30天内死亡率3．5％。术后平均随访（25．3±13．1）（13—55）个月。1例于术后3个月死于大咯血,1例死因不明。1例因近端内漏行二次腔内修复术。5例患者因降主动脉覆膜支架远端再发现破口,行二次腔内修复术。术后截瘫发生率为0,无支架移位、狭窄等并发症。术后住院及随访期内总死亡率为7．0％。与传统手术相比,腔内治疗急性胸主动脉综合征具有创伤小、严重并发症少、住院时间较短的优势。结论 覆膜支架是治疗急性胸主动脉综合征优良且有效的方法,也可用于外科手术高风险患者,近中期随访结果良好,远期结果有待于进一步随访。     

国产Willis覆膜支架治疗复杂性颅内动脉瘤的疗效及长期随访

目的评价国产Willis覆膜支架系统治疗复杂性颅内动脉瘤患者的安全性及远期疗效。方法 2006年10月—2007年9月,复旦大学附属华山医院神经外科单纯采用国产颅内Willis覆膜支架系统,治疗7例复杂性颅内动脉瘤,其中颈内动脉海绵窦段3例,颈内动脉眼动脉段1例,4例均为大型宽颈动脉瘤;颈内动脉后交通段复发动脉瘤1例,椎动脉小脑后下动脉下段大型夹层动脉瘤2例。结果①对7例（7个动脉瘤）患者共置入9枚覆膜支架,其中8枚成功置入载瘤动脉。对5例置入支架后即刻造影,显示病变完全消失,载瘤动脉通畅;1例巨大海绵窦段动脉瘤置入1枚支架后,有少量对比剂漏入动脉瘤（内漏）;1例椎动脉瘤置入第1枚支架内漏明显,再置入1枚支架后好转。②术中发生颅内远端血管破裂出血1例,经开颅手术清除血肿后痊愈。其他病例无手术相关并发症。③术后5～12个月对7例患者均行临床及DSA随访,均无神经功能障碍。2例有内漏者,1例消失,1例仍有少量内漏,但较术后即刻明显好转;7例均无动脉瘤复发,载瘤动脉通畅。5例术后45～55个月再获得DSA随访,动脉瘤无复发,载瘤动脉通畅、无狭窄。1例失访,1例非动脉瘤性死亡。结论对部分复杂性颅内动脉瘤,采用Willis覆膜支架治疗安全有效,长期疗效较好。     

GORE EXCLUDER覆膜支架在腹主动脉瘤腔内修复术中的临床应用

目的：探讨GORE EXCLUDER覆膜支架腔内修复治疗腹主动脉瘤的疗效.方法：回顾性分析自2012年1月至2013年5月,采用GORE EXCLUDER覆膜支架行腹主动脉瘤腔内修复术的21例患者,其中男性19例,女性2例,年龄48 ～ 84岁,平均（68.9±9.9）岁.21例均为肾下型腹主动脉瘤,术后1,3,6及12个月之后,每年行主动脉CTA复查.结果：21例均成功置入GORE EXCLUDER覆膜支架,技术成功率100％;共置入主体覆膜支架21枚,对侧分支支架21枚,延长支6枚,Cuff支架1枚.主体覆膜支架直径23 ～31 mm,长度为120 ～ 180 mm,平均（152-±20.7）mm; 16例封闭一侧髂内动脉,5例保留双侧髂内动脉.围手术期无死亡病例及严重并发症发生;随访3 ～15个月,未见支架移位及内漏发生.结论：GORE EXCLUDER腔内修复肾下腹主动脉瘤近、中期效果良好.     

Midterm results of aortic diameter outcomes after thoracic stent-graft implantation for aortic dissection: a multicenter study.

PURPOSE: To evaluate aortic diameter outcomes after stent-graft implantation for aortic dissection in the descending thoracic aorta. METHODS: Fifty patients with type A dissection after ascending aortic surgery (n = 10), type B dissection (n = 34), or intramural hematoma (n = 6) underwent stent-graft repair in 3 centers. Thrombosis and aortic diameter were analyzed by computed tomographic angiography at different levels of the aorta before stent-graft implantation, at discharge, and at follow-up. Measurements were standardized. RESULTS: In all, 67 stent-grafts were implanted for acute (n = 18) and chronic (n = 32) dissection. Stent-graft placement was successfully performed with high technical success (100%) despite 4 major complications (iliac thrombosis in 2 cases, aortic rupture, and a type A dissection) in 3 (6%) patients. Complete thrombosis of the thoracic false lumen was observed in 42% and 63% of cases at discharge and at follow-up (mean 15 months), respectively. At follow-up, the diameters of the entire aorta (mean 5 mm, p < 0.05) and the false lumen (mean 11 mm, p < 0.0001) decreased. Diameters of the abdominal aorta remained stable in association with persistent false lumen perfusion at this level. Aortic diameter results were better in the subgroup of patients with intramural hematoma compared to patients with Marfan syndrome. Three early deaths unrelated to the stent-graft procedure occurred; 2 patients with partial thrombosis of the false lumen died in follow-up secondary to aortic diameter growth. CONCLUSION: Complete thrombosis of the false lumen by stent-graft coverage of the entry tear results in decreased diameter of the entire aorta. In patients with partial thrombosis of the false lumen, the aneurysm continues to enlarge.     

Provisional extension to induce complete attachment after stent-graft placement in type B aortic dissection: the PETTICOAT concept.

PURPOSE: To report the use of a technique (PETTICOAT: provisional extension to induce complete attachment) to obliterate sustained abdominal false lumen flow and pressurization despite successful stent-graft sealing of the thoracic entry tear in patients with complicated type B aortic dissection. METHODS: Of 100 initial patients subjected to stent-graft repair for complex type B aortic dissection with thoracoabdominal extension, 12 patients (10 men; mean age 58.7 years, range 44-76) demonstrated distal true lumen collapse and a perfused abdominal false lumen despite successful sealing of the proximal tears. As an adjunctive or staged procedure, a scaffolding stent was placed for distal extension of the previously implanted stent-graft. In each case, a Sinus aortic stent, Fortress stent, or a Z-stent system was customized with maximum 2-mm oversizing versus the original stent-graft diameter. Magnetic resonance or computed tomographic angiography was performed at discharge, at 3 months, and then annually to determine false channel thrombosis, true and false lumen dimensions, and re-entry flow. RESULTS: Delivery was successful in all cases (100%). The compressed distal true lumen (mean 4+/-3 mm) was reconstructed to a mean width of 21+/-3 mm, and malperfusion was abolished without any obstruction of the abdominal side branches. At up to 1-year follow-up, there were no signs of expansion or distal progression of the scaffolded dissected aorta. All patients with complete thoracic thrombosis showed evidence of improved aortic remodeling; 1 patient with no false lumen thrombosis died at 11 months from thoracoabdominal aortic rupture. CONCLUSION: The PETTICOAT technique may offer a safe and promising adjunctive endovascular maneuver for patients with distal malapposition of the dissecting membrane and false lumen flow. The technique can both abolish distal true lumen collapse and enhance the remodeling process of the entire dissected aorta.     

Endovascular stent-graft repair of aortic dissection

Between October 2000 and January 2002, 9 consecutive male patients with subacute or chronic aortic dissection underwent stent-graft placement. The indication for surgery was continuous pain or aneurysm development. One patient had a type A dissecting aortic aneurysm with a primary tear in the ascending thoracic aorta; the other 8 had type B dissection. Placement of an endovascular stent-graft was technically successful in 8 patients, and one underwent an open procedure for abdominal aortic fenestration. The entry site was sealed and the false lumen disappeared in 8 cases, and thrombosis of the false lumen was obtained. Rupture of an iliac artery dissecting aneurysm occurred in one patient 2 days after stent-graft placement; abdominal aortic fenestration with prosthetic replacement of the distal abdominal aorta was performed. One patient died of myocardial infarction 3 days after the stent-graft procedure. During a mean follow-up period of 7 months (1-16 months), one patient died of acute myocardial infarction at 11 months. It was concluded on the basis of these short-term results that endovascular repair of aortic dissection is a promising treatment, and abdominal aortic fenestration is a useful adjuvant procedure.     

主动脉壁间血肿的治疗与转归

目的：总结主动脉壁间血肿（aortic intramural hematoma,AIMH）的治疗选择与转归,以指导临床.方法：回顾分析北京大学人民医院2009年1月至2013年4月间,共收治AIMH患者16例,A型5例,B型11例.分析患者特征及治疗选择,电话及门诊随访确定治疗效果.结果：16例患者10例药物治疗,4例介入治疗,2例外科手术治疗,1例药物治疗患者死亡,其余患者均痊愈出院,随访1 ～48个月,平均13.3个月,1例失访,其余患者均存活.结论：AIMH是一组需要临床重视并积极治疗的主动脉病变,应根据其病理特征选择合适治疗方案.     

Endovascular repair of acute type B aortic dissection: midterm results.

PURPOSE: To evaluate midterm results of endovascular stent-graft placement for acute Stanford type B dissection (TBD). METHODS: A retrospective review was conducted of 35 consecutive patients who were treated with stent-graft implantation for acute TBD between July 1996 and July 2007. Computed tomographic (CT) volumetric analysis of the true lumen (TL) and false lumen (FL) changes in 23 patients was performed, as well as evaluation of the influence of re-entry points and length of stent-graft coverage on volume changes. In addition, complications were evaluated. Follow-up was performed at 6 and 12 months and yearly thereafter. RESULTS: The technical success rate was 82.7%, and the 30-day mortality rate was 8.5%. Mean follow-up was 34 months. The overall survival rate at 5 years was 78.4%. Complications included retrograde type A dissections in 3 patients during the perioperative period and in 1 patient during midterm follow-up. In addition, 5 early and 3 late endoleaks were observed. Three patients were converted to open surgery and 2 needed secondary interventions. In the stented segment, stabilization of the aorta was achieved even during midterm follow-up, with a TL volume increase of 59% at 5 years and nearly stable FL volume. The segment from the distal end of the stent-graft to the celiac artery, however, showed unstable TL and FL volumes, with high standard deviations after the first postinterventional year and circumferential aneurysmal dilatation of the aorta immediately adjacent to the stent-graft in 5 patients. The abdominal aorta showed no substantial volume changes over time. The length of stent-graft coverage and the occurrence of re-entries greatly influenced FL volume changes distal to the stent-graft. CONCLUSION: Serious complications can occur during and after endovascular repair of TBD. Therefore, it should be reserved for high-risk patients.     

心力衰竭伴不同宽度左束支传导阻滞患者对心脏再同步治疗反应的分析

目的:分析左束支传导阻滞(left bundle branch block,LBBB)心力衰竭患者对心脏再同步(cardiac resynchronization therapy,CRT)治疗的疗效反应情况。方法:回顾性分析在本院成功置入CRT伴LBBB的心力衰竭患者。所有患者术前、术后1w及最终随访均行12导联心电图、超声心动图、组织多普勒。均对比术前、术后左心室大小、射血分数及生存现状。结果:共44例完成随访,随访6~61个月,平均27.12个月,4例再同步治疗后仍反复出现心力衰竭并因此再入院治疗,无死亡病例。共有38例(86.4%)患者对CRT治疗有反应,真性LBBB亚组中29例(93.5%)有反应,非真性LBBB亚组中9例(69.2%)有反应。真性LBBB亚组左心室射血分数绝对值、左心室舒张末径(left ventricular end diastolic diameter,LVEDD)及左心室射血分数的改善程度更显著,差异有统计学意义。结论:CRT治疗可使大部分患者获益,真性LBBB患者心功能改善更为显著,但终末期心脏失代偿严重者难于从中获益。     

经导管堵闭婴幼儿大型动脉导管未闭(≥4mm)的短中期随访

目的分析经导管封堵婴幼儿大型动脉导管未闭(patent ductus arteriosus,PDA)(≥4 mm)的短中期随访的结果。方法回顾性分析50例大型PDA患儿的疗效、并发症及中期随访结果 ,评价其中期疗效、安全性及影响疗效的因素。结果成功植入封堵器49例(98%),其中46例(92%)应用PDA封堵器,2例(4%)应用肌部室间隔缺损封堵器,1例(2%)应用膜周对称型室间隔缺损封堵器。术后3 d内复查超声心动图:4例(8%)有残余分流;封堵后左心室舒张末内径、左心房内径、左心室射血分数、肺动脉收缩压、三尖瓣反流(tricuspidregurgitation,TR)及二尖瓣反流(mitral regurgitation,MR)均较术前缩小或减轻,差异有统计学意义(P<0.05);封堵后主动脉收缩压及主动脉瓣反流(aortic regurgitation,AR)较术前增加或加重,差异有统计学意义(P<0.01)。出院后完成随访41例,随访率81.6%,中位随访时间6个月(1～57个月)。残余分流率在术后3 d、1个月、6个月分别为8%、4%、2%。术后1～6个月内左心室射血分数较术后3 d有明显改善,差异有统计学意义(P=0.000);术后1年内随访:MR较术前降低,差异有统计学意义(P=0.000);AR及TR与术前比较,差异无统计学意义(P>0.05);术后1年内11例患儿出现左肺动脉血流速度加快(1.5～2.1 m/s),其中9例随访1年以上左肺动脉血流1.5～2.0 m/s,4例随访3～5年左肺动脉血流速度1.5～2 m/s,但左肺动脉至主肺动脉压差均≤20 mm Hg(1 mm Hg=0.133 kPa)。无降主动脉狭窄。结论经导管用封堵器治疗婴幼儿大型PDA(≥4 mm)的短中期疗效确切,安全性好,但对左肺血流速度增快患者需长期随访。     

Recurrent aortic aneurysms following thoracic aortic stent-graft repair in a patient with Cogan syndrome.

PURPOSE: To report the need for multiple surgical interventions to treat recurrent aortic aneurysms in a patient with Cogan syndrome. CASE REPORT: A 17-year-old Chinese man with clinical Marfanoid features had a left common carotid artery pseudoaneurysm electively repaired with an autologous saphenous vein graft. Four months later, he presented with acute chest pain. Computed tomography (CT) revealed a 1-cm pseudoaneurysm at the mid descending aorta; a 24 x 100-mm Talent stent-graft was implanted to exclude the pseudoaneurysm. He was also found to have increasing left-sided hearing loss. A month later, the patient was re-admitted with vertigo and keratitis, which were treated appropriately. Nine months following stent-graft insertion, he was admitted with acute hemoptysis. Urgent CT showed a rupture at the proximal end of the stent-graft, with hemorrhage into the lung parenchyma. In an emergent procedure, the stent-graft was removed, and the descending thoracic aorta was repaired. Intraoperatively, a large pseudoaneurysm was found arising from the proximal part of the stented aorta, which appeared thickened. His postoperative recovery was uneventful. Nine months after the thoracotomy, a routine CT revealed an aneurysm at the distal descending thoracic aorta. On re-thoracotomy, a de novo saccular aneurysm was found 2.5 cm from the distal anastomosis. The affected segment was replaced with a Dacron graft. The distal aorta appeared thickened and edematous; histology confirmed aortitis. The patient was subsequently diagnosed with Cogan syndrome and given corticosteroids and methotrexate. There is no evidence of recurrence at nearly 2 years after the last intervention. CONCLUSION: This case highlights the pitfalls of stent-graft repair in a patient with presumed connective tissue disease.