先天性心脏病合并右位主动脉弓的诊断与治疗

目的 探讨先天性心脏病合并右位主动脉弓的诊断与治疗。 方法 回顾性分析2012年1月1日至2013年4月1日北京儿童医院小儿心脏中心27例先天性心脏病合并右位主动脉弓行外科手术治疗患儿的临床资料,其中男20例,女7例;年龄（10.96±12.08） 个月,体重（7.70±3.13） kg。法洛四联症14例,室间隔缺损9例,动脉导管未闭1例;肺动脉吊带1例;单纯双主动脉弓2例;均合并右位主动脉弓。其中13例合并血管环畸形,包括迷走左锁骨下动脉畸形和双主动脉弓畸形。所有患儿均接受手术治疗,在矫治心内畸形的同时,进行左锁骨下动脉移植,矫治迷走左锁骨下动脉畸形,双主动脉弓主要是切断一侧非优势弓。 结果 围术期死亡3例,其中1例术后无法停体外循环死亡,1例未合并血管环的患儿术后7 d死亡,1例合并双主动脉弓患儿术后并发急性呼吸窘迫综合征（ARDS） 死亡;1例放弃治疗。随访23例,随访时间3～17个月,心脏超声心动图提示心内畸形矫治满意,恢复良好,双侧上肢动脉波动良好。 结论 对先天性心脏病合并右位主动脉弓患者行外科矫治时应慎重,术前尽量完善相关检查,选择增强CT、磁共振成像检查,明确诊断,制定手术计划。一期同期矫治血管环畸形及其它先天性心脏病,手术效果满意,近期效果良好。     

先天性主动脉弓中断及合并畸形的一期手术治疗

目的回顾总结先天性主动脉弓中断及合并畸形一期手术治疗经验。方法1988年7月至2004年8月,手术治疗主动脉弓中断及合并畸形20例。男12例,女8例;手术年龄18d～9岁。其中<1岁者10例,平均年龄(3.37±2.45)个月,平均体重(5.33±1.72)kg;>1岁者10例,平均年龄(2.60±1.24)岁,平均体重(13.10±6.53)kg。所有病儿术前均有反复呼吸道感染史。A型15例,B型5例。2例经双切口手术,即先通过左后外侧切口纠治主动脉弓中断,再经正中切口纠治合并畸形。18例采用正中切口同时纠治主动脉弓中断和合并畸形。结果全组死亡3例,死于术后严重肺动脉高压危象2例、严重心功能衰竭1例。17例随访3个月～12年,均恢复良好。结论主动脉弓中断采用正中切口方法,操作简便,暴露清楚,创伤小,有利于术后恢复。本病早期即出现肺动脉高压,临床上一经确诊宜尽早手术。     

一期手术矫治主动脉弓缩窄合并心内畸形

目的：探讨主动脉弓缩窄合并心内畸形病人的一期手术矫治方法及治疗效果。方法：自１９８９年１２月至１９９８年２月,运用一期手术方法为１０例主动脉弓缩窄合并心内畸形病人进行外科矫治。合并的心内畸形有室间隔缺损７例,二尖瓣关闭不全２例,主动脉瓣狭窄１例;６例同时合并动脉导管未闭。采用左后外侧切口矫治主动脉弓缩窄,正中切口行心内畸形矫治６例、正中切口采用主肺动脉内隧道同时矫治主动脉弓缩窄及合并心内畸形３例;     

自体肺动脉组织一期矫治主动脉弓病变合并心内畸形

目的 探讨自体肺动脉组织一期矫治主动脉弓病变合并心内畸形的手术方法和治疗效果.方法 1993年4月至2009年11月,共25例主动脉弓病变合并心内畸形的患儿接受了一期矫治手术.男性17例,女性8例;年龄26 d～6.5岁,平均11个月;体质量3.0～14.5 kg,平均12.4 kg.其中6例患儿为主动脉弓缩窄,6例为主动脉弓中断,13例为主动脉弓发育不良.手术技术包括切开肺动脉前壁,切除动脉导管,应用部分自体肺动脉壁重建主动脉弓,重建肺动脉,并同时矫治合并心内畸形.结果 24例患儿术后恢复顺利,1例患儿死于围手术期肺动脉高压危象.随访心脏超声及心脏CT检查证实主动脉弓重建及心内畸形矫治满意.术后未发生神经系统及其他并发症.随访过程中,患儿生长发育正常,主动脉弓无再狭窄.结论 自体肺动脉具有生长潜力且柔韧性好,是一种理想的重建主动脉弓的材料.一期矫治手术治疗先天性主动脉弓病变合并心内畸形可以得到满意的结果.     

主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治

目的 总结主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治经验.方法 2003年2月至2007年8月,一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压病婴6例,男、女各3例.手术年龄18 d～14个月,平均(8.3±4.5)个月;平均体重(8.5 ±2.2)kg.A型4例,B型2例.所有病例术前均有反复呼吸道感染史.手术均采用胸骨正中切口.结果 全组病例均生存.随访3～36个月,恢复良好.结论 一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压效果良好,需同时加强对围术期肺动脉高压的管理.     

婴幼儿主动脉弓中断合并心内畸形胸骨正中切口一期手术治疗

目的 探讨婴幼儿主动脉弓中断(IAA)合并心内畸形胸骨正中切口一期矫治手术效果以及影响因素.方法 2005年8月至2012年1月,采用胸骨正中切口体外循环(CPB)下一期纠治IAA合并心内畸形患婴23例,其中男12例,女11例;年龄18天～3岁,体质量(6.61 ±3.26) kg.按Rastelli 分型,A型20例,B型3例.患儿均合并粗大动脉导管未闭及大型室间隔缺损,其他合并畸形包括主肺动脉窗、右肺动脉起源于主动脉、主动脉瓣两瓣畸形和主动脉瓣下狭窄等.患儿术前均行超声心动图检查,14例行64排CT检查,4例行造影检查.22例术前明确诊断,1例术中明确诊断.行主动脉直接吻合3例,补片扩大主动脉19例,人工管道经肺动脉内连接主动脉弓1例;同期矫治合并心内畸形.结果 全组体外循环(129.76±38.98) min,主动脉阻断(74.47±24.30)min,住ICU院平均96 h.术后2例死亡,其中1例2月龄患婴死于肺动脉高压危象,1例7月龄患婴术中出血、术后死于严重低心排血量综合征.21例患儿生存,随访2个月～6年,超声检查示患儿术后心功能良好;5例术后6个月上、下肢平均压差＞30 mm Hg(1 mm Hg =0.133 kPa).无因再狭窄再次手术患儿.无神经系统并发症发生.结论 IAA采用胸骨正中一期外科治疗简化了手术过程,减少了手术死亡,提高了患儿的生活质量,早、中期预后良好,是一种值得推广的治疗方法.     

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.     

先天性血管环的外科治疗

目的 讨论先天性血管环的诊断和手术治疗及影响预后的因素.方法 回顾性分析2010年1月至2013年12月收治的42例先天性血管环患儿(占同期手术患儿的1.04％)的病例资料.其中男性26例,女性16例;手术年龄24d至6岁,平均10.7个月.包括肺动脉吊带26例,双主动脉弓10例,右位主动脉弓合并迷走左锁骨下动脉3例,肺动脉吊带合并右位主动脉弓并迷走左锁骨下动脉2例,肺动脉吊带合并左位主动脉弓并迷走右锁骨下动脉1例.38例患儿合并其他的心血管畸形.36例患儿行螺旋CT检查气道重建,23例患儿行纤维支气管镜检查,除2例气道未见异常外其余患儿均存在不同部位不同程度的气管狭窄或软化.所有患儿均行手术矫治先天性血管环,根据情况同期行心内畸形矫治手术;1例同期行气管狭窄切除并Slide成形术,1例在术后行气管内支架植入术.结果 3例患儿院内死亡(7.1％),死亡原因均为术后反复发生气管内肉芽肿形成;其余39例均顺利出院.存活患儿总体术后呼吸机辅助时间14 h(22 h)[M(QR)],心脏ICU停留时间5d(8 d),住院时间19 d(9 d).肺动脉吊带患儿呼吸机辅助时间、心脏ICU术后停留时间均长于双主动脉弓患儿[23 h(123 h)比9 h(9 h),7 d(13 d)比4 d(2 d)],但差异无统计学意义(P＞0.05);住院时间明显长于双主动脉弓患儿[23 d(9 d)比16 d(6 d)],差异有统计学意义(x2=10.157,P=0.006).结论 先天性血管环早期手术治疗是安全、有效的,近期效果良好.合并的气管软化和狭窄的程度和范围是影响患儿预后的关键.     

一期手术矫治先天性主动脉弓中断

目的　探讨先天性主动脉弓中断 (IAA)一期手术矫治的手术方法、疗效 ,总结其临床经验。　方法　对10例少见的先天性 IAA进行一期手术矫治 ,平均手术年龄 2 .7± 2 .4岁 ,其中 5例为 A型 IAA,3例为 B型 ,另 2例IAA合并残存第 5弓狭窄 ;8例患者均合并其它心血管畸形和重度肺动脉高压。一期矫治术中有 7例进行了主动脉弓直接端侧或端端吻合连接术 ,2例行 Gore- Tex管道连接重建主动脉弓 ,1例 IAA合并残存第 5弓狭窄用自身心包补片作狭窄处扩大成形术 ;8例患者于矫治 IAA的同时矫治心血管其他畸形。　结果　术后早期发生心功能不全、心律失常、肺动脉高压危象等并发症 5例 ,其中近 10年仅发生 1例。住院死亡 3例 ,近 10年连续 6例无住院死亡。术后早期 5例肺动脉收缩压 /体循环动脉收缩压 (Pp/ Ps)由术前的 0 .84± 0 .0 4显著下降至正常范围 (0 .2 8± 0 .0 3) ,1例主动脉弓部压力阶差为 30 mm Hg(1k Pa=7.5 mm Hg)。随访 7例 ,平均随访 2 .6± 4 .0年 ,均存活 ,其中有 3例主动脉弓部压力阶差≥ 30 mm Hg。心功能均正常。　结论　先天性 IAA一旦诊断明确 ,应尽早进行一期矫治术 ;主动脉弓直接吻合连接术效果较佳。     

婴幼儿血管环外科诊治

目的 总结婴幼儿先天性血管环外科诊治的临床经验.方法 2009年9月至2010年12月诊断并手术治疗14例血管环患儿,其中男9例,女5例;年龄2月～6岁;体重4.5～15.0 kg.所有病例术前均行X线胸片、超声心动图及增强多排螺旋CT检查确诊,行食管造影及纤维支气管镜检查各5例.病理类型包括双主动脉弓1例、肺动脉吊带7例、右位主动脉弓伴左位动脉导管/韧带6例,7例患儿合并心内畸形.全组患儿存在不同范围(12％～62％)和程度(45％～74％)的气管狭窄,5例术前存在局限性食管狭窄.12例患儿在体外循环下手术同期矫治心内畸形,2例在非体外循环下手术.结果 术后辅助呼吸7～ 308 h,中位数21 h;ICU停留16 ～314 h,中位数79.5 h.1例肺动脉吊带患儿住院死亡(7.1％).13例出院.失访1例,12例随访1～ 15个月,患儿消化道症状消失,活动耐力改善.呼吸道症状不同程度缓解,5例(41.7％,5/12例)仍然存在呼吸道症状.结论 临床上对有长期呼吸道和(或)消化道症状的患儿需考虑血管环的可能,增强CT是确定血管环诊断的最佳方法.血管环应尽早手术治疗,合并的长段气管狭窄需手术治疗,近-中期效果良好.     

Vascular rings and slings. Diagnosis and surgical treatment of 49 patients

Forty-nine infants with symptomatic vascular rings and slings, ranging in age from 20 days to 12 months, required surgical intervention between 1973 and 1984. The following anomalies were present in our patients: double aortic arch with left descending aorta (14), double aortic arch with right descending aorta (6), anomalous innominate artery (13), right aortic arch with aberrant left subclavian artery (4), left aortic arch with aberrant right subclavian artery (10), aberrant left pulmonary artery (pulmonary sling) (2). All the babies had symptoms related to compression of the trachea and/or esophagus. Four patients required temporary tracheostomy in the early postoperative period; 1 patient, affected by a pulmonary sling, required tracheal resection and anastomosis, for severe tracheomalacia. There was one hospital death in a patient with severe tracheal compression from an anomalous innominate artery and brain damage as a result of metabolic problems. Forty-eight patients survived and follow-up ranged from 3 months to 11 years. For each type of vascular anomaly encountered, and based on personal experience, we have outlined a diagnostic scheme allowing an accurate morphological definition and a subsequent surgical procedure.     

Single-Stage Arterial Switch With Aortic Arch Enlargement for Transposition Complexes With Aortic Arch Obstruction

Background. Patients with transposition complexes and aortic arch obstruction are a surgical challenge with significant mortality. We have adopted an aggressive approach of concurrent aortic arch repair and arterial switch operation with excellent results.

Methods. Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch.

Results. There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view.

Conclusions. Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.     

永存第五弓残存伴狭窄合并主动脉弓中断的外科治疗

目的总结永存第五弓残存伴狭窄合并主动脉弓中断的外科治疗经验,以提高手术疗效。方法自2000年1月至2008年5月,共手术治疗永存第五弓残存伴狭窄合并主动脉弓中断5例,手术年龄1．8～108．0个月,体重3．7～31．0kg。3例患者术前存在慢性心功能不全及反复呼吸道感染,5例患者均一期纠治第五弓狭窄／及合并的心血管畸形。结果手术死亡2例,1例术后并发心功能不全,肺动脉高压危象,严重肺部感染,依赖呼吸机,撤机困难,家属放弃治疗;另1例合并室间隔缺损,肺动脉高压,死于反应性肺动脉高压危象、低心排血量综合征和左心功能衰竭。术后随访3例,随访时间55．67±48．64个月,随访期间无死亡,无并发症发生。其中1例患者已随访8年,恢复正常学习,心脏磁共振成像（MRI）提示：第五弓与降主动脉连接处（Gore—Tex补片扩大修补处）轻度狭窄,直径9．3mm。结论永存第五弓残存伴狭窄合并主动脉弓中断患者手术采用胸骨正中切口径路,操作简便,暴露清楚,剖面小,有利于术后恢复。由于该病晚期亦出现体动脉高压,应尽早明确诊断及时手术治疗。     

完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。     

Asymptomatic balanced-type double aortic arch in an elderly patient: a case report

Double aortic arch is a congenital abnormality and sporadic cases have been reported in adult patients, who are usually diagnosed after complaining of asthma-like symptoms or swallowing difficulties because of the compression of the trachea or esophagus by the abnormal aortic arches. We present the case of a 67-year-old male patient with double aortic arch, found coincidentally during coronary angiographic examination.     

主肺间隔缺损的外科治疗

目的 探讨主肺间隔缺损的外科治疗经验.方法 1987年12月至2007年3月,16例主肺间隔缺损病儿(人),其中男11例,女5例,年龄5个月～22岁,平均(8.2±7.1)岁;体重5.8～50.0 kg,平均(22.2±13.9)kg.Mori分型Ⅰ型7例,Ⅱ型7例,Ⅲ型2例.主肺间隔缺损大小10～35mm,平均(22.6±7.3)mm.其中合并室间隔缺损、房间隔缺损和肺主动脉弓离断畸形(B型)各2例,动脉导管未闭1例.术前10例行心导管检查,肺动脉收缩压50～120 mm Hg(1 mm Hg=0.133 kPa),平均(75.4±25.2)mm Hg,肺动脉平均压21～100 mm Hg,平均(60.3±24.8)m Hg.14例在中低温体外循环下完成手术,2例在深低温停循环完成手术.结果 主动脉阻断(70.6±48.1)min,体外循环(110.5±62.9)min.术后10例肺动脉收缩压(50.2±18.5)mm Hg,平均肺动脉压(40.0±15.2)mm Hg,术后呼吸机使用(22.6±14.5)h,滞留蕈症监护室1～70 d,总住院14～127 d.2例合并主动脉弓离断者围术期分别死于急性肾衰和术后低心排出量综合征.14例痊愈,平均随访(8.8±7.5)年,1例主肺动脉间隔小残余漏,余心功能明显改善.结论 主肺间隔缺损者肺血管病理改变早,早期诊断、及时手术,能降低病死率.     

"杂交"手术治疗DeBakey Ⅰ型主动脉夹层

目的 总结应用无深低温停循环的升主动脉置换、升主动脉-主动脉弓分支血管旁路、腔内隔绝的"杂交"手术治疗DeBakey Ⅰ型主动脉夹层的方法和经验.方法 2009年1月至2010年6月,39例、平均年龄(55±16)岁的DeBakey Ⅰ型主动脉夹层病人进行无深低温停循环的"杂交"手术.病人经股动脉、右腋动脉插管灌注,先于常规体外循环下行升主动脉和主动脉瓣置换,再采用四分支人工血管、"Y"形人工血管或单根人工血管行升主动脉-主动脉弓分支血管旁路手术,再经股动脉逆行径路数字减影血管造影(DSA)下或术中顺行径路食管超声定位下行主动脉弓-降主动脉腔内隔绝术.结果 全组均手术成功,升主动脉处理时8例行单纯升主动脉人工血管置换,20例行Bentall手术(其中冠状动脉移位采用Carbrol法11例),11例行Wheat手术;升主动脉-主动脉弓分支血管旁路手术采用四分支人工血管16例、"Y"形人工血管15例、单根人工血管序贯法8例;主动脉弓-降主动脉腔内隔绝术采用DSA下股动脉逆行径路36例,术中人工血管分支顺行径路3例,均使用1枚支架.体外循环(61±22)min,主动脉阻断(48±18)min.术后(30±9)h拔除气管插管,24 h胸液小于300 ml,无偏瘫、截瘫、严重感染、凝血障碍等并发症.所有病人均治愈出院,平均术后(21±6)天出院.术后随访1～15个月,平均(8.4±7.2)个月,病人均健康生存,无脏器功能不全.术后3个月行CT主动脉血管成像检查,显示膈肌水平假腔闭合率为91.2%.结论 常温体外循环下升主动脉置换、升主动脉-弓部血管旁路、腔内隔绝的"杂交"手术治疗DeBakey Ⅰ型主动脉夹层,简化了DeBakey Ⅰ型主动脉夹层外科治疗方法,该手术方法避免了深低温停循环,减少了术后并发症,提高了外科治疗效果.

Abstract：

Objective Some major procedures for DeBakey type Ⅰ aortic dissection used to be performed with deep hypothermic circulatory arrest, which had been associated with more complications than seen with standard extracorporeal circulation. We reviewed the cases who received the treatment for DeBakey type Ⅰ aortic dissection by hybrid procedure without deep hypothermic circulatory arrest. The procedure consisted of ascending aorta replacement, ascending aorta-aortic arch branch vascular bypass reconstruction and endovascular graft exclusion. Methods From January 2009 to June 2010, 39 patients [mean age (55 ±16) years] who had DeBakey Ⅰ aortic dissection underwent hybrid procedure without deep hypothermic circulatory arrest. The femoral artery and right axillary artery were cannulated for perfusion. The ascending aorta and/or aortic valves were replaced under conventional extracorporeal circulation with Bentall procedure or Wheat procedure. The aortic arch branch vessels were dissected and the proximal part was sealed. Then the ascending aorta-aortic arch branch vascular bypasses were constructed with 4-bifurcation vascular grafts, Y-shape bifurcated vascular grafts or artificial vessels. Finally the endovascular grafts were deployed via the femoral incisions monitored dynamically with DSA, or via the ascending aortic bifurcated vessels monitored with transesophageal echocardiography. Results The operation succeeded in all 39 patients. Eight patients underwent ascending aorta replacement without aortic valve replacement or prosthesis, 20 patients underwent Bentall procedure ( Carbrol procedure were used in 11 cases), and 11 underwent Wheat procedure. For ascending aorta-aortic branch vascular bypass reconstruction, sequential anastomoses were performed in 8, Y-shaped bifurcated grafts were used in 15, and 4-bifurcated grafts were employed in 16 patients. The endovascular stent grafts were deployed via the former femoral incisions in 36 patients and via ascending aortic bifurcated vessels in 3. The cardiopulmonary bypass time was (61 ±22) minutes, the aortic crossclamp time was (48 ±18) minutes, and the post-operative intubation time was (30 ±9) hours. The thoracic drainage from each patient was less than 300 ml in 24 hours. No complication, such as hemiplegia, paraplegia, severe infections, renal failure or coagulation disorder, was observed. The duration of hospitalization was (21 ±6) days. No hospital death occurred. Follow-up was performed 1 to 15 months [mean (8.4 ±7.2) months] postoperatively. All patients survived without any organ dysfunction at follow up. The CTA examination 3 months after operation revealed that the false lumens had been closed in 91.2% of the patients. Conclusion Our findings indicated that the hybrid procedure, which combining ascending aorta replacement,ascending aorta-aortic arch branch vascular bypass reconstruction and endovascular graft exclusion under conventional extracorporeal circulation, may be an option for avoiding the possible complications associated with profound hypothermic circulatory arrest. The novel hybrid operation may improve the surgical outcomes and provide a simplified surgical approach for the treatment of DeBakey Ⅰ aortic dissection.     

68例主动脉夹层外科治疗体会

目的 总结主动脉夹层（aortic dissection,AD）的外科治疗经验.方法 2006年10月至2012年5月手术治疗AD 68例.急性AD（发病＜2周）57例,慢性AD 11例.其中DeBakey Ⅰ型11例,DeBakeyⅡ型7例,DeBakeyⅢ型50例.18例DeBakey Ⅰ、Ⅱ型患者进行体外循环手术,48例DeBakeyⅢ型患者进行主动脉腔内隔绝术（endovascular aortic repair,EVAR）,1例DeBakeyⅢ型患者EVAR术中造影示双侧股动脉严重狭窄而转行体外循环手术治疗,1例DeBakeyⅢ型患者EVAR失败后行双侧股动脉转流术.结果 体外循环手术组死亡2例,死亡率10.5％,EVAR手术组死亡1例,死亡率2.1％,全组患者围手术期死亡3例,总死亡率5.8％.出院患者随访6～ 79个月,平均（15.2±18.7）个月,无死亡,无再次手术,生活质量良好.结论 早期明确诊断和个体化的手术方案能提高AD生存率.     

Diagnosis and management of congenital vascular rings: a 22-year experience.

Between 1968 and 1990, we operatively treated 39 patients (19 boys, 20 girls) with congenital aortic arch anomalies. Median age was 7 months (range, 1.5 months to 23 years). Thirty-seven patients (95%) had respiratory symptoms. Barium swallow was diagnostic in 95%. Right arch with aberrant left subclavian artery and double aortic arch were the most common types (11 each). Treatment of an aortic diverticulum was documented in 19 patients; the aortic diverticulum was excised (9), managed by aortopexy (7), or left in situ (3). Postoperative recovery was rapid, with a median intensive care unit stay of 2 days, time to oral feeding of 1 day, and postoperative time to discharge of 7 days. Two deaths occurred: 1 infant had undergone emergent operation for control of hemorrhage from an aortotracheal fistula due to tracheostomy tube erosion, and the other had multiple associated congenital heart defects. Postoperative complications included bleeding (1), pneumonia (5), and chylothorax (4). One boy had persistent severe symptoms due to an untreated aortic diverticulum and underwent subsequent excision of the aortic diverticulum with complete relief of symptoms. Median length of follow-up was 12.5 months, with at least 97% of survivors completely or nearly completely free of symptoms from the vascular ring. These results suggest that early repair of congenital aortic vascular rings, including fixating or excising an associated serious aortic diverticulum, is safe and effective and allows for normal tracheal growth.