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;     

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。     

19例三房心的病理解剖和外科治疗

为探讨三房心的病理解剖特点和外科治疗经验，总结１９８３年１２月至１９９５年７月，１９例三房心病人施行外科治疗的结果。年龄为１０个月～２４岁，术前确诊率为７３．７％，合并其他心内畸形１８例（９４．７％），手术死亡１例，手术死亡率为５．３％。作者认为，三房心是一种罕见的心脏畸形，多合并各种心内畸形，手术切除左心房内隔膜并同期矫治各种合并心内畸形是有效的治疗方法。     

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。     

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。     

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.     

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。     

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。     

One stage correction of coarctation of the aorta associated with intracardiac defects in infancy

A two stage surgical approach is usually prepared for symptomatic babies affected by coarctation of aorta associated with intracardiac defects, the preliminary operation being the resection of the coarctation. One stage correction has been attempted in six patients, ranging from 2 to 24 m. of age and from 3,2 to 9,7 Kg. of weight. The associated lesions were a large V.S.D. (3 patients), a V.S.D. with pulmonary stenosis, a mitral stenosis and a mitral insufficiency. The coarctation was approached through a left thoracotomy and the intracardiac defect through a midline sternotomy incision, after the patient was turned around. All patients survived and did well following the double procedure. The two-stage surgical approach is unnecessary and probably unadvisable for patients with coarctation of the aorta and associated intracardiac lesions.     

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.     

Coarctation of the aorta and severe aortic insufficiency: What to repair first?

A new surgical approach is proposed for patients with coarctation of the aorta associated with severe aortic valvular insufficiency. The valvular lesion should be repaired first and the coarctation corrected during a second operation; both interventions should be done during the same hospital stay. We base our approach on the belief that improved coronary perfusion can be achieved when the aortic insufficiency is corrected first. The disadvantages of the opposite surgical approach, such as anticoagulation problems, renal underperfusion, and hypertensive complications are easily avoided.     

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.     

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.     

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。     

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.     

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.