升主动脉-降主动脉心包内旁路术治疗成人主动脉缩窄及主动脉弓中断合并心脏畸形的临床分析

目的探讨经正中切口行升主动脉-降主动脉心包内旁路术治疗成人主动脉缩窄及主动脉弓中断合并心脏畸形的技术要点。方法 2010年4月至2015年1月2例成人主动脉缩窄和1例成人主动脉弓中断合并心脏畸形患者行手术治疗,其中男2例,女1例;年龄35.6(27~46)岁。患者的疾病包括先天性主动脉弓缩窄、二尖瓣前叶脱垂伴中度关闭不全1例,先天性主动脉瓣二瓣化畸形伴主动脉瓣重度关闭不全、升主动脉瘤及主动脉弓缩窄1例,先天性主动脉瓣二瓣化畸形伴主动脉瓣轻度狭窄、房间隔缺损(继发孔)及主动脉弓中断(A型)1例。患者均在升主动脉及股动脉,上下腔静脉插管建立体外循环,经正中切口行升主动脉-降主动脉心包内旁路术及合并心脏畸形矫治手术。结果本组无围术期死亡病例,术后患者症状明显好转,出院随访2~59个月,除1例患者仍有上肢高血压需服用药物控制外,其他患者血压恢复到正常水平,术后下肢乏力症状消失。主动脉CTA检查示人工血管通畅,无人工血管扭曲压缩及假性动脉瘤形成等并发症。结论经正中切口行升主动脉-降主动脉心包内旁路术治疗成人主动脉缩窄及主动脉弓中断,同时行合并心脏畸形矫治的一期手术,患者手术安全及疗效确切,临床可选择性应用。     

外科手术治疗主动脉缩窄合并复杂心脏畸形79例单中心回顾性分析

目的 总结主动脉缩窄合并复杂心脏畸形的手术治疗效果。方法 回顾性分析2010年1月19日—2017年9月7日于广州市妇女儿童医疗中心住院治疗的主动脉缩窄合并复杂心脏畸形79例患儿的临床资料,其中男52例、女27例,中位年龄71 d,中位体重4.3 kg。术前合并气管狭窄26例,术前气管插管7例。采取胸骨正中切口入路,以扩大端侧吻合矫治主动脉缩窄,同期处理合并心脏畸形。所有手术由同一组外科医师完成。结果 中位深低温停循环时间18(13～28) min,中位主动脉阻断时间62(15～199) min,中位体外循环时间145(71～674) min;中位术后机械通气时间72(9～960) h,中位ICU滞留时间144(12～1 944) h,中位总住院时间24(2～93) d;早期死亡9例,晚期死亡5例;再手术28例,再缩窄10例。主动脉缩窄段压差在术后明显下降;经皮血氧饱和度、C-反应蛋白（CRP）、乳酸和肌酐水平在术后明显上升。与非死亡组相比,死亡组的术前和术后即刻血氧饱和度均较低,而术后即刻脑钠肽（BNP）和CRP均较高。结论 胸骨正中单一切口下,以扩大端侧吻合法矫治主动脉缩窄,同期处...     

正中切口解剖外旁路移植术一期治疗主动脉缩窄合并心脏畸形

目的总结正中切口解剖外旁路移植术一期治疗主动脉缩窄合并心脏畸形的外科治疗经验,以提高手术疗效。方法1997年7月至2008年7月,采用正中切口解剖外旁路移植术一期治疗主动脉缩窄合并心脏畸形31例,其中男20例,女11例;年龄31．9±11．7岁。合并的心脏畸形包括：主动脉瓣狭窄或关闭不全22例,二尖瓣狭窄或关闭不全9例,动脉导管未闭5例,升主动脉瘤4例,室间隔缺损3例,冠心病2例。解剖外旁路移植术包括升主动脉-腹主动脉旁路移植术22例,升主动脉-心包后降主动脉旁路移植术9例。同期手术包括主动脉瓣置换术16例,主动脉根部置换术6例,二尖瓣成形或置换术9例,升主动脉置换或成形术4例,动脉导管未闭缝合术5例,室间隔缺损修补术3例,冠状动脉旁路移植术2例。结果住院死亡1例（3．2％）,术后39d死于感染性中毒性休克。术后上、下肢收缩压压差较术前明显下降（13．7±10．2mmHg vs．64．2±25．3mmHg,P〈0．05）。随访27例,随访时间4～73个月,无晚期死亡、与人工血管相关的并发症和再次手术患者。结论正中切口解剖外旁路移植术是一期治疗成人及青少年主动脉缩窄合并心脏畸形的一种安全有效的手术方法。升主动脉腹主动脉旁路移植术及升主动脉-心包后降主动脉旁路移植术均可获得满意疗效。     

非体外循环下成人复杂型主动脉缩窄的解剖矫治

目的为提高成人复杂型主动脉缩窄的矫治效果,探讨其最佳外科治疗策略。方法回顾性分析北京阜外心血管病医院2005年1月至2008年12月在非体外循环下一期解剖矫治7例成人复杂型主动脉缩窄患者的临床资料,其中男5例,女2例;年龄16~41岁,平均年龄24.4岁。合并主动脉弓发育不良2例,主动脉弓部动脉瘤1例,胸降主动脉瘤3例（其中合并B型主动脉夹层1例）,主动脉缩窄手术后再狭窄1例。全组患者术前均通过彩色超声心动图、CT或磁共振成像确诊。均在全身麻醉常温非体外循环下手术,手术入路为胸骨正中切口1例,左后外侧切口6例;解剖矫治为：主动脉弓补片加宽4例,降主动脉人工血管置换3例（其中1例同期行腹主动脉置换,1例B型主动脉夹层同期植入支架象鼻）。结果全组无手术死亡,无严重手术并发症。随访7例,随访时间6~49个月,平均随访20.1个月,无远期死亡,无主动脉缩窄复发二次手术患者。有1例术后出现声音嘶哑,随访11个月时声音嘶哑未改善。结论非体外循环下对成人复杂型主动脉缩窄行一期解剖矫治是安全可行的,近、远期效果良好。     

主动脉弓补片成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良

目的总结缩窄段切除加主动脉弓补片成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的临床经验。方法 2007年5月至2009年12月,上海交通大学医学院附属上海儿童医学中心对49例婴幼儿主动脉缩窄合并主动脉弓发育不良患者采用主动脉缩窄段切除加主动脉弓补片成形术治疗,其中男30例,女19例;年龄23 d至3岁1个月,6个月34例,6个月~1岁10例,1岁5例。31例患者在深低温体外循环下完成手术,采用选择性脑灌注技术;15例在深低温停循环下手术;3例在中低温体外循环下完成手术。31例用自体心包行主动脉弓成行补片,14例采用自体肺动脉壁组织,4例采用异种心包。合并的心内畸形同期纠治。结果围术期死亡1例,死于循环衰竭,手术死亡率2.04%(1/49)。5例术后发生低心排血量综合征,1例合并肾功能衰竭患者,均经相应处理治愈。术后复查超声心动图提示无残余梗阻。随访48例,随访时间4个月至3年。随访期间有1例患者主动脉弓压力阶差40 mm Hg,计算机断层扫描显示主动脉弓再狭窄,于术后8个月再次手术;2例主动脉弓压力阶差20 mm Hg,仍在继续随访;其余患者主动脉弓形态良好,与术后当时比较,随访期间主动脉弓降部血流速度无明显变化,计算机断层扫描显示:主动脉弓几何构型正常。术前存在左主支气管受压的患者在主动脉弓成形术后半年,左主支气管受压表现有明显改善或完全消失,无主动脉夹层动脉瘤发生。结论缩窄段切除术加主动脉弓补片成形术是治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的理想手术方法。     

成人主动脉缩窄的临床特点和外科矫正

目的探讨成人主动脉缩窄的临床特点和外科治疗方法。方法40例成人主动脉缩窄患者行手术矫治，其中心脏不停跳手术28例，常规体外循环下手术12例。行主动脉狭窄段切开、人工血管补片扩大术12例，人工血管置换术15例，锁骨下动脉降主动脉旁路移植术1例，采用人工血管行胸一腹主动脉旁路移植术9例，经心包后径路行升主动脉一降主动脉旁路移植术3例。结果全组无手术死亡，几种术式术后均无脑部和脊髓等神经系统并发症。术后36例得到随访，平均随访12个月。36例患者上、下肢血压差均小于10mmHg（1kPa－7．5mmHg）。超声心动图和超高速CT检查提示人工血管血流通畅，无假性动脉瘤形成。结论成人主动脉缩窄的外科术式有多种选择，掌握好手术适应证，根据患者的具体病理生理状况选择合适的手术方式可获得满意的治疗效果。     

经胸骨正中切口一期矫治主动脉缩窄或主动脉弓中断合并心内畸形

目的 总结经胸骨正中切口一期矫治主动脉缩窄或弓中断合并心内畸形的经验.方法 2007年1月-2008年7月手术治疗24例.包括主动脉缩窄9例,主动脉缩窄合并主动脉弓发育不良12例,主动脉弓中断3例.4例合并右室双出口(Traussig-Bing型).22例合并非限制性室间隔缺损,2例不合并室间隔缺损病儿1例合并主动脉瓣下狭窄,另l例合并肺静脉狭窄.主动脉弓降部成形均在深低温低流量持续性选择性脑灌注下进行.3例主动脉弓中断及9例主动脉缩窄病儿采用端端吻合术.12例主动脉缩窄合并主动脉弓发育不良病儿中采用扩大端端吻合术8例,端侧吻合术2例,补片成形术2例.结果 死亡2例.全组病儿围术期未出现神经系统并发症及肾功能损害.术后反复呼吸道感染2例.除l例残存压差大于20mm Hg外,最长随访18个月,尚未发现再缩窄发生.结论 主动脉缩窄或弓中断合并心内畸形一经诊断即需尽早手术.经胸骨正中切口一期矫治是安全、有效的.充分切除动脉导管组织,广泛彻底游离松解胸部各血管进行无张力吻合以及选择恰当的组织一组织吻合术式是主动脉弓降部成形手术成功及减少再缩窄发生的关键.     

婴幼儿主动脉缩窄合并心内畸形的外科治疗

目的 探讨婴幼儿主动脉缩窄合并心内畸形的外科治疗经验.方法 2000年1月至2006年12月,84例主动脉缩窄合并心内畸形患儿接受了外科手术治疗,手术年龄1个月～3岁(平均13.5个月),体重3.3～15.0 kg(平均7.3 kg).12例合并复杂心内畸形,72例合并室间隔缺损和其他简单心内畸形,23例伴有主动脉弓发育不良.一期手术62例,49例正中开胸同时矫治主动脉缩窄和心内畸形,13例左侧开胸矫治主动脉缩窄,正中开胸修补心内畸形;分期手术22例.主动脉缩窄的手术方式包括补片成形42例,切除端端吻合30例,锁骨下动脉翻转6例,血管旁路3例,球囊扩张1例.在49例正中切口一期手术中,43例应用选择性脑灌注加下半身停循环,4例应用全身低流量灌注,2例应用深低温停循环.结果 围手术期死亡8例,死亡率9.5%,其中3例为术前漏诊主动脉缩窄.结论 婴幼儿主动脉缩窄合并心内畸形的外科治疗可获得良好的近期疗效,绝大部分患儿可采取正中切口一期手术.选择性脑灌注和下半身停循环可以有效地保护脑和重要脏器.     

经胸骨正中切口一期矫治小儿主动脉缩窄及合并畸形

目的探讨经胸骨正中切口一期矫治小儿主动脉缩窄(CoA)及合并畸形的手术方法和治疗效果。方法2002年6月至2005年8月手术治疗CoA及合并畸形36例,其中男27例,女9例;年龄18d~8岁,平均(14·3±9·5)个月,其中小于1岁27例,小于3月龄18例;体重3~23kg,平均(6·6±3·0)kg。有症状的婴儿型27例,无症状的大儿童型8例,经左胸切口矫治CoA后再狭窄1例;合并室间隔缺损、动脉导管未闭、房间隔缺损、主动脉弓发育不良、完全性大动脉转位及主动脉瓣下狭窄等心内畸形。36例均经胸骨正中切口以扩展端端吻合术一期矫治CoA及合并畸形。结果手术死亡1例,系术前反复心力衰竭及充血性肺炎,术后15d死于心肺功能衰竭。35例随访4~36个月,晚期死亡1例。生存34例恢复良好,无再缩窄发生。结论经胸骨正中切口一期矫治CoA及合并畸形效果良好;扩展端端吻合法是一种值得推荐的技术。     

Taussig-Bing畸形合并大动脉侧侧位的外科治疗

目的总结外科手术一期治疗Taussig—Bing畸形合并大动脉侧侧位的临床经验。方法2000年5月～2007年9月手术治疗26例（手术年龄3.1±2．2个月）Taussig—Bing畸形合并大动脉侧侧位患者，其中合并主动脉弓病变13例。25例患者采用动脉转位术（arterial switch operation，ASO），1例采用Kawashima手术治疗。合并主动脉弓病变的矫治方法主要是切除缩窄段后，将胸主动脉与主动脉弓下缘行端端吻合，或与升主动脉行端侧吻合。结果住院死亡3例（11.5％，3／26）。2005年6月以后连续手术治疗的15例Taussig—Bing畸形伴大动脉侧侧位，无住院死亡。随访21例，随访时间1～5年；患者生长发育均明显改善，心脏较术前缩小，肺血明显减少。2例患者因肺动脉瓣上狭窄而再次行手术修补。结论Taussig—Bing畸形合并大动脉侧侧位的病理解剖比较复杂，必须根据不同的解剖条件选择不同的手术方法，才能降低手术死亡率。     

Correction of coarctation of the aorta in neonates and young infants. An individualized surgical approach

Because of the controversy concerning the ideal surgical repair for symptomatic coarctation of the aorta presenting in neonates and infants, our entire series of 179 children under the age of 12 months undergoing repair between January 1, 1976 and December 31, 1984 was reviewed. Of this group, 109 were neonates, 43 infants aged 31-90 days and 27 infants aged 90 days-12 months. Twenty patients had a simple coarctation and 159 had complex coarctation with additional intracardiac anomalies such as ventricular septal defect (37 patients also had pulmonary artery banding). One hundred and twenty-four were repaired with a subclavian flap operation, 32 with resection and end-to-end anastomosis and 23 with complex repairs (e.g. patch and reversed flap). Type of repair was the surgeon's choice and was selected on the basis of the anatomy of the coarctation. Total perioperative mortality was 15% (N = 27) while late mortality was 12% (N = 21). Twenty-one risk factors for mortality were evaluated by logistic analysis and the method of Cox. There was no risk difference between end-to-end versus subclavian flap repairs and all but one death occurred in patients with complex coarctations. Risk for in hospital death was increased by only one variable: the need for repair in the neonatal period. Risk for death in the first year of life was increased by the presence of congestive heart failure at initial presentation while later death correlated with intracardiac surgery. Recoarctation occurred in 28 patients (18.4%), all but 4 of these occurred in patients undergoing neonatal repairs.(ABSTRACT TRUNCATED AT 250 WORDS)     

787例小于6月龄先天性心脏病患者外科治疗的临床分析

目的回顾性总结婴幼儿先天性心脏病的手术时机和手术治疗方法,以进一步提高手术成功率和远期疗效。方法1988年1月～2003年6月,手术纠治年龄小于6个月的先天性心脏病患者787例。主要病种包括完全性大动脉错位109例,完全性肺静脉异位引流51例,肺动脉闭锁16例,主动脉缩窄33例,室间隔缺损伴肺动脉高压299例,法洛四联症44例,右心室双出口23例,室间隔完整型肺动脉闭锁9例等;对787例患者根据不同病种采取相应的手术方法纠治。结果手术死亡77例,手术死亡率9.78%(77/787)。随着手术方法的不断改进,手术总死亡率从1988～1995年的25%降至2003年的4.11%。随访完全性大动脉错位患者中发生VSD残余漏1例,术后3个月再次手术治愈;随访中发生肺动脉和主动脉瓣上狭窄2例。完全性肺静脉异位引流心内型患者中2例分别在术后4d和2个月出现肺静脉回流梗阻,1例死亡,1例再次手术解除梗阻。室间隔缺损患者中发生残余漏5例,分流量小,不需再次手术。其余病例随访资料不完整。结论对婴幼儿先天性心脏病施行手术治疗的时间非常重要,危重复杂型先天性心脏病患者如不早期手术,将失去手术机会,增加术后危险性和死亡率。     

Aneurysms after patch graft aortoplasty for coarctation of the aorta: long-term results of surgical management

Twenty-two (32.8%) of 67 patients who had patch graft aortoplasty for aortic coarctation underwent reoperation because of aneurysm formation opposite or at the site of the patch. The mean age at primary repair was 25.3 years (standard deviation, 9.5 years). Reoperation took place 12.3 years (standard deviation, 3.7 years) later. In 19 patients, the aneurysm was resected and the aorta reconstructed with a tubular prosthesis. In 3 patients, the aneurysm was given external support. There were no hospital deaths. Recurrent nerve paralysis (36%) and bleeding necessitating repeat thoracotomy (32%) were the most common complications. Mean follow-up was 4.6 years. No aneurysms developed at the site of reoperation. Two patients died of causes not related to reoperation. Reconstruction with a tubular prosthesis gives good long-term results in patients with aneurysm formation after patch grafting for coarctation. External support offers technical advantages in select patients. Close follow-up of patients who have coarctation repair with a patch graft is essential.     

Subclavian flap aortoplasty for treatment of coarctation in early infancy

In a 2-yr period 12 infants less than 2 mo of age underwent surgery for critical coarctation of the aorta. All had severe congestive failure with unsatisfactory response to medical therapy. In all patients the coarctation was located in the preductal or preligamentous region. Eleven patients had an associated ventricular septal defect (VSD). Early (1-mo) survival in the patients with VSD was 82%. Two early deaths occurred; there have been five late deaths on follow-up of up to 30 mo. In all patients who expired, the cause of death was unrelated to the coarctation repair. Recurrent coarctation has occurred in one patient. Early aggressive surgical intervention is indicated in the management of coarctation of the aorta both with and without VSD.     

Surgery for aortic coarctation: a 30 years experience

Objective: A retrospective study to review the experience of a single center with surgery for aortic coarctation over a period of 30 years (1970–1999). Methods: Criteria for inclusion: (a) aortic coarctation, isolated or associated with congenital heart defect; (b) surgery between 1970 and 1999. Data recorded: (1) date of surgery; (2) age at surgery; (3) associated lesions; (4) surgical technique; (5) simultaneous surgical procedures; (6) early and late surgical results in term of: (a) deaths; (b) need for reoperation because of re-coarctation or other cardiac lesion; (c) residual/recurrent pressure gradient, evaluated at cuff/Doppler at rest; (d) systemic hypertension, requiring medical treatment. Results: One hundred and forty-one patients underwent surgery for aortic coarctation: 30 neonates, 29 infants, 45 children and 37 adults. Associated lesions were found in 8/37 (=21.6%) adults and in 73/104 (=70.1%) pediatric patients. There were no hospital deaths. During the follow-up there were one late death in the adults group (1/37=2.7%) and three late deaths in the pediatric group (3/104=2.9%), all unrelated with aortic coarctation. Re-operation because of re-coarctation occurred only in ten late survivors of the pediatric group (10/101=9.9%), 9/10 operated on before 1980 (P<0.00001). End-to-end anastomosis, enlarged to the aortic arch in neonates, was associated with the lowest incidence of re-coarctation (P<0.005). A significant (>20 mmHg at rest) pressure gradient was found in none of the adults, and in seven of the 91 pediatric patients (7/91=7.7%) late survivors. Three adults (3/36=8.3%) late survivors are on medical treatment to control systemic hypertension. Conclusions: The long-term results of our retrospective study confirm that surgery has to be considered the gold standard for the treatment of aortic coarctation. The interventional angioplasty techniques have to provide long-term outcome at least similar to the results obtained with surgery.     

Repair of aortic coarctation in the first three months of life: immediate and long-term results

The optimum surgical procedure for treatment of coarctation of the aorta in the neonatal period remains controversial. To assess immediate and long-term results of using primarily the subclavian flap angioplasty procedure (SFA), we reviewed our initial 5-year experience. The average follow-up was 6 years. From 1977 to 1981, 25 infants under 3 months of age (1 to 86 days, mean 21) required emergency surgery for repair of coarctation of the aorta. Three groups of patients were identified. Group I consisted of 10 patients with or without patent ductus arteriosus. In group II, 10 patients had coarctation association with one or multiple ventricular septal defects (VSDs) without other congenital defects. In group III, 5 patients had coarctation associated with more complex congenital heart lesions. Twenty-three SFAs and two patch aortoplasties were performed. No patient with isolated VSD was banded. All patients except one in group III with an associated atrioventricular canal survived initial hospitalizations. Four late deaths occurred, all in patients with associated complex heart defects. There were three recurrent coarctations requiring surgery or balloon angioplasty (12%)--one in each group, with a total rate of 0.77 recurrences per 100 patient-months. SFA for coarctation in the neonatal period is a safe and effective operation with a low initial mortality (4%, 0-19%, 70% confidence limits) well tolerated in this group of ill patients. Long-term outcome is primarily related to the presence of associated complex congenital defects. Infants with VSD associated with coarctation did not require pulmonary artery banding unless primary intracardiac repair was not feasible.(ABSTRACT TRUNCATED AT 250 WORDS)     

Modified end-to-end anastomosis combined with subclavian flap aortoplasty for repair of coarctation of the aorta with extended hypoplasia of the aortic isthmus

Recoarctation is a serious complication of the surgical repair of coarctation of the aorta. A combined technique using end-to-end anastomosis and subclavian flap angioplasty has shown good results. However, this technique is not entirely free from longitudinal traction in cases of extended hypoplasia of the aortic isthmus. To obviate these problematic sequelae, we have modified the combined technique for repair of coarctation of the aorta having extended hypoplasia of the aortic isthmus. Almost the entire aortic isthmus is left unresected and a lesser period of interruption of blood flow through the descending aorta is required. During the period from 1991 to 1998, five infants with this abnormality underwent surgical repair with the modified method. The results were excellent with no postoperative death and no recoarctation during the follow-up period.     

先天性主动脉缩窄合并心内畸形的外科矫治

目的 探讨先天性主动脉缩窄(CoA)合并心内畸形的外科治疗方法。方法 1994～2001年共收治45例CoA合并心内畸形患者,其中一期手术组26例,分期手术组19例。23例合并有中～重度肺动脉高压(平均肺动脉压56mmHg)：心内畸形以室间隔缺损(VSD)最常见(36例,80％),一期组选择单纯正中切口21例,左外侧和正中双切口5例。分期手术组平均间隔105d行二期手术矫正心内畸形。结果 两组分别死亡2例。24例术后上下肢动脉压差消失,10例平均动脉收缩压差均小于10mmHg平均随访29个月。患者生活质量有明显提高。超声心动图检查提示无假性动脉瘤或主动脉再狭窄。结论 选择一期或分期手术矫治CoA合并心内畸形,手术结果无明显差异,一期手术中选择左外侧和止中双切口方法安全有效。     

Left aortic arch and right descending aorta — interruption or severe coarctation of the aortic arch in the newborn

Three consecutive neonates (aged 7, 14 and 30 days, body-weight 2980g, 3000g and 3400g respectively) with interruption of the aorta (n = 2) and severe coarctation (n = 1) in the presence of left aortic arch and right descending aorta are reported. Associated lesions were an aortopulmonary window in the first case and an unrestrictive ventricular septal defect in the two others. Intractable heart failure and the complexity of the malformation led to the decision of a staged operation. A prosthetic graft was interposed between the ascending and descending aorta via a right thoracotomy in order to bridge the atretic or hypoplastic segment without using extracorporeal circulation. There was no intraoperative complication. One patient developed a thrombocytopenia within the frame of a sepsis syndrome and died on day 5 after operation, death being caused by a massive bleeding into the left thoracic cavity, although the operation was carried out via a right thoracotomy. Angiography 1 year after operation revealed a good flow through the grafts and no stenosis at the site of the anastomoses. The two surviving infants are clinically well without any medication. The reported operative technique provides an alternative palliative possibility to manage critically ill neonates without any obstacle to later definitive repair.     

Management of Ascending Aortic Aneurysm Complicating Coarctation of the Aorta

Four patients with coarctation of the aorta complicated by an aneurysm of the ascending aorta are described. One patient, treated only medically, died suddenly. For the 3 patients who underwent operation, management was similar. Pharmacological control of blood pressure and repair of the aortic coarctation to achieve anatomical reduction in afterload (stage I) were followed after an interval of one to five weeks by surgical repair of the ascending aortic aneurysm (stage II).Initial repair of the coarctation relieves proximal hypertension, thereby decreasing the chance of progressive dissection or rupture of the aneurysm. It also permits safe arterial cannulation for perfusion during correction of the aneurysm in the second stage. In patients not requiring valve replacement in stage II, continued long-term follow-up for progression of aortic valvular disease appears to be necessary.