大动脉转换术在复杂先天性心脏病治疗中的应用

目的 回顾总结我院近3年采用大动脉转换术治疗小儿复杂先天性心脏病(先心病)的临床经验。方法2000年1月至2003年5月,采用大动脉转换术(Switch手术)治疗小儿复杂先心病61例。其中完全性大血管错位(TGA)45例,包括室间隔完整型(TFGA-IVS)26例,伴室间隔缺损(TGA,VSD)19例;右心室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing)16例。结果TGA,IVS死亡2例;TGA-VSD死亡4例;Taussig-Bing死亡4例;总手术病死率16％。术后随访3个月-3年,所有病例青紫症状消失,活动能力明显增强。1例Taussig-Bing术前二尖瓣轻～中度返流,术后仍为中度返流。2例TGA术后出现主动脉和肺动脉瓣上狭窄,压差40mmHg,1例肺动脉瓣下狭窄和残余室间隔缺损,3个月后再次手术治愈。结论大动脉转换术应用于完全性大血管错位的纠治,手术效果满意;应用于右室双出口肺动脉瓣下室间隔缺损的纠治,不但可早期纠治,防止其肺血管阻塞性病变发生,而且避免了心内修补左室流出道梗阻的远期并发症。     

107例大动脉转换术的冠状动脉解剖类型和治疗结果

目的总结分析近年来107例大动脉转换术的冠状动脉解剖分类和手术结果，以进一步提高大动脉转换术的手术成功率。方法2000年1月至2004年9月，采用大动脉转换术纠治完全性大血管错位室隔完整型（TGA／IVS）44例，完全性大血管错位伴室间隔缺损（TGA/VSD）38例，右室双出口伴肺动脉瓣下室间隔缺损、肺动脉高压（Taussig-Bing）25例，其中冠状动脉畸形28例，占全组28％。结果大动脉转换术107例中死亡17例，总病死率15．88％。其中TGA/IVS组死亡4例，病死率9．02％；TGA／VSD组死亡8例，病死率21．05％；Taussig-Bing组死亡5例，病死率20．00％。90例术后随访6个月～4年，VSD残余漏3例，2例分别在术后1个月和2个月自愈，1例同时伴右室流出道梗阻，术后3个月再次手术治愈。肺动脉瓣上狭窄2例尚在随访中。其余病儿活动良好，无任何心肌缺血表现。结论冠状动脉畸形的变异很多，分型比较困难，Leiden方法简单，易掌握。Taussig-Bing的冠状动脉畸形发生率较高，TGA／VSD的冠状动脉畸形达40％左右，进行Switch手术时应注意。     

大动脉转换术治疗新生儿完全性大动脉错位

目的 回顾总结完全性大动脉错位（D－TGA）的手术治疗。方法 1999年11月至2001年8月，采用大动脉转换术（Switch）手术方法纠治新生儿D－TGA 16例，其中室间隔完整型大动脉错位（D－GA／IVS）6例，大动脉错位伴室间隔缺损（D－TGA／VSD）10例。所有病例都在深低温低流量和深低温停循环下行Switch纠治术。结果 Switch手术治疗TGA共16例，其中TGA／IVS 6例，无死亡；TGA／VSD 10例，死亡2例，均为伴有冠状动脉畸形者。14例随访2－20个月，VSD残余漏1例，肺动脉轻度残余梗阻2例，14例心功能均良好。结论 Switch手术是D－TGA纠治的首选方法，但必须早期手术。     

高压氧治疗心内直视手术后脑损害的护理

我科1989～1995年应用高压氧治疗心内直视手术后发生不同性质脑损害病人22例，治愈率77．27％，现报告如下。1临床资料与方法本级22例，男13例，女9例，年龄6～51岁。均行心内直视手术，其中主动脉瓣、二尖双瓣替换术3例，预激综合征肯氏京电击切断术1例，法鲁氏四联症纠治术2例．房间隔缺损修补术4例．右室汉出口纠治术1例，下腔静脉成型术1例,室间隔缺损修补术4例，二尖居替换术6例。术后引起脑气检12例，脑干损害（无自主呼吸）5例，弥漫性脑损害5例。治疗方法：分别于术后1～12d行高压氧治疗。舱内压力2kg／cm2，截面罩吸纯氧．定时通风…     

大动脉转换术113例的手术结果分析

目的回顾性分析大动脉转换术的手术疗效。方法2001年1月至2005年12月,采用大动脉转换术纠治完全性大动脉错位113例,其中室间隔完整型大动脉错位（TGA／IVS）60例,伴室间隔缺损大动脉错位（TGA／VSD）53例。患儿体重最轻2,3kg,年龄最小出生后6h。在深低温停循环和低流量下行大动脉转换术。结果手术总死亡率9．7％,其中TGA／IVS死亡5例,手术死亡率8．3％,TGA／VSD死亡6例,手术死亡率11.3％。随着手术方法的不断改进和围手术期以及体外循环转流技术的提高,其手术死亡率不断下降,从早期的手术死亡率16.6％降至目前的5．6％。结论冠状动脉畸形是导致大动脉转换术死亡的关键,TGA／VSD的冠状动脉畸形发生率较TGA／IVS要高。主动脉和肺动脉的位置并不影响手术成功率。左心室与右心室压力比例〈0．6时,不能行大动脉转换术,否则术后将出现严重左心室低心排。     

动脉转位术的临床应用

目的 总结动脉转位术(arterial switch operation，ASO)治疗完全型大动脉转位(transposition of the great arteries，TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(VSD)的临床经验。方法 采用ASO治疗小儿先天性心脏病32例，其中TGA22例，伴室间隔完整型(intact ventricular septum，IVS)9例，伴VSDl3例；右心室双出口伴肺动脉瓣下VSD(Taussig—Bing)10例。结果TGA／IVS9例中死亡1例，TGA／VSD13例中死亡4例，Taussig-Bing10例死亡3例，总手术死亡率25％(8／32)。术后随访3个月～2年，所有患者紫绀消失，活动能力明显增强。1例Taussig—Bing术前二尖瓣轻-中度反流，术后仍为中度反流；2例TGA主动脉和肺动脉瓣上狭窄，压差40mmHg(1kPa=7．5mmHg)，1例肺动脉瓣下狭窄和残余VSD，3个月后再次手术治愈。结论 ASO已广泛应用于TGA的纠治，手术效果满意；应用于右心室双出口肺动脉瓣下VSD的早期纠治，不但可防止发生肺血管阻塞性病变，而且避免了心内修补左心室流出道梗阻的远期并发症。     

一期手术纠治主-肺动脉窗及伴发畸形

目的总结一期手术纠治主－肺动脉窗及伴发畸形的临床经验，以提高手术疗效。方法我科共收治26例主－肺动脉窗患者．男14例．女12例；年龄1．4±1．6岁；体重7．8±3．8kg。其中单纯主－肺动脉窗8例，合并主动脉弓中断、右肺动脉异常起源于主动脉、法洛四联症、房间隔缺损、室间隔缺损、二尖瓣反流和气管狭窄等18例。25例患者一期手术经主动脉切口补片关闭瘘口纠治主－肺动脉窗，同时纠治伴发的畸形；1例放弃治疗。结果全组无手术死亡。2例伴主动脉弓中断、动脉导管未闭和右肺动脉起源于升主动脉的新生儿术后3d延迟关胸；1例术后出血，3h后再次开胸止血。术后随访22例．随访时间1个月～4年。所有患者无明显的主动脉瓣上狭窄和肺动脉分支狭窄，2例残留轻度二尖瓣反流，1例残留轻至中度二尖瓣反流。结论主－肺动脉窗患者早期易发生肺动脉高压，一经诊断应立即手术。手术方式首选修补主－肺动脉窗及一期纠治伴发畸形。尽管主－肺动脉窗可合并各种心内外畸形，但早期手术纠治可获得较好的中长期疗效。     

肺动脉右心室直接吻合纠治复杂先天性心脏病

目的 探讨肺动脉右心室直接吻合纠治复杂先天性心脏病的效果。方法 2001年3月至2004年6月，对17例复杂先天性心脏病病儿采用肺动脉右心室直接吻合方法予以纠治，其中3周岁以下者11例。肺动脉闭锁合并室间隔缺损6例，完全性大动脉错位合并室间隔缺损和肺动脉狭窄4例，永存动脉干4例，法洛四联症合并冠状动脉横跨右心室流出道3例。12例一期根治，5例分期纠治。结果 手术死亡1例。5例分期手术中的3例已成功二期根治。随访1～40个月，所有病儿生长发育良好，心功能0～Ⅰ级。结论 肺动脉右心室直接吻合应用于纠治某些复杂先天性心脏病可取得满意效果。对无同种带瓣管道可选用的婴幼儿病例，此术式尤为适用。     

右心室双出口的解剖矫治

目的 总结不同方法进行解剖矫治右心室双出口(DORV)经验.方法 2007年5月至2012年5月,收治135例DORV患儿,男79例,女56例;年龄为出生后25天～12岁;体质量3.5 ～ 30.0 kg.根据室间隔缺损的位置选择不同手术方法:89例主动脉瓣下缺损及33例双瓣下缺损患儿均行右心室双出口解剖矫治术;8例肺动脉瓣下缺损(Taussig-Bing畸形)患儿中5例行Rastelli手术,3例行动脉调转术±室间隔修补术;5例远离大动脉缺损中3例行Rastelli手术,2例行动脉调转术±室间隔修补术.结果 住院死亡5例,占3.70％;分别为1例肺动脉瓣下缺损行动脉调转术±室间隔修补术,1例双瓣下缺损,2例主动脉瓣下缺损(均合并肺动脉瓣狭窄)均行合右室双出口解剖矫治术,1例远离大动脉缺损行Rastelli手术者.结论 右室双出口病理解剖比较复杂,须根据不同的解剖条件选择不同的手术方法才能减少手术死亡.     

肺动脉瓣缺如综合征的外科治疗

目的总结肺动脉瓣缺如综合征的外科治疗经验。方法1985年1月至2003年9月，纠治肺动脉瓣缺如综合征18例中男13例，女5例。伴法洛四联症17例，伴室间隔缺损1例。全组均在中低温体外循环下行纠治手术，即切除、折叠整形扩张的肺动脉；解除右心室流出道梗阻同时关闭室间隔缺损。7例肺动脉瓣处置单瓣。结果术后死亡1例，死亡率5．6％。术后并发低心排血量综合征3例，Ⅲ度房室传导阻滞和心包积液各1例，经治疗均痊愈。术后随访0．5—5．0年，轻度残余右室流出道梗阻1例、轻度肺动脉瓣反流7例、中度肺动脉瓣反流3例、重度肺动脉瓣反流伴充血性右心衰1例。结论婴幼儿型肺动脉瓣缺如需立即手术，在解除右室流出道梗阻和肺动脉做切除、折叠整形时，须在肺动脉瓣区置入单瓣或带瓣管道。儿童型肺动脉瓣缺如选择性根治，处理原则同普通伴发畸形。     

Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases

BACKGROUND: Double outlet right ventricle with L-malposition of the great arteries is a rare type of double outlet right ventricle. This article reviews our experience in treating this disease surgically. METHODS: Between September 1995 and May 1999, 9 patients with double outlet right ventricle [S, D, L] underwent modified Rastelli procedure at Fu Wai Hospital. In all patients, the two great arteries originated completely from the right ventricle. The malformation was associated with subaortic conus and L-transposition of the great arteries. The ventricular septal defect (VSD) was subpulmonary in 3 patients, and non-committed in 6. All but one patient had either pulmonary valvular stenosis or subpulmonary stenosis. No patient had any type of palliative operation before. A right ventriculotomy was made to repair the VSD with a Dacron or vascular prosthesitic patch, and an intraventricular tunnel was made between the left ventricle and the aorta. The main pulmonary artery was divided and the proximal end was closed. A homograft conduit was implanted between the inlet of the right ventricle and the main pulmonary artery. In the Rastelli procedure, the conduit is usually positioned between the right ventricular outflow tract and the pulmonary artery. RESULTS: All patients survived and recovered uneventfully. Echocardiography demonstrated that all intraventricular tunnels and valved conduits were functioning well. The results were satisfactory. CONCLUSIONS: Modified Rastelli procedure is an optimal method for surgically treating double outlet right ventricle with left-malposition of the great arteries. It can completely correct the right ventricular outflow tract stenosis, and right to left shunt, and avoid injuring the right coronary artery.     

Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD.

OBJECTIVE: An arterial switch operation is considered a good alternative for the repair of double-outlet right ventricle (DORV) with atrioventricular concordance connection and subpulmonary ventricular septal defect (VSD) when intraventricular rerouting is not feasible. The clinical results of an arterial switch operation with ventricular septal defect closure for this anomaly were studied. METHODS: Between 1986 and 1997, 27 patients ranging from 10 days to 5 years of age (mean 0.4 years) underwent an arterial switch operation with ventricular septal defect closure for the correction of double outlet right ventricle with subpulmonary VSD. The 50% rule was used to define double-outlet right ventricle. Arch anomalies were associated in nine cases, and were corrected either previously or simultaneously. A subarterial muscle resection was performed in 14 without any subsequent stenosis of the ventricular outflow tract. The relationship of the great arteries was mostly anteroposterior in 15 and mostly side by side in 12. The left coronary artery (main trunk or circumflex artery) courses behind the pulmonary artery in 15/27 (six/15 in the anteroposterior relation and ten/12 in the side by side relation). The Lecompte maneuver was used to reconstruct the pulmonary artery in all but five cases with a side by side relationship of the great arteries. RESULTS: There was one operative death (3.7%) and three late deaths. The actuarial survival rate was 83 +/- 8% at 9 years. Right ventricular outflow tract obstruction including peripheral pulmonary stenosis developed in seven cases operated on in the early era. The reoperation free rate was 46 +/- 20% at 9 years. CONCLUSION: Although double-outlet right ventricle with subpulmonary VSD has complex features, including an aortic arch obstruction and coronary artery anomalies, an optimal definitive surgical repair using an arterial switch operation can be performed safely with a thorough understanding of this variable anomaly. The prevention of right ventricular outflow tract obstruction at the time of an arterial switch operation may thus help improve the rate of late morbidity.     

双调转术治疗矫正型大动脉转位合并心内畸形

目的总结双调转手术治疗矫正型大动脉转位（cTGA）合并心内畸形的经验。方法2002年4月至2004年6月，行双调转手术治疗cTGA合并心内畸形病儿7例，其中男6例，女1例；年龄4—15岁，平均8．4岁。SLL型6例，IDD型1例。合并室间隔缺损6例，右室双出口1例，继发孔房间隔缺损2例，肺动脉狭窄6例；3例SLL型为右旋心，1例IDD型为左旋心。手术方式包括改良Senning＋Rastelli 4例，Mustard＋Rastelli＋双向Glenn手术、Senning＋Rastelli手术、改良Senning＋Switch手术各1例。结果术后早期死亡1例，为cTGA合并室间隔缺损、肺动脉高压，行改良Senning＋Switch手术者，死因为左心功能衰竭。早期主要并发症有低心排血量综合征、房室分离各1例，胸腔积液和低蛋白血症2例。随访2—24个月，恢复良好，复查多普勒超声心动图、心电图和X线胸片显示，窦性心律5例，结性心律1例，心功能均为Ⅰ级。结论双调转手术可以达到解剖矫治cTGA合并心内畸形，早期手术死亡率低，中、远期心功能效果良好。SLL型病例采用改良Senning心房内调转手术效果优于Senning和Mustard手术。     

Arterial switch operation in transposition of great arteries and double-outlet right ventricle. Experience at the Brompton Hospital, London

Between 1981 and 1989 89 patients underwent anatomic correction of transposition of the great arteries with intact ventricular septum (n = 32), transposition with ventricular septum defect (n = 37) and double outlet right ventricle with subpulmonary ventricular septal defect (n = 20). The perioperative mortality was 17% (n = 15). The causes of death are discussed. The most common postoperative complication is right ventricular outflow tract-obstruction (RVOTO), accounting for two late deaths and necessitating reoperations in many patients. Besides RVOTO the mid-term results in arterial switch-operation are encouraging. Especially satisfactory are the absence of any problems with growth of the transferred coronary arteries, the function of the new aortic valve as well as the performance of the left ventricle. Of course, long-term experience is not yet available.     

Surgical results in patients with double outlet right ventricle: a 20-year experience.

BACKGROUND: The objective of this study was to review our surgical strategy in children with double outlet right ventricle and to assess risk factors for early and late mortality and reoperation. METHODS: Patients (n = 124; June 1980 to January 2000; age range, 7 days to 16 years; mean, 2.8 years) who underwent repair of double outlet right ventricle. The patients were divided into three groups. Group 1 (n = 47) had noncomplex patients with atrioventricular concordance, a single ventricular septal defect, balanced ventricles, no straddling atrioventricular valves, and no major pulmonary artery anomalies. Group 2 (n = 39) included patients with double outlet right ventricle and a subpulmonary ventricular septal defect (Taussig-Bing). Group 3 (n = 38) had patients with complex anomalies including straddling atrioventricular valves, atrioventricular septal defects or a hypoplastic valve or ventricle, or a combination of atrioventricular septal defects and hypoplastic valve or ventricle. RESULTS: Four types of definitive repairs were performed: (1) intraventricular tunnel repair with a baffle from the left ventricle to the aorta (n = 53); (2) use of a valved or nonvalved conduit (n = 20); (3) arterial switch operation with a patch committing the left ventricle to the neo-aorta (n = 16); and (4) cavopulmonary shunt and Fontan procedures (n = 33). Two patients with late postoperative cardiomyopathy had heart transplantation. Potential risk factors included location of the largest ventricular septal defect, presence of additional ventricular septal defects, ventricular outflow obstruction or hypoplasia, or both ventricular outflow obstruction and hypoplasia, previous palliation, and type of definitive operation. There were six early deaths (4.8%) and four late deaths (3.2%), and two heart transplants (1.6%). Overall 15-year survival was 95.8%, 89.7%, and 89.5% for groups 1, 2, and 3, respectively (p = 0.08). Thirteen patients (11.4%) have required 15 reoperations. Mean follow-up for survivors was 76.6 +/- 52.8 months. Up-to-date follow-ups are available on 114 surviving patients. Ninety-five of these patients (83.3%) were in New York Heart Association class I, and the remaining 19 patients (16.7%) were in New York Heart Association class II. Freedom from reoperation was 87%, 72%, and 100% at 15 years for groups 1, 2, and 3, respectively (p = 0.11). CONCLUSIONS: Survival was high for all patients with double outlet right ventricle undergoing intraventricular tunnel repair, arterial switch operation, and repair with a conduit or a modified Fontan procedure. Careful attention to preoperative anatomy dictates the best surgical approach and will enhance outcomes.     

The morphologic nature of noncommitted ventricular septal defects in specimens with double-outlet right ventricle

BACKGROUND: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications. METHODS: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices. RESULTS: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle. CONCLUSION: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.     

半旋转动脉干调转术治疗完全性大动脉转位合并室间隔缺损和肺动脉狭窄

目的 报道半旋转动脉干调转术治疗完全大动脉转位(TGA)合并室间隔缺损(VSD)和肺动脉狭窄(PS)的近期疗效.方法 2例病儿男、女各1例,分别为16岁和19个月.均为TGA/VSD/PS.采用半旋转动脉干调转术治疗.结果 2例病儿均生存.女病儿术后发生低心排出量综合征和毛细血管渗漏综合征,治疗后痊愈.男病儿术后恢复良好.术后分别随访10、9个月,心功能Ⅰ级.结论 半旋转动脉干调转术可有效治疗TGA合并VSD和PS.长期效果仍需观察.