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。     

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.     

小儿主动脉缩窄或主动脉离断合并心内畸形的诊断与外科治疗

目的探讨主动脉缩窄(CoA)或主动脉离断(IAA)合并心内畸形的诊断及手术方式选择。方法2003年1月至2010年3月济宁医学院附属医院手术治疗14例小儿CoA(9例)或IAA(5例)合并心内畸形[包括室间隔缺损(VSD)、房间隔缺损(ASD)、房室隔缺损(APSD)等畸形,但不包括单纯合并动脉导管未闭(PDA)]患者,其中男10例,女4例;年龄0.7～12.0(3.2±4.5)岁;体重5.5～25.5(10.2±5.5)kg。分期手术3例,经胸骨正中切口径路一期手术矫治11例。结果手术死亡2例,其中1例为分期手术,二次手术行VSD修补术后死于急性左心衰竭;1例术前诊断为VSD+PDA合并重度肺动脉高压,术前未发现IAA,术后死于急性肾功能衰竭。随访12例,随访时间6～84(32±22)个月,患者恢复良好。复查心脏超声心动图提示:胸主动脉轻度狭窄2例,继续随访观察。结论多层螺旋CT和核磁共振成像是目前诊断CoA和IAA的首选检查方法。经胸骨正中切口径路一期手术治疗CoA或IAA合并心内畸形婴幼儿,可获较好的显露及疗效。     

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.     

婴幼儿主动脉缩窄的外科治疗

目的评价近年来婴幼儿主动脉缩窄（CoA）手术疗效。方法外科治疗118例婴幼儿主动脉缩窄病例。年龄21d～3岁,平均（1．8±1．1）岁;体重2．9～13．5 kg,平均（7．5±2．2）kg。单纯CoA或伴动脉导管未闭（PDA）32例,合并其他心内畸形86例,后者大部分在正中切口深低温停循环（DHCA）或深低温低流量（DHLF）下一期纠治CoA和合并畸形。术中停循环21～48min,平均（32．13±11．72）min。结果因肺高压危象和心律失常死亡2例,病死率1．7％。呼吸机应用11～256 h,平均（98．51±6．68）h。术后随访6～24个月,无神经系统并发症,6例有声音嘶哑,其中5例经正中胸骨切口、1例外侧切口进胸施术。超声检查示2例有残余主动脉缩窄,压力阶差分别为29和36min Hg（1mmHg=0．133 kPa）。结论CoA无论是否合并心内畸形,均主张早期手术,正中切口一期根治术手越来越成为首选方案;主动脉远端与主动脉弓下缘广泛端端吻合术（EEEA）等手术方法的应用扩大了手术根治的指征,也提高了术后疗效。     

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;     

新生儿主动脉弓中断合并心内畸形的一期手术矫治长期结果

目的探讨新生儿主动脉弓中断(IAA)合并心内畸形的一期手术矫治效果。方法回顾性分析2003年5月至2014年9月我院采用经胸骨正中切口下一期手术矫治IAA合并心内畸形新生儿21例的临床资料,其中男18例、女3例,年龄6~26(15.9±5.8)d。A型14例,B型7例。手术均采用胸骨正中切口,端侧吻合降主动脉及主动脉弓,同期矫治合并心内畸形。结果机械通气时间14~809(237.8±179.7)h,体外循环时间92~174(132.6±27.1)min,主动脉阻断时间48~118(70.9±18.8)min,深低温停循环时间5~60(28.8±15.5)min,住院时间4~52(28.0±12.1)d。住院期间死亡3例(14.3%),分别因手术后败血症、肺动脉高压危象、心脏骤停而死亡。18例新生儿生存,随访3个月至11年,超声检查示患儿术后心功能良好。结论新生儿IAA合并心内畸形采用经胸骨正中切口一期手术矫治减少了手术次数,提高了患儿生活质量,手术效果良好。     

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.     

一期手术纠治婴幼儿主动脉缩窄或离断合并心内畸形

目的 总结婴幼儿主动脉缩窄或离断合并心内畸形胸骨正中切口一期纠治的外科治疗策略.方法 2004年5月至2010年3月,采用胸骨正中切口一期纠治主动脉缩窄或离断合并心内畸形患儿52例.其中男性32例,女性20例;年龄25 d～7个月,平均(2.03±0.15)个月;体质量3.5～8.0 kg,平均(3.9±0.5)kg.采用自体心包片修补心内缺损;主动脉缩窄者40例,34例采用端侧吻合或扩大端侧吻合术,3例行纵切横缝术,3例假性主动脉缩窄行导管韧带切断主动脉松解;主动脉离断12例,均采用扩大端侧吻合.结果 所有病例心肺转流时间平均为(98±41)min,术中出血量(78±13)ml.1例主动脉离断合并右心室双出口患儿术后因左侧支气管狭窄,脱离呼吸机困难,于术后43 d死亡.其余病例术后生存良好,随访1～6年,术后再缩窄率为11%.结论 采用胸骨正中一期切口纠治主动脉缩窄或离断近期手术效果显著,可避免二次手术.

Abstract：

Objective To sum up one-stage complete correction of infantile aortic coarctation (CoA) or interrupted aortic arch (IAA) associated with intracardiac anomailes through median sternotomy.Methods The clinical data of 52 infants with CoA or IAA associated with intracardiac anomalies from May 2004 to March 2010 was analyzed. There were 32 male and 20 female, aged from 25 d to 7 months with a mean of (2.03 ±0. 15) months, weighted from 2.5 to 8.0 kg with a mean of (3.9 ±0.5) kg. All of intracardia defect were corrected by self-arcula cordisand. Forty cases with CoA were underwent by operative techniques, including resection with end to side anastomosis, extented end to side anastomosis (n = 34),and vertical incision and cross joint ( n = 3). Three cases of pseudo-CoA were cuted and ductus arterissus or ligamentum arteriosus and dissected aorch. Twelve cases of IAA were underwent by extented end to side anastomosis. Results The time of cardiopulmonary bypass was (98 ± 41 ) min, and all patients hemorrhaged (78 ± 13) ml during operation. One case of IAA associated with double outlet right ventricle died after 43 d post-operation because of left bronchinal stenosis. The other patients were in good condition.The rate of aneurysm formation was 11% in 1 to 6 years' follow-up. Conclusions One-stage complete correction of infantile CoA or IAA associated with intracardiac anomailes through median sternotomy yieldes excellent intermediate surgical results. This operative approach is beneficial, not only with shorten period of therapy and loss operative cost.     

婴儿主动脉缩窄合并心内畸形的一期矫治

目的总结婴儿主动脉缩窄（CoA）合并心内畸形一期矫治的手术方法和临床经验。方法2001年1月至2006年1月，对28例CoA合并心内畸形患者行一期手术矫治。CoA为导管前型18例，邻近或正对导管处10例；伴主动脉弓发育不良6例，动脉导管未闭22例。合并的心内畸形包括：室间隔缺损16例、室间隔缺损＋房间隔缺损5例、完全性房室间隔缺损3例，完全型大动脉错位伴室间隔缺损（D—TGA／VSD）2例，右心室双出口2例。采用左胸后外侧联合胸骨正中切口（双切口）径路手术12例，胸骨正中切口径路（单一切口）手术16例。采用缩窄段切除端端吻合术20例，Gore—Tex补片扩大成形术4例，左锁骨下动脉翻转扩大成形术4例。结果全组无手术死亡，术后呼吸机辅助呼吸时间7h～13d，住ICU时间3～18d。因术后心脏扩大延迟48～72h关胸4例，术后发生急性肾功能衰竭行腹膜透析3例，吸入一氧化氮（NO）治疗重度肺动脉高压3例。术后下肢收缩压高于上肢10～20mmHg18例（64．3％），收缩压上、下肢相差不大8例（28．6％），上肢分别高于下肢15mmHg和20mmHg2例（7．1％）。所有患者均得到随访，随访时间3～50个月，1例D—TGA／VSD患者因重度三尖瓣反流、肺部严重感染和呼吸功能衰竭于术后3个月死亡；其余27例患者恢复顺利。心脏超声心动图提示：1例采用端端吻合法、1例采用Gore～Tex补片扩大成形术的患者在CoA纠治处仍存在压差，分别为25mmHg、28mmHg，均未再次手术。结论一期手术矫治CoA合并心内畸形可以缩短疗程，有利于患者术后心、肺功能的恢复，避免二次手术的痛苦，降低治疗费用。可采用单一胸骨正中切口或胸骨正中联合左胸后外侧切口完成手术。     

Surgical treatment of coarctation and interrupted aortic arch complex in infants

One hundred forty-two consecutive neonates or early infants with coarctation and interrupted aortic arch complex who underwent biventricular repair at the Fukuoka Children's Hospital between January 1991 and December 2000 were reviewed. One-stage repair was performed in 33 patients (35%) with coarctation complex and in 41 patients (85%) with interrupted aortic arch complex. The overall mortality rate was 6.1% in one-stage repair and 6.6% in two-stage repair of coarctation complex patients and 9.8% in one-stage repair and 28.6% in two-stage repair of interrupted aortic arch complex patients. The recoarctation rate was 5.3% in coarctation complex and 2.1% in interrupted aortic arch complex. All patients with recoarctation underwent successful catheter intervention and required no reoperation. In conclusion, one-stage repair of interrupted aortic arch and coarctation complex with the anterior approach resulted in good outcomes. Then descending aorta cannulation through a median sternotomy combined with the cerebral perfusion technique enables complete avoidance of circulatory arrest and is a useful technique. However, a two-stage procedure can be useful in the patients whose condition has deteriorated substantially or in whom intracardiac anomalies are severe.     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

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。     

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.     

One-stage repair with separated cardiopulmonary bypass for coarctation of the aorta with left aortic arch and right thoracic descending aorta

We treated a 7-day-old neonate with aortic coarctation, left aortic arch, right-side descending aorta, aberrant right subclavian artery, and outlet ventricular septal defect. Surgical one-stage repair consisting of coarctation repair by extended direct anastomosis, patch closure of the ventricular septal defect, and translocation of the right subclavian artery was performed under a separated (upper and lower body) perfusion with ascending and thoracic descending aortic cannulation. The postoperative course was complicated with massive chylous effusion, which was successfully treated by thoracic duct ligation.     

One-stage total repair of aortic arch anomaly using regional perfusion.

OBJECTIVE: Primary repair of aortic arch obstructions and associated cardiac anomalies is a surgical challenge in neonates and infants. Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction. METHODS: From March 2000 to December 2005, 69 neonates or infants with aortic arch anomaly underwent one-stage biventricular repair with continuous cerebral perfusion in the presence of a nonworking beating heart using the dual perfusion technique on the innominate artery and aortic root. Preoperative diagnoses of arch anomaly comprised aortic coarctation (n=54) or an interrupted aortic arch (n=15). Combined anomalies were ventricular septal defect (n=52), anomalous origin of the right pulmonary artery from ascending aorta (n=3), hypoplastic left heart syndrome (n=2), truncus arteriosus (n=2), atrioventricular septal defect (n=2), double outlet right ventricle (n=1), total anomalous pulmonary venous return (n=1), partial anomalous pulmonary venous return (n=1), and aortic stenosis (n=1). RESULTS: The mean regional perfusion time was 27.8+/-9.8 min. There was no operative mortality. Postoperative low cardiac output was present in four patients (5.8%). A neurologic complication was noted in one patient (1.5%) who developed transient chorea, but recovered completely. During 32.8+/-17.5 months of follow-up, one late death (1.5%) occurred. There was neither reoperation associated with arch anomaly nor recoarctation except in one patient. One patient developed left main bronchial compression necessitating aortopexy. CONCLUSIONS: One-stage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants.     

Off-Pump Extraanatomic Bypass from the Ascending to the Descending Aorta for Re-Operation of Interrupted Aortic Arch in an Adolescent

Abstract   The appearance of re-stenosis after repair of an interrupted aortic arch may be a surgical challenge due to adhesions. Here, we describe an approach using off-pump coronary artery bypass grafting techniques to reach the descending aorta through a median sternotomy in a patient with aortic arch stenosis after conduit repair. The 17-year-old patient with diagnoses of interrupted aortic arch and ventricular septal defect presented after two previous operations (one left lateral thoracotomy and one median sternotomy) with a stenosed vascular graft between ascending and descending aorta. Surgery was done via re-sternotomy without cardio-pulmonary bypass. An extraanatomic graft was used to connect ascending and descending aorta. When performing the distal anastomosis, the heart was exposed using a standard suction device. This case demonstrates that the use of modern techniques may facilitate surgical approaches dramatically. In our opinion the above-described technique is the first choice for all patients requiring arch repair following multiple previous operations, performed via sternotomy and thoracotomy.