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.     

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.     

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;     

主动脉缩窄合并心内畸形的一期手术治疗

１９９３年６月至１９９４年８月为５例主动脉缩窄合并先天性心内畸形病儿施行了一期手术治疗。男４例，女１例，年龄１．５～１３岁，体重７．５～４５ｋｇ。除主动脉缩窄外，合并的心内畸形有室间隔缺损、主动脉瓣及瓣下狭窄、主动脉瓣关闭不全等。手术采用左后外侧第４肋间切口，矫治主动脉缩窄后，同期行胸骨正中切口体外循环下心内畸形矫治。本组无手术死亡和并发症，取得了满意的治疗效果。作者认为，对主动脉缩窄合并心内畸形施行一期手术矫治是完全可行的。     

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。     

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。     

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。     

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。     

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.     

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.     

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.     

Repair of hypoplastic or interrupted aortic arch via sternotomy.

Herein we describe our experience with repair of interrupted aortic arch and coarctation plus hypoplastic aortic arch in 55 consecutive infants (1984 to 1990). Median age at operation was 6 days and median weight 3.1 kg. Associated severe intracardiac anomalies were the rule. All patients had significant congestive cardiac failure, and the majority required prostaglandin E1 resuscitation and inotropic support (with or without ventilation) before the operation. All operations were performed via sternotomy with core cooling and circulatory arrest. Isolated myocardial perfusion was used in 13 patients during arch repair. A complete intracardiac (biventricular) repair was performed except in patients expected to require a Fontan operation as definitive treatment. The operative mortality overall was 14.5% (confidence limits 10% to 22%). For arch repair plus biventricular intracardiac repair, the operative mortality was 9% (confidence limits 5% to 15%), and for arch repair plus palliative intracardiac repair, 40% (confidence limits 22% to 60%). The mortality in the isolated myocardial perfusion group was 0% (confidence limits 0% to 14%), which may be related to reduced myocardial ischemic time (p less than 0.05). Actuarial survival was 75% (confidence limits 65% to 83%) at 12 months, with no subsequent deaths over 1294 patient-months (mean 28 months) of follow-up. Actuarial freedom from recurrent arch obstruction was 69% (confidence limits 48% to 85%) at 46 months' follow-up. Primary repair of interrupted aortic arch and coarctation plus hypoplastic arch compares favorably with a staged approach and is recommended even when complex intracardiac anatomy is present.     

Aortic arch reconstruction using regional perfusion without circulatory arrest.

OBJECTIVES: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. Regional cerebral and myocardial perfusion may eliminate those potential side effects. METHODS: From March 2000 to March 2002, 48 neonates or infants with complex arch anomaly were operated on using the regional perfusion technique. Thirty-three patients were male and the median age was 24 days (range 5-301 days). Preoperative diagnosis consisted of coarctation or interruption of the aorta associated with ventricular septal defect (group I, n = 26) and arch anomaly with complex intracardiac defects such as hypoplastic left heart syndrome or its variants (group II, n = 22). Arterial cannula was inserted through the innominate artery and the flow rate was regulated to about 50-100 ml/kg per min during regional perfusion. Simultaneous myocardial perfusion was maintained using a Y-connected infusion line. Cardioplegia was applied during intracardiac repair. RESULTS: Cardiopulmonary bypass and aortic cross-clamp times were 154 +/- 49 and 39 +/- 34 min, respectively. Temporary circulatory arrest for intracardiac procedures was performed in eight patients. However, the mean arrest time was minimized (range 1-18 min). The descending aorta clamping time was 33 +/- 16 min. Operative mortality rates in each group were 0 and 18.2% (0/26 and 4/22). Late mortality rates were 0 and 11.1% (0/26 and 2/18) during 9.1 months of follow-up. Complications consisted of low cardiac output in eight cases, transient neurological problems in two cases, and transient renal insufficiency in two cases, respectively. CONCLUSIONS: Regional perfusion is feasible and can be used with acceptable results. It may reduce potential complications following aortic arch reconstruction using circulatory arrest. However, repair of aortic arch in the patients with complex intracardiac defects still imposes a significant rate of mortality and morbidity.     

Repair of Coarctation of the Aorta in Neonates and Young Infants

Abstract In repair of coarctation in neonates or young infants, inadequate removal of ductal tissue, failure to address hypoplasia of the aortic arch, and suture line tension have been reported to be important factors of residual or early recurrent stenosis at the coarctation repair site. In a consecutive series of neonates and young infants with coarctation, who were all operated without delay with extended resection, the clinical outcome regarding the development of restenosis and hypertension was studied. In addition, the resected specimens were investigated regarding the completeness of resection of ductal tissue. Twenty-five consecutive neonates and young infants (median age 22 days, range 5 to 39 days) who underwent surgical correction of coarctation were reviewed; the resected specimens were examined histologically to document the extent of ductal tissue in the aortic wall. Fifteen patients had a preductal coarctation with associated cardiovascular anomalies including a hypoplastic aortic arch (n = 11). The remaining 10 patients had a paraductal coarctation without associated intracardiac anomalies. In all patients, the isthmus was bypassed and an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch (n = 13) or the distal ascending aorta (n = 12). In 13 patients without marked hypoplasia of tbe aortic arch, the coarctation repair was performed through a left thoracotomy. In the remaining 12 patients, the coarctation was repaired through a median sternotomy with CPB and hypothermic circulatory arrest, on the basis of an associated hypoplastic aortic arch (n = 4), hypoplastic aortic arch with intracardiac anomalies (n = 7), or a “bovine” innominate artery (n = 1). There was no perioperative or late mortality. At a median follow-up of 15 months, 1 patient (4%) developed a recurrent stenosis at the coarctation repair site; in the remaining 24 patients, echocardiograpby showed a widely patent anastomosis with no evidence of a hemodynamically significant gradient. None of the patients had hypertension. Histologic examination of the resected specimens demonstrated the presence of ductal tissue in the descending aorta with maximal extension into its lateral wall (mean 5.2 mm). In all specimens of the paraductal subtype, there was also extension of ductal tissue into the lateral wall of tbe isthmus (mean 3.9 mm). We conclude that: (1) in the absence of marked hypoplasia of the proximal aortic arch, coarctation can be repaired with low mortality and morbidity via a left thoracotomy; (2) in the presence of marked hypoplasia of the proximal aortic arch and/or if associated intracardiac defects also need to be repaired, we advocate repair of the coarctation and associated defects through a median sternotomy with circulatory arrest; (3) in view of the absence of postoperative hypertension in this series, early repair of aortic coarctation is recommended; and (4) because ductal tissue may extend not only into the descending aorta but also into the isthmus, complete excision of the coarctation and bypass of the isthmus are valuable techniques to avoid secondary constriction of the aorta by ductal tissue.     

Operation of mid-arch coarctation.

BACKGROUND: Coarctation occurring within the aortic arch is rare and may present difficulties during surgical repair. We describe the operative technique and outcome in 6 patients with this unusual anomaly. METHODS: Five patients had antegrade perfusion with circulatory arrest. Three patients with presubclavian narrowing (one presenting with type B dissection) were operated through extended left thoracotomy. Two precarotid and paracarotid lesions were approached through a median sternotomy. All patients were perfused antegradely from the ascending aorta and operated with hypothermic circulatory arrest. One patient who had a complex presubclavian coarctation after two previous repairs received an ascending aorta to abdominal aorta bypass graft without cardiopulmonary bypass. RESULTS: All patients survived operation and are well at a mean follow-up of 3.3 years after the procedure. None had cerebral problems or spinal cord injury. Renal function was unchanged. The mean (+/- standard error of the mean) resting gradient across the coarctation decreased from 42+/-4.0 mm Hg to 6+/-1.2 mm Hg (p = 0.0004). CONCLUSIONS: Hypothermic circulatory arrest using antegrade ascending aortic perfusion allows safe and effective repair of mid-arch coarctation. Complicated reoperations can be managed safely using ascending-to-abdominal aortic bypass.     

Surgical treatment for aortic arch aneurysm: newly developed procedures and their outcomes

The surgical treatment of aortic arch aneurysm including newly developed procedures and their outcomes is reviewed. Major advances in aortic arch repair have been made by meticulous brain protection with antegrade-selective and retrograde cerebral perfusion in addition to hypothermia circulatory arrest and refinement of surgical techniques. Total arch replacement using a multibranched prosthetic graft with antegrade-selective cerebral perfusion (SCP) under hypothermia through a median sternotomy has been standardized, resulting in lower mortality and cerebral mortality rates. In particular, the impact of the use of the axillary artery for cardiopulmonary bypass and of the stepwise or elephant trunk technique for distal anastomosis has recently been assessed. In addition, arch repair under moderate hypothermia in conjunction with SCP has been attempted without any serious problems. The surgical strategy for extended aortic aneurysms is also of concern. A two-stage approach with an elephant trunk procedure is employed predominantly for high-risk patients, while one-stage repair is aggressively applied for relatively young, low-risk patients. In contrast, there has been great progress in stent graft therapy for aortic arch lesions. Arch stent graft repairs including hybrid procedures have been attempted in elderly, high-risk patients. Consequently, these comorbid procedures can be used satisfactorily.     

Transpericardial extra-anatomic intrathoracic aortic bypass conduits in the management of coarctation of the aorta

Two varied cases of coarctation of the aorta are described, in which transpericardial ascending to descending thoracic aortic conduits were routed around the right atrium, utilizing deep hypothermic circulatory arrest. The approach was through a median sternotomy in two patients. The first case is a 52-year-old female who underwent combined aortic valve replacement for aortic stenosis and repair of aortic coarctation distal to the left subclavian artery. The second case was a fifth time reoperation in a 12-year-old girl with recurrent coarctating. Both patients remain well and asymptomatic.