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。     

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.     

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.     

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.     

主动脉缩窄或主动脉弓中断合并心内畸形一期修复

目的 探讨采用经胸骨正中切口一期修复主动脉缩窄(CoA)或主动脉弓中断(IAA)合并心内畸形的治疗效果.方法 2002年7月至2009年6月,经胸骨正中切口行降主动脉远端和主动脉弓下缘端侧吻合术一期修复CoA或IAA合并心内畸形病儿43例,其中CoA 34例,IAA 9例(A型6例、B型3例),合并心内畸形包括室间隔缺损42例、动脉导管未闭34例、房间隔缺损12例、主动脉瓣下隔膜狭窄5例、二尖瓣关闭不全2例,右心室双出口1例.结果 手术死亡1例,为术后肺动脉高压和严重低心排血量综合征者.术后并发症包括严重低心排血量综合征3例,低氧血症6例,肺部炎症11例,肺不张14例,声音嘶哑19例,室上性心动过速23例.8例失访.34例随访3个月～5年,生活质量明显改善,心脏超声心动图和CT检查显示吻合口无明显再缩窄发生.结论 经胸骨正中切口,采用主动脉远端和主动脉弓下缘端侧吻合技术一期修复CoA或IAA合并心内畸形的手术早、中期效果良好,能明显减少术后再狭窄.     

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。     

降主动脉-升主动脉吻合术矫治婴儿期主动脉缩窄合并心内畸形15例

目的探讨降主动脉-升主动脉吻合术治疗婴儿期主动脉缩窄合并主动脉弓发育不良及心内畸形的疗效。方法选取2011-05—2015-05间治疗的主动脉缩窄合并主动脉弓发育不良、心内畸形的患儿15例。患儿均采用胸骨正中切口、开胸后先游离出主动脉弓、头臂干、动脉导管、弓降部等血管。建立体外循环、选择性脑灌注下行降主动脉-升主动脉端侧吻合术,恢复全身灌注后完成心内畸形的矫治。体外循环时间86~132 min,主动脉阻断时间51~94 min。结果术后早期死亡2例,低心排出量综合征6例,室上性心动过速6例,肺炎7例。13例患儿随访2个月~3 a,无死亡及再次主动脉狭窄。结论降主动脉-升主动脉吻合术治疗婴儿期主动脉缩窄合并主动脉弓发育不良及心内畸形的临床效果满意。     

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。     

Repair of interrupted aortic arch and associated malformations in infancy: indications for complete or partial repair

There is uncertainty regarding the best method of repair of interrupted aortic arch. The question is whether to perform primary definitive repair of this anomaly plus the associated defects versus arch repair only and palliation of the intracardiac defects, usually by pulmonary artery banding. Since 1976, 16 infants with interrupted aortic arch have been treated surgically. They were seen at 5.2 +/- 3 days of age and weighed 3.2 +/- 0.7 kg. The interruption occurred between the left carotid and left subclavian arteries (type B) in 9 and between the left subclavian artery and the descending aorta in 7 (type A). Isolated ventricular septal defect (VSD) was the only associated anomaly in 7 and aortopulmonary window, in 4. Two patients had truncus arteriosus type 1. Three had transposition of the great arteries: 1 with VSD and 2 with single ventricle. Prior to 1980, our policy was to palliate all patients. Between 1976 and 1980, 4 infants underwent left thoracotomy with arch repair plus pulmonary artery banding (3, VSD; 7, transposition of the great vessels and single ventricle) with only 1 (25%) survivor. Because of this high mortality, 8 patients with interrupted aortic arch and VSD or aortopulmonary window, seen since 1980, received complete repair with median sternotomy, end-to-end arch anastomosis, and closure of the VSD or aortopulmonary window utilizing profound hypothermia and circulatory arrest. All 8 survived.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Off pump repair of aortic arch anomalies with concomitant intracardiac defects via anterior approach

BACKGROUND: The authors evaluated the surgical treatment of aortic arch anomalies associated with intracardiac pathologies, through median sternotomy on beating heart without using cardiopulmonary bypass (CPB). METHODS: A consecutive series of 10 patients with aortic coarctation were operated upon. Median age at repair was 3.5 months (range, 5 days to 72 months), median weight was 4 kg (range, 2.2 to 30 kg). All aortic obstruction repairs were done via midsternotomy without using CPB and it is used only for repair of intracardiac defects. The aortic reconstruction included resection and end-to-side anastomosis in six patients and pulmonary autograft patch aortoplasty in four patients. RESULTS: There was no operative mortality. Mean follow-up value was 17.6 +/- 8.07 months. There was no restenosis. CONCLUSION: Most of the aortic coarctation and interrupted aorta type A can be well-treated surgically through median sternotomy without using CPB. Thus, the need for profound hypothermia and circulatory arrest and its potential neurological and other side effects are removed and CPB is reserved only for associated intracardiac defects, if present.     

Staged repair of interrupted aortic arch with ventricular septal defect compared with primary repair in infancy

Interrupted aortic arch is a poor prognosis cardiac anomaly with nearly 100 percent mortality if not recognized and treated early. The associated intracardiac lesions often lead to death if only the arch defect is repaired. Several recent reports have described patients with interrupted aortic arch who were treated as infants by primary repair of the arch defect with simultaneous repair of the intracardiac lesions. The improved survival data from these series have been attributed to the simultaneous repair of both lesions. We report herein on nine patients with both interrupted aortic arch and ventricular septal defect seen at Children's Hospital and Medical Center in Seattle from 1979 to 1987. Three patients had partial expression of DiGeorge's syndrome. All patients underwent primary repair of the interrupted aortic arch with concomitant pulmonary artery banding during infancy (mean age 18 days, range 2 days to 4 months). Operative mortality was 11 percent (1 of 9 patients). Eight patients had eventual repair of the ventricular septal defect (mean age 18 months, range 6 to 29 months) with one death occurring at 5 months postoperatively (12 percent mortality). The overall mortality of these nine patients was 22 percent. Staged repair of interrupted aortic arch with associated ventricular septal defect can be performed with results comparable to simultaneous primary repair in infancy. The improved survival from either approach is more likely to be attributable to improved perioperative stabilization, particularly the use of prostaglandin E.     

Total anatomic correction of interrupted aortic arch complex. Experience in 4 infants

In two cases of interrupted aortic arch (IAA) of type A, one associated with a ventricular septal defect (VSD) and one with an aortopulmonary window, and two of type B, both associated with a VSD, total anatomic repair was performed at respective ages of 6 months and 24, 8 and 3 days. All four operations were performed through a median sternotomy, using profound hypothermia and circulatory arrest. The repair included resection of the patent ductus arteriosus, direct end-to-side anastomosis of the descending to the ascending aorta and closure of the VSD or, in one case, of the aortopulmonary window. The two oldest infants (with type A IAA) survived. Reexamination two years postoperatively demonstrated good width of the aortic anastomosis with no gradient. In the child who had had an aortopulmonary window there was a proximal tight stenosis of the right pulmonary artery, which was corrected at reoperation. Total anatomic correction of IAA through an anterior approach is technically feasible and the aortic anastomosis seems to grow satisfactorily. The management of very sick neonates with IAA remains a great challenge.     

先天性永存动脉干的外科治疗

报道１９８９年１月至１９９３年１２月外科治疗先天性永存动脉干６例。按ＶａｎＰｒａａｇｈ分型，Ｉ型２例、ＩＩ型４例。Ｉ型中伴主动脉弓中断１例，按Ｃｅｌｏｒｉａ等分类为Ａ型。伴其它心内畸形３例。手术在中度低温体外循环下进行，其中１例采用深低温停循环技术处理主动脉弓中断。手术５例成功，随访７个月～４年８个月，疗效良好。１例术后肺动脉高压并发感染，自动出院后失访。作者主张永存动脉干一旦确诊宜早期手术。并对Ｉ型、ＩＩ型的手术方法，肺动脉高压的治疗作了分析探讨。     

Report of a case of an atypical huge patent ductus arteriosus

An atypical huge patent ductus arteriosus (PDA) associated with extreme pulmonary hypertension in a 6-month old female infant was reported. The left 3rd intercostal thoracotomy revealed huge PDA which has an appearance of pulmonary-ductus-descending aorta-trunk (PDDT) on the left side. The right side of the ductus continued to the aortic arch without demarcation between the ductus and the aorta. The median sternotomy clarified the ascending aorta, normal aortic arch, and main pulmonary artery. However, the top of the main pulmonary artery seemed to be fused with the aortic arch. The diagnosis of distal type of aortopulmonary window with wide communication was made and the total circulatory arrest with deep hypothermia and the intracavitary patching for separation of systemic and pulmonary blood flow seemed to be required for the complete repair. Total repair was decided to be postponed one week later and the chest was closed. The patient died of multi-organ failure originating from acute respiratory failure 23 days after exploratory thoracotomy without being repaired. Postmortem examination revealed a huge PDA freely communicating with the ascending aorta and the aortic arch. This is a report of an atypical huge PDA rarely seen and the conventional division or ligation is unfeasible. The circulatory arrest with deep hypothermia and intracavitary patching are obligatory for the surgical repair.