婴幼儿主动脉缩窄伴室间隔缺损的Ⅰ期纠治

目的总结婴幼儿主动脉缩窄伴室间隔缺损胸骨正中切口Ⅰ期纠治的手术方法和临床经验。方法2000年1月~2004年5月,采用胸骨正中切口Ⅰ期纠治17例主动脉缩窄伴室间隔缺损患儿,月龄1~41个月;体重3.0~13kg,平均为(6.32±2.35)kg。主动脉缩窄位于导管前11例,邻近动脉导管处6例;其中4例伴主动脉弓发育不良。9例伴有动脉导管未闭,17例均伴有室间隔缺损。结果本组17例中,1例于矫治术后第2d死于肺动脉高压危象;余16例术后恢复良好,其中14例上下肢动脉压差消失,另2例上下肢动脉压差24h内降至15mmHg(1mmHg=0.133kPa),72h后压差消失。随访至今超声心动图检查无假性动脉瘤或主动脉再狭窄。结论采用胸骨正中切口Ⅰ期纠治主动脉缩窄伴室间隔缺损,可同时纠治并发的主动脉弓部发育不良,降低了残余梗阻的发生率。Ⅰ期矫治还具有明显降低手术费用,减少患儿二次手术痛苦的优点。Ⅰ期手术在死亡率和并发症方面与Ⅱ期手术没有显著性差异。     

经正中切口Ⅰ期矫治小儿主动脉弓中断及合并畸形

目的探讨经胸骨正中切口Ⅰ期矫治小儿主动脉弓中断及合并畸形的手术方法和治疗效果。方法2002年7月-2005年10月手术治疗主动脉弓中断及合并畸形13例。男5例,女8例;年龄23 d-1．8岁,平均(4．5±2．4)个月,其中小于3个月8例(61．5％);体重3．0-8．0kg,平均(4．6±1．4)kg;A型8例,B型5例。13例患儿均在深低温停循环下经胸骨正中切口Ⅰ期矫正主动脉弓中断及合并畸形。结果死亡3例:死于肺动脉高压危象1例,心跳骤停1例,急性心包填塞1例;再次手术1例。10例随访2个月-3年,恢复良好。结论经胸骨正中切口Ⅰ期矫治主动脉弓中断及合并畸形效果良好;应在婴儿期尽早手术治疗。     

Ⅰ期矫治主动脉弓中断及合并畸形

目的 总结Ⅰ期矫治小儿主动脉弓中断(IAA)及合并畸形的诊治经验.方法 2005年1月至2007年5月诊治主动脉弓中断畸形10例.患儿术前均有反复呼吸道感染史,合并心脏畸形和重度肺动脉高压.其中A型8例,B型2例.采用正中切口Ⅰ期纠治主动脉弓中断和心脏畸形.结果 术后早期死亡1例,另9例随访4～19个月,恢复良好.结论 IAA一旦诊断明确,应尽早治疗,正中开胸Ⅰ期矫治术疗效满意.降主动脉与主动脉弓直接端侧吻合效果较佳.     

新生儿期根治主动脉弓中断伴合并畸形

目的介绍新生儿围术期主动脉弓中断伴合并畸形的治疗经验。方法全组13例,年龄3～28d,平均体重3．5kg,伴发畸形有主肺动脉窗、有心室双出口Taussig—Bing畸形、右肺动脉起源于升主动脉、室间隔缺损、房间隔缺损、左室流出道梗阻、动脉导管未闭等。均一期行主动脉弓中断及伴发畸形根治术。结果手术死亡1例。随访中轻度吻合口梗阻1例。结论主动脉弓中断是一种少见的先天性心脏病,新生儿期死亡率高,一经发现须及时采取一期解剖根治手术。     

小婴儿先天性主动脉弓中断合并心内畸形的Ⅰ期手术治疗

目的总结小婴儿主动脉弓中断合并心内畸形Ⅰ期手术治疗经验。方法2001年9月~2004年8月,手术治疗小婴儿主动脉弓中断合并心内畸形10例。男性7例,女性3例,手术年龄18d~7个月,平均年龄(3.37±2.45)个月,平均体重(5.33±1.72)kg。所有病例术前均有反复呼吸道感染史,病例分型A型7例,B型3例。均采用胸骨正中切口Ⅰ期手术纠治主动脉弓中断和心内畸形。结果全组病例均存活,随访3个月~3年,恢复良好。结论小婴儿主动脉弓中断合并心内畸形采用胸骨正中切口,操作简便,术野暴露清楚,创伤小,有利于术后恢复。由于该病早期发生肺动脉高压,宜尽早手术。     

右胸外侧小切口用于先天性单纯心内畸形矫治术

目的：探讨右胸外侧小切口在小儿先天性单纯心脏畸形心内直视手术中的应用价值。方法：采用该切口体外循环手术２５例,其中继发孔房间隔缺损１３例;室间隔缺损１２例。同期纠正合并畸形右下肺静脉异位引流３例,右流出道狭窄２例。结果：全组手术顺利无死亡。结论：认为这种切口具有损伤小,疤痕小和隐蔽,不引起胸骨鸡胸畸形,美观效果好。除复杂心脏畸形外,可安全有效地替代正中切口矫治某些单纯心脏畸形或可作为传统手术方式的一种补充。     

婴幼儿主动脉缩窄合并心内畸形的治疗策略

目的 总结婴幼儿主动脉缩窄(CoA)合并心内畸形的治疗经验.方法 2003年1月至2009年12月手术治疗21例CoA合并心内畸形患儿.15例患儿行左侧开胸矫治CoA,正中开胸修补心内畸形;6例正中开胸同时矫治CoA和心内畸形.结果 因低心排血量综合征和多器官功能衰竭死亡2例,病死率为9.5%.呼吸机应用7 h～13 d,ICU住院时间3～18 d.术后无神经系统并发症,超声检查无残余主动脉缩窄.结论 术前避免漏诊、尽早手术、选择正中切口一期根治、术后严防并发症是CoA矫治手术成功的关键.     

婴幼儿主动脉缩窄合并心内畸形的治疗策略

目的 总结婴幼儿主动脉缩窄(CoA)合并心内畸形的治疗经验.方法 2003年1月至2009年12月手术治疗21例CoA合并心内畸形患儿.15例患儿行左侧开胸矫治CoA,正中开胸修补心内畸形;6例正中开胸同时矫治CoA和心内畸形.结果 因低心排血量综合征和多器官功能衰竭死亡2例,病死率为9.5%.呼吸机应用7 h～13 d,ICU住院时间3～18 d.术后无神经系统并发症,超声检查无残余主动脉缩窄.结论 术前避免漏诊、尽早手术、选择正中切口一期根治、术后严防并发症是CoA矫治手术成功的关键.     

手术治疗新生儿重症先天性心脏病28例

目的 总结新生儿重症先天性心脏病的手术治疗以及围手术期处理经验,评价其临床疗效.方法 2008年1月至2010年3月广州市妇女儿童医疗中心手术治疗新生儿先天性心脏病28例,其中2例动脉导管未闭（PDA）患儿行左侧开胸结扎术;1例右室发育不良合并PDA、三尖瓣重度关闭不全患儿行中央分流术;8例室间隔缺损（VSD）患儿行体外循环下VSD修补术;3例主动脉缩窄、1例主动脉弓离断（IAA）患儿行体外循环下主动脉成型术;9例完全性大动脉转位（TGA）患儿中,8例做了Switch手术,其中合并VSD的2例同时做了VSD修补术;1例TGA/IVS PDA行肺动脉banding＋中央分流术,7 d后作了SWITCH手术;3例完全性肺静脉异位连接（TAPVC）纠治;重度肺动脉狭窄3例,肺动脉闭锁1例行右室流出道疏通,佰仁思带单瓣牛心包片重建右室流出道;重症法洛四联症、肺动脉发育不良先行中央分流术,3 d后低氧无法缓解,行右室流出道跨肺动脉瓣环补片扩大术.结果 早期（术后30 d内）无死亡病例,晚期死亡2例：1例为心上型TAPVC,术后因膈肌麻痹、乳糜胸、营养不良衰竭死亡,1例为IAA、VSD、PDA合并主动脉瓣下狭窄患儿,术后因持续严重低心排综合征死亡.26例术后痊愈出院.出院时1例VSD患儿术后膈肌折叠有气促,二氧化碳轻度潴留;2例SWITCH患儿术后肺动脉血流速度稍快;1例右室发育不良、PDA、三尖瓣关闭不全中央分流术后三尖瓣关闭不全中-重度;重度肺动脉狭窄、肺动脉闭锁、重度法洛氏四联症右室流出道疏通术后残余肺动脉轻-中度狭窄3例;VSD合并主动脉畸形术后主动脉仍有轻度狭窄.结论 新生儿重症先天性心脏病手术治疗效果满意.适当把握好手术适应症,选择正确的手术方法,精心细致的围手术期处理是手术成功的关键.术后低心排、肺功能障碍、肾功能衰竭的防治是降低手术后病死率的关键.     

先天性心脏病Taussig-Bing畸形的外科治疗

目的总结一期手术治疗Taussig-Bing畸形的经验。方法2000年5月～2006年12月上海儿童医学中心手术治疗Taussig-Bing畸形43例,其中合并主动脉弓病变12例;采用大动脉换位手术(ASO)矫治Taussig-Bing畸形42例,采用Kawashima手术1例,主动脉弓病变的矫治主要是将胸主动脉与主动脉弓下缘端端吻合或与升主动脉端侧吻合。结果住院死亡8例,总死亡率18.6%。合并主动脉弓病变者并发大动脉侧侧位和冠状动脉畸形比较常见,手术死亡率(25.0%,3/12例)比单纯Taussig-Bing畸形(16.1%,5/31例)者高,但差异无统计学意义。结论Taussig-Bing畸形病理解剖比较复杂,须根据解剖条件的不同选择不同的手术方法才能降低手术死亡率。     

新生儿主动脉缩窄合并室间隔缺损27例一期手术矫治效果分析

目的探讨新生儿主动脉缩窄合并室间隔缺损一期手术矫治的效果。方法回顾性分析2015年1月至2018年12月湖南省儿童医院收治的主动脉缩窄合并室间隔缺损新生儿的临床资料,排除合并其它复杂心脏畸形的病例;所有患儿一期矫治主动脉缩窄,同期行室间隔缺损修补术,采用围手术期精细化管理,共27例患儿纳入本研究,日龄范围4~28 d,平均(20.1±6.9)d;体重2.7~4.5 kg,平均(3.6±0.5)kg。结果27例中,术后延迟关胸11例,出血6例,急性肾功能衰竭2例,乳糜胸1例,气胸2例,胸腔积液1例,心律失常2例,血压升高1例,再插管3例。严重低心排出量综合征并早期死亡1例,其余26例均经过精细化管理治愈出院。随访3~48个月,吻合口狭窄再手术1例,其余患儿复查结果均满意。结论新生儿主动脉缩窄合并室间隔缺损一期矫治手术效果良好,若术前乳酸已出现进行性升高会影响术后结局,围手术期精细化管理对提高手术成功率非常重要。     

小儿主动脉缩窄的外科治疗

目的 回顾和总结小儿主动脉缩窄的外科治疗经验。方法 66例患儿，年龄20d～8．5岁。21例单纯CoA，45例同时合并心内畸形。单纯CoA从左后外侧第4肋间进胸。若合并有心内畸形，早期9例患儿先左后外侧肋间进胸纠治CoA，再正面开胸体外循环下纠治心内畸形，2000年1月之后，35例采取正中劈开胸骨，体外循环下Ⅰ期纠治CoA和心内畸形。另有1例分期手术。纠治CoA方法主要有：缩窄段切除行端端吻合或端侧吻合术，补片扩大成形术和左锁骨下动脉瓣翻转成形术。结果 患儿术后早期下肢动脉血压超过上肢47例，与上肢相同13例，仍低于上肢6例，无一例发生肾功能衰竭和截瘫。死亡2例(1例死于严重心力衰竭，另1例死于心律失常)。随访39例，下肢动脉血压超过上肢34例，相同4例，1例伴有主动脉弓发育不良术后6个月仍存在缩窄(上肢血压高于下肢35mmHg)。3例有声音嘶哑，远期随访无高血压出现。结论 单纯主动脉缩窄或合并心内畸形宜早期手术治疗，Ⅰ期根治术是安全和有效的。     

Aortic arch interruption presenting with absence of all limb pulses.

Three patients with aortic arch anomalies presented with severe heart failure, acidaemia, and poor or absent upper and lower limb pulses. Prominent carotid arterial pulsations were detected which distinguished the condition clinically from aortic atresia. In 2 patients with a left aortic arch, interruption was distal to the left common carotid artery and there was an aberrant right subclavian. In the 3rd patient with a right aortic arch, interruption was distal to the right common carotid and there was an aberrant left subclavian artery. By dilating the narrowed ductus, prostaglandin E2 infusion corrected the acidosis. Successful surgical correction was achieved in one patient by direct anastomosis of descending aorta to ascending aorta and the base of the left common carotid artery.     

Total one-stage repair of aortopulmonary window and interrupted aortic arch in a neonate

The congenital anomaly of aortopulmonary window is rare. Its combination with interruption of the aortic arch has been previously recognized and reported in infants and children. Early and complete repair of such a congenital anomaly may prevent progression to cardiac failure and premature death. We describe the successful echocardiographic diagnosis and one-stage surgical repair, via a median sternotomy, of the aortopulmonary window, interrupted aortic arch, patent ductus arteriosus, and patent foramen ovale in a 3-day-old neonate. The literature is also reviewed.     

Aortic valvular atresia

Summary The pathological anatomy of 109 specimens of aortic valvular atresia was reviewed for the purpose of identifying the cardiovascular anomalies associated with that condition. We found the most commonly associated anomaly to be coarctation of the aorta, which was present in 71 percent of our cases and judged to be of hemodynamic significance in one-third of the involved cases. Other associated anomalies, in order of decreasing frequency, were mitral atresia, anomalous systemic and pulmonary venous connections, abnormalities of branching of the aortic arch, and ventricular septal defect. The study demonstrated that aortic atresia is associated with a significant incidence of other cardiovascular anomalies. Additional anomalies, when present, may complicate emerging attempts at surgical correction of this condition. Supported by Public Health Service Research Grant 5 RO1 HL05694 from the National Heart, Lung and Blood Institute.     

主肺动脉窗25例临床分析

目的 分析主肺动脉窗（APW）的发病、分型和伴发畸形情况,总结诊断及治疗经验。方法 回顾性分析复旦大学附属儿科医院2003至2013年诊治的25例APW患儿的临床症状、病理分型、合并畸形、诊断、治疗和预后资料。结果 25例APW患儿进入分析,男14例,女11例,年龄5 d至13岁（6月龄以下12例）,占同期住院先天性心脏病患儿的0.18%。Ⅰ型5例（20%）,Ⅱ型16例（60%）,Ⅲ型1例（4%）,Ⅴ型3例（12%）。单纯APW 10例,APW合并单纯卵圆孔未闭3例;余12例（48.0%）均合并其他需纠正的心血管畸形,其中合并单纯房间隔缺损（ASD）1例,单纯动脉导管未闭（PDA）2例,单纯室间隔缺损(VSD)3例,右室双出口和ASD 1例,主动脉瓣狭窄和右位主动脉弓1例,肺动脉瓣闭锁伴VSD、右位主动脉弓、永存左上腔静脉1例, 主动脉弓离断及PDA 3例。超声心动图诊断17例,误诊或漏诊8例（32%）中5例通过心导管检查及造影诊断,3例外科术中诊断。25例均有不同程度肺动脉高压,其中2例心导管检查评估为阻力性肺动脉高压,4例家长放弃诊治,其余19例施行一期根治术,术后死亡2例（10.5%）。17例术后随访3个月至9年3个月,平均（23±27）个月,其中15例临床无症状,复查超声心动图APW修补无残余,肺动脉压力逐渐下降至正常。结论 APW缺损主要发生在主动脉远端,常伴有间隔缺损、PDA和主动脉弓病变等;超声心动图为首选检查方法,但必要时需行心导管检查及造影。APW患儿易早期发生肺动脉高压,一经诊断尽早手术,可获得较好的中长期疗效。     

Surgical approaches for interrupted aortic arch in infants北大核心CSCD

Objective To summarize the surgical experiences and follow-up results of different surgical approaches for interrupted aortic arch (IAA) and to optimize surgical planning. Methods From October 2012 to September 2020, 53 IAA children aged under 1 year were selected as study subjects.The concurrent conditions included ventricular septal defect (VSD, n=41), subaortic membrane and severe stenosis, parachute mitral valve & intramitral annulus (n=1), main aorta-pulmonary window (APW, n=6), common arterial trunk (n=1), complete atrial ventricular septal defect (n=1), double outlet of right ventricle & abnormal origin of coronary artery (n=1) and bicuspid aortic valve (n=1). Primary anatomical correction (n=52) and staged surgery (n=1) were performed.The procedures included end-to-end anastomosis of descending aorta & proximal aortic arch (n=8), extended end-to-side anastomosis of descending aorta & proximal aortic arch (n=28), extended end-to-side anastomosis of descending aorta and proximal aortic arch + bovine pericardial patch widened anterior wall of aortic arch (n=12), left subclavian artery inversion and direct anastomosis of descending aorta (n=2) and extended end-to-side anastomosis of descending aorta and proximal aortic arch and widening of anterior aortic wall with pulmonary vascular patch (n=3). The parameters of delayed sternal closure time, postoperative mortality and restenoticc rate were statistically analyzed with SPSS 22.0.Results The outcomes were survival (n=48), death (n=5) and delayed thoracic closure (n=26). Two postoperative children underwent extracorporeal membrane oxygenation (ECMO) assisted circulation due to low cardiac output syndrome.One case of postoperative cardiac tamponade underwent emergency thoracotomy for hemostasis; Staphylococcus aureus was detected in blood culture of 1 child and cure obtained after antibiotic dosing of imipenem and vancomycin.In another case, chylothorax disappeared after a combination of fasting and with parenteral nutrition.One child developed postoperative convulsions and cranial MRI hinted at hypoxic-ischemic encephalopathy.After intensive nutrition and rehabilitation, he was discharged after improvements.One child underwent percutaneous balloon dilation of aortic arch with a pressure difference of 86 mmHg(1 mmHg=0.133 kPa) at 1 year postoperatively.And others were followed up with a pressure difference of <30 mmHg for 1 year.Conclusions Upon a definite diagnosis of IAA, early surgical intervention is mandatory.Proper surgical approaches should be based upon the location, extent and associated intracardiac abnormalities.Surgical intervention is indicated for such chronic conditions as subaortic membrane formation and left ventricular outflow obstruction. © 2023 Chinese Medical Journals Publishing House Co.Ltd. All Rights Reserved.     

Persistent Fifth Aortic Arch and Fourth Arch Interruption in a 28-Year-Old Woman

A 28-year-old woman having persistent fifth aortic arch and aortic coarctation associated with the fourth arch interruption, without other cardiac malformation, underwent graft interposition, 20 mm in diameter, between the fourth arch including the left subclavian artery and the descending aorta.