完全性房室隔缺损的外科治疗

目的 总结完全性房室隔缺损的手术方法和临床经验，以提高手术疗效。方法 27例完全性房室隔缺损患者，年龄88天～24岁。合并右心室双出口、肺动脉瓣狭窄和镜像右位心1例，动脉导管未闭1例，继发孔型房间隔缺损1例，左上腔静脉残留2例，重度肺动脉高压13例，中度肺动脉高压14例。手术均采用双补片法修补，室间隔缺损采用Gore—Tex补片修补，房间隔缺损采用自体心包补片修补。结果 术后早期死亡2例，其中1例死于脑出血，1例死于右侧心力衰竭。所有患者术后均为窦性心律，无完全性房室传导阻滞。随访23例，随访时间2个月～10年，均为窦性心律，无死亡。复查心脏彩色超声心动图提示：二尖瓣轻度反流6例，中度反流2例，心功能均较术前增强。结论 完全性房室隔缺损患者早期行手术治疗，防止肺血管阻塞性病变的发生、发展，术中注意防止左心室流出道狭窄，矫治二尖瓣反流是提高手术疗效的关键。     

儿童室间隔缺损合并二尖瓣关闭不全的外科治疗及中期随访

目的 总结同期行手术治疗儿童室间隔缺损合并二尖瓣关闭不全的临床经验.方法 回顾性分析2006年1月至2012年1月同期手术治疗84例室间隔缺损(VSD)合并二尖瓣关闭不全患儿,男33例,女51例；年龄2个月～6岁,平均(15.6±6.9)个月；体质量3～ 20 kg,平均(10.6±3.2) kg.患儿术前肺动脉压32～85 mm Hg,中重度肺动脉高压45例,术前多有反复肺部感染.VSD直径0.7～1.6cm;左向右分流45例,双向分流39例.二尖瓣反流轻度(+～++)27例,“+”9例,“++”18例；中度(+++)33例；重度(++++)24例.均在全麻中低温体外循环下应用人工dacron补片修补VSD,并同期行二尖瓣成形术,术中通过食管超声(TEE)评价VSD修补及二尖瓣成形的效果.结果 术中TEE提示,患儿均无残余分流,二尖瓣成形效果满意,无反流80例,反流程度“+”4例.术中体外循环(84.6±18.5) min,主动脉阻断(50.8 ±11.5) min.术后呼吸机辅助(38.7 ±30.2)h,术后住院(10.5±4.6)天.术后早期死亡1例(1.2％).随访2 ～ 72个月,完整随访78例,随访期间无死亡.复查超声心动图,VSD均无残余分流,二尖瓣无反流62例,反流程度“+”10例,“++”4例,“+++”2例.术后5年免除再手术率(97.4±1.8)％.结论 室间隔缺损合并肺动脉高压患儿应尽早行手术根治,同期矫治合并的二尖瓣关闭不全,患儿预后良好.正确的围术期处理是降低患儿病死率的重要因素.     

二尖瓣成形术治疗132例中、重度二尖瓣关闭不全患儿的中期疗效

目的 探讨儿童中重度二尖瓣关闭不全成形术的手术方法及治疗效果.方法 回顾性分析132例中重度二尖瓣关闭不全患儿资料,年龄2个月～6岁,平均(18.9±7.2)个月;体质量4～21kg,平均(11.3±4.8)kg.先天性心脏病126例,感染性心内膜炎5例,马方综合征1例.全组患儿均在全麻中低温体外循环下,采用瓣环环缩术、人工瓣环成形术、瓣叶裂缺修补术、后瓣矩形或三角形切除成形术、腱索折叠等个体化的二尖瓣综合成形技术,同期矫治合并的心脏畸形,术中经食管超声(TEE)检查评价成形效果.结果 全组患儿术中TEE示131例无反流或轻度反流;1例中度反流再次行体外循环下二尖瓣成形.术中平均体外循环(80.0±31.1) min,平均主动脉阻断(48.0±17.9) min.早期死亡3例,病死率2.3％,其中2例为完全型房室间隔缺损患儿,分别于术后第7天死于心力衰竭,术后第2天死于低心排血量综合征;1例为大型室间隔缺损合并重度肺动脉高压患儿,术后1个月死于肺部感染.129例成功治愈出院,术后呼吸机辅助(34.4±31.9)h,术后住院(9.0±5.4)天.完整随访122例,时间2～74个月,平均(40.5±8.3)个月.随访期间无死亡.复查超声心动图提示中度反流7例,重度反流3例,4例患儿再次行二尖瓣成形或二尖瓣置换术.本组患儿5年生存率97.7％,免除再手术率92.0％.结论 儿童中重度二尖瓣关闭不全应早期行手术治疗,合并其他心脏畸形需同期矫治,手术治疗的早、中期效果满意.术中根据二尖瓣的具体病变情况,采取个体化的综合成形方法是成功治疗儿童中重度二尖瓣关闭不全的关键.     

完全性房室隔缺损的外科治疗

目的回顾性总结手术纠治４５例完全性房室隔缺损（ｃｏｍｐｌｅｔｅａｔｒｉｏｖｅｎｔｒｉｃｕｌａｒｄｅｆｅｃｔ，ＣＡＶＤ），以期把握好手术时机，提高手术成功率和生存质量。方法根据Ｒａｓｔｅｌｉ分型：Ａ型２６例，Ｂ型２例，Ｃ型２例，过渡型１５例。手术采用单片心包补片修补方法。结果手术死亡３例，死亡率６．７％。死亡原因为伴发心内严重畸形法洛四联症２例，重度肺动脉高压１例；远期死亡２例，均为二尖瓣反流并发肺部感染和心力衰竭。结论ＣＡＶＤ早期出现肺动脉高压，手术应在６个月～１岁内为好，术后必须定期随访，早期了解房室瓣反流情况     

完全型房室间隔缺损的外科治疗

目的　总结完全型房室间隔缺损外科治疗的经验。方法　16例完全性房室间隔缺损患儿,平均年龄(1.2±0 .9)岁,平均体重(6.8±3 )kg。其中10例伴有Down综合征,1例合并法洛四联征。术前超声心动图显示房室瓣轻度反流12例,中度反流3例,重度反流1例。行单片法修补10例,双片法修补6例。结果　术后恢复顺利,无围手术期死亡。除1例患儿于出院4个月后因肺部感染合并心衰死亡外,余随访0 .3～5 .2年,经超声心动图检查显示房室瓣功能良好,未见明显反流。结论　完全型房室间隔缺损患者早诊断,早手术,可获良好疗效。     

右腋下小切口二尖瓣成形术68例

目的探讨经右腋下小切口行二尖瓣成形术的临床效果。方法回顾性分析2003年1月～2011年12月经右腋下5～10 cm小切口行二尖瓣成形术68例的资料。二尖瓣成形方法包括瓣叶裂隙修补6例,瓣叶部分切除28例,交界成形12例,双孔成形10例,人工腱索6例,腱索缩短3例,瓣叶心包补片修补3例;68例中人工瓣环植入38例。结果 68例手术均顺利完成,无严重并发症发生,无手术死亡。术后1周复查超声心动图,二尖瓣无明显反流或仅微量反流52例,轻度反流16例。随访65例,随访时间3个月～8年,其中〉3年32例,2例复发二尖瓣重度关闭不全,经胸骨正中切口行二尖瓣置换手术。其余患者二尖瓣反流均在中度以下。结论右腋下小切口二尖瓣成形术创伤小,出血少,临床效果良好,并且切口位置隐蔽,美容效果好。     

部分性房室管畸形外科治疗及疗效探讨

目的 探讨外科治疗部分性房室管畸形的手术方法及其疗效。方法 ４８例部分必室管畸形患者均在体外循环心内直视术下缝合二尖瓣大瓣裂缺及修补房间隔缺损,其中９例同时行瓣膜成形手术。结果 术后早期（３０天内）死亡２例,死亡率４．１７％,发生Ⅲ度房室传导阻滞２财次手术４例,术后３９例随访３个月～１２年,平均随访９年。结论经随访,手术后 瓣无反流或少量反流者长期疗效良好,中等量以上反流者长期疗效差。     

66例部分性房室管畸形的外科治疗

目的总结部分性房室管畸形手术治疗的经验,以提高治疗效果。方法回顾性分析我院1984年1月－2007年12月经外科治疗部分性房室管畸形患者66例的临床资料,对二尖瓣大瓣裂的处理：单纯裂缺缝合52例,缝合加交界折叠缝缩8例,加小瓣成形1例,加缝置St．Jude软质人工成形环3例,人工机械瓣置换术2例;对原发孔型房间隔缺损的修补：采用涤纶补片修补12例,自体心包54例;采用Kirklin法将冠状静脉窦口隔入左心房5例,MeGoon法将冠状静脉窦口隔入右心房61例;同期处理其他合并畸形。结果术后早期死亡2例（3．03％）,1例死于心律失常,另1例死于呼吸功能衰竭。术后发生Ⅲ°房室传导阻滞2例,均安装永久性心脏起搏器。术后随访52例（81．3％）,随访时间5个月～22年,平均15年,心功能均有明显改善,尤其是术前心功能Ⅲ～Ⅳ级的19例患者,术后改善为Ⅰ～Ⅱ级。再次手术4例,其中1例经再次行二尖瓣置换术治愈;术后死亡3例,1例死于急性。肾功能衰竭,2例死于低心排血量综合征。结论早期手术治疗可以保全房室瓣结构、功能,避免发生肺动脉高压和降低死亡率。手术治疗的关键是消除二尖瓣关闭不全、修补原发孔型房间隔缺损和避免损伤传导组织,术后近、中期疗效良好;有残留中度以上二尖瓣反流者,远期效果不满意。     

房室间隔缺损矫治术后左侧房室瓣反流的外科治疗

目的为提高房室间隔缺损修补术后左侧房室瓣反流的外科治疗效果,探讨其瓣膜的病理改变、手术方法的选择,总结围手术期处理经验。方法回顾分析上海交通大学医学院附属新华医院1995年1月至2009年12月收治29例房室间隔缺损矫治术后左侧房室瓣反流患者的临床资料,其中男16例,女13例;年龄4~62岁,平均年龄26.5岁;术前心功能分级（NYHA）Ⅱ级10例,Ⅲ级17例,Ⅳ级2例;曾行部分性房室间隔缺损矫治术18例,完全性房室间隔缺损矫治术11例;再次手术行心瓣膜修复术17例,心瓣膜置换术12例。结果手术后早期因多器官功能衰竭死亡1例;1例患者行心瓣膜置换术后发生永久性Ⅲ°房室传导阻滞而安装永久心脏起搏器,1例4岁患者行心瓣膜置换术后不能撤离体外循环而行左心辅助56 h后痊愈出院。术后随访25例,失访3例,均为行瓣膜修复患者;术后随访6个月~14年,平均8.2年;随访14例心瓣膜修复患者,左侧房室瓣前向血流均无明显加快,10例左侧房室瓣轻微或轻度反流,1例中度反流,3例分别于再次手术后10 d、3年和6年再发左侧房室瓣重度反流,最终行心瓣膜置换术;25例患者术后心功能明显改善,Ⅰ级17例、Ⅱ级6例、Ⅲ级2例;胸部X线片示：心影较术前明显缩小,心胸比率0.53~0.67（0.60±0.11）;未发现远期死亡。结论对房室间隔缺损矫治术后再发生的左侧房室瓣反流,进行及时的手术治疗,选择恰当的手术方式,能取得满意的近期和远期疗效。     

完全性房室间隔缺损的外科治疗

目的回顾性总结手术治疗完全性房室间隔缺损的经验。方法112例病儿,≤6个月43例(38%,X组),>6个月69例(62%,Y组)。85例行心导管检查。Rastelli A型89例,Rastelli B型10例,Ras- telli C型13例。手术技术分单片法,双片法和简化单片法。术中经食管超声检查发现异常而即刻再次手术者7例(二尖瓣反流4例,二尖瓣狭窄2例,左室流出道梗阻1例)。术后入重症监护室,左房压8～21 mm Hg,中心静脉压7～12mm Hg。呼吸机平均应用47h,监护室平均滞留6.3d。结果室间隔缺损残余分流(直径>2mm)13例,二尖瓣中度反流12例,完全性房室传导阻滞4例。院内死亡6例(X组1例,Y组5例)。术后随访91例(81%),随访1～5年,平均2.3年。1例术后1年因肺炎心衰死亡,1例术后2年因二尖瓣中-重度反流而换瓣。结论院内死亡率提示,小于6月龄完全性房室间隔缺损病婴手术是安全的。随着年龄增大,瓣膜成形效果、肺动脉高压的预后可能会更差。双片法修补室间隔缺损较易发生残余漏(9例,18%),简化单片法出院时二尖瓣关闭不全发生率明显高于另外两种方法(6例,16%)。     

法洛四联症合并完全房室间隔缺损手术方法探讨

目的探讨法洛四联症合并完全房室间隔缺损(TOFAVSD)心内矫治的手术方法和效果。方法1985年1月至2004年10月,TOFAVSD心内矫治术16例中男7例,女9例;年龄2～16岁,平均(8.1±3.8)岁。采用右房、右心室纵切口,前7例采用3块补片;后9例用2块补片修复房室间隔缺损,左侧房室瓣裂隙采用间断缝合,右心室流出道用跨瓣补片加宽。结果术后早期死亡4例,前7例死亡3例;后9例死亡1例。死因为严重低心排血量综合征3例,灌注肺1例。8例随访2个月至13.5年。心功能I或II级,无临床症状。结论采用右房、右心室纵切口,2块补片修复房室间隔缺损,常规间断缝合左侧房室瓣裂隙,跨瓣补片加宽右室流出道可取得较良好的疗效。     

Two-patch repair of complete atrioventricular septal defect in the first year of life. Results and sequential assessment of atrioventricular valve function

Before January 1987, 62 infants underwent two-patch repair of complete (51) or intermediate (11) atrioventricular septal defect at the Royal Children's Hospital, Melbourne. Median age at repair was 4.3 months and median weight was 4.4 kg. Early deaths (3%) were confined to two infants with preoperative respiratory tract infections; a further two patients died during follow-up (late mortality rate 3%). Reoperation for severe postoperative mitral regurgitation was necessary in 10 infants (16%), two of whom subsequently required mitral valve replacement with a prosthesis. Preoperative atrioventricular valve regurgitation was assessed retrospectively in 49 patients from angiography or Doppler echocardiography and was found to be absent or mild in 33 (68%), moderate in 9 (18%), and severe in 7 (14%). At the time of latest review (at a mean of 2.4 years after repair), judged from a combination of clinical and echocardiographic criteria, mitral regurgitation was absent or mild in 49 (84%) of the 58 survivors; none of them had symptomatic regurgitation or were requiring continuing medical treatment. Analysis of sequential atrioventricular valve function in 46 of the 49 patients in whom objective preoperative data were available showed no relationship between the degree of preoperative and postoperative atrioventricular valve regurgitation. Infants without Down's syndrome, however, had a significantly higher reoperation rate for severe postoperative mitral valve regurgitation (50%) than those with Down's syndrome (10%) (p = 0.007). Complete atrioventricular septal defect can be repaired in early infancy with a low mortality rate and good intermediate term results.     

Complete atrioventricular septal defect repair: Simplified single patch technique

Background: There has been a rekindling of interest in alternatives to conventional two patch technique for the repair of complete atrioventricular septal defect in infancy in the recent past. We applied the simplified single patch technique to 15 consecutive infants and herein report our intermediate term results. Methods: Between March 1998 and September 2001, fifteen patients underwent repair of complete atrioventricular septal defect with this technique (mean age 6 months, mean weight 5.4 kg). Downs syndrome was present in 11 patients. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum irrespective of the size of the ventricular septal component. The cleft in the anterior mitral leaflet was closed in all patients. The atrial septal component was closed by a pericardial patch. Results: There was no mortality. There were no pulmonary arterial hypertensive crises or heart block. The mean follow up was 13.2 months. One patient underwent mitral valve replacement after one year due to severe mitral regurgitation. The remaining fourteen patients had no significant mitral regurgitation, residual ventricular septal defect or left ventricular outflow tract obstruction on echocardiography. Conclusion: Simplified single patch technique is an easily reproducible method for surgical repair of complete atrioventricular septal defect. It is less time consuming and minimises ischaemic time. Atrioventricular valve function is preserved and there is no incidence of obstruction to left ventricular outflow tract. The intermediate term results are encouraging. Presented in the poster session of the 37^th Annual Meeting of Association for European Paediatric Cardiology (AEPC) at Porto, Portugal, May 2002     

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.     

Unusual mitral valve abnormalities complicating surgical repair of endocardial cushion defects

A review of 155 cases of surgically repaired endocardial cushion defects revealed 16 patients (10%) with additional unusual mitral valve abnormalities that complicated the surgical procedure. Eight patients had accessory mitral valve tissue that connected the anterior and posterior leaflets to form a double-orifice valve (Group I). In four (50%), the lesion was associated with intermediate atrioventricular canal and small left ventricle; all four died following repair. In the other four, it was associated with ostium primum defect; all survived and are well. A single papillary muscle in the left ventricle was present in six patients (Group II). Two had intermediate atrioventricular canal and both died postoperatively. The other four had complete endocardial cushion defect and three are well following the operation. Perforation of the valve leaflets was present in two patients with ostium primum (Group III). Both patients are well postoperatively. Modification of the surgical technique is required to effect satisfactory repair. The bridge connecting the posterior and anterior leaflets of the mitral valve should be left undisturbed. Otherwise, severe regurgitation may result. In patients with single papillary muscle and complete atrioventricular canal, repair may be accomplished by borrowing from the tricuspid portion of the anterior leaflet, rotating that part posteriorly, and partially closing the cleft. Small perforations of the mitral leaflet do not require closure and do not result in regurgitation. Echocardiographic and angiographic delineation of these abnormalities and thorough intraoperative exploration are important in avoiding pitfalls at the time of repair.     

Intraoperative transoesophageal echocardiography in a low birth weight neonate with atrioventricular septal defect

An 18-day-old male neonate (45 cm, 1.8 kg) with a history of cyanosis and congestive heart failure from an atrioventricular septal defect (AVSD) with a large left-to-right shunt was scheduled for surgical repair of the AVSD. After routine induction of anaesthesia with fentanyl and vecuronium, a 4.5-mm diameter transoesophageal echocardiography (TOE) probe was inserted into the oesophagus, and systematic echocardiographic evaluation was performed during surgery. After cardiopulmonary bypass was stopped, intraoperative TOE revealed mild residual mitral valve regurgitation. Because good left ventricular wall motion was confirmed and haemodynamic parameters were stable, cardiopulmonary bypass was not reinitiated. The patient's cardiac output was low in the postoperative intensive care unit. TOE was performed the next day to detect the source of this problem, revealed severe regurgitation compared with that observed intraoperatively. TOE was useful for evaluation of the residual mitral valve regurgitation, and we reconfirmed the importance of continuous monitoring even in a low birthweight neonate undergoing repair of a complete AVSD.     

改良单片法矫治儿童完全型房室间隔缺损单中心应用

目的总结应用改良单片法(modified single-patch,MSP)矫治儿童完全型房室间隔缺损(complete atrioventricular septal defect,CAVSD)的相关经验。方法回顾性分析2009年6月至2017年12月间在我中心采用MSP技术行CAVSD双心室矫治术141例患儿的临床资料,其中男62例、女79例,手术时中位年龄6(3,11)个月,中位体重5.8(4.5,7.0)kg。Rastelli分型:A型116例,B型14例,C型11例。合并Down’s综合征15例。记录体外循环时间、主动脉阻断时间、房室瓣反流等相关临床数据。结果术后17例患者出现重度左侧房室瓣反流(left atrioventricular valve regurgitation,LAVVR),6例患者出现重度右侧房室瓣反流(right atrioventricular valve regurgitation,RAVVR)。末次随访5例患者出现重度LAVVR,1例患者出现重度RAVVR。随访期间左室流出道梗阻(left ventricular outflow tract obstruction,LVOTO)1例,末次随访无LVOTO发生。早期死亡5例,随访死亡2例。再手术患者12例,距首次手术的中位间隔时间为268(8,1270)d。结论利用MSP技术外科矫治儿童CAVSD的预后良好,改善了患者术后死亡率和房室瓣反流程度。