右心室双出口的手术治疗

目的　总结右心室双出口手术治疗的早期和远期结果及经验。　方法　 1985年 5月～ 2 0 0 1年 12月 4 1例右心室双出口患者接受了外科治疗 ,其中男 2 6例、女 15例 ,年龄 10个月～ 2 1岁 ,平均 (10± 6 )岁 ,体重 6 5～ 6 5 0kg ,平均 (2 6± 16 )kg。 4 1例中 ,14例行经典的Rastelli手术 (内隧道外管道手术 ) ;11例行经内隧道成形左心室流出道、右心室流出道直接疏通或用心包片加宽 ;11例行改良Rastelli手术 ;2例行全腔静脉肺动脉吻合术 ;2例行双向格林手术 ;1例行大动脉调转术。结果　早期 (术后 1个月 )死亡 1例 ,出院 4 0例。随访 36例 ,随访 5个月～ 17年 ,中位数为 7年 ,无死亡 ,二次手术 3例。所有患者紫绀消失 ,症状明显改善 ,心功能Ⅰ级 34例 ,心功能Ⅱ级 2例 ,能够进行正常的生活、学习和工作。　结论　绝大多数右心室双出口患者经双心室矫治可以取得很好的早期及远期结果 ;对于合并其他严重畸形的患者可行右心室旁路手术。     

阜外医院71例Rastelli手术的近中期结果

目的总结分析阜外医院Rastelli手术的近中期结果。方法2010年5月至2017年3月阜外医院完成71例Rastelli手术治疗大动脉转位(TGA)或者右心室双出口(DORV)合并室间隔缺损(VSD)和肺动脉狭窄患儿,男48例,女23例;手术时年龄(4.7±2.7)岁,其中10例完全型大动脉转位;27例DORV,34例矫正型TGA(CTGA)。30例有手术史,其中Blalock-Taussig分流13例,双向Glenn手术17例。31例同时扩大室间隔缺损。外管道使用同种带瓣管道9例,牛颈静脉管道56例,自制Gore-Tex外管道6例;管道直径(17.9±3.3)mm。结果本组主动脉阻断(132.0±71.1)min,体外循环(209.0±83.4)min。机械通气时间(102.6±81.7)h。术后住院(13.6±12.8)天。早期死亡1例(1.4%)。早期并发症12例(16.9%),其中因Ⅲ度房室传导阻滞安装永久起搏器4例,心包积液开窗3例,延迟关胸3例,二次开胸2例。术后随访4个月~6.8年。1、5年生存率分别为97.2%,97.2%;1、5年免除右心室流出道狭窄(RVOTO)率分别为98.6%,84.1%;1、5年免除干预率分别为98.6%,90.0%。随访期间置换外管道1例,介入球囊扩张7例10次。所有患儿最后一次随访均未见左心室流出道狭窄,左心室到主动脉压差(10.5±8.8)mmHg。结论Rastelli手术适用于部分DORV、CTGA、TGA合并VSD和严重的肺动脉狭窄或肺动脉闭锁患儿,早期死亡比例低,中期效果好,但是远期常需要再手术,尤其是置换外管道。     

心室双出口手术治疗临床研究

目的：探讨心室双出口的手术治疗效果。方法：回顾性分析72例心室双出口病人手术治疗方法及治疗效果。右室双出口71例中SDD型64例，ILL型3例，SDL型3例，IDD型1例；左室双出口1例（ILD型）。采用左心室-主动脉心内隧道连接61例，全腔静脉-肺动脉连接2例，左心室-主动脉心内隧道并右心室-肺动脉心外管道连接3例，Glenn分流6例。结果：死亡2例。1例术后3个月发现残余室间隔缺损，予再次手术缝合。余者效果良好，无远期死亡及相关并发症发生。结论：根据心室双出口的不同类型，选择最佳手术方式及掌握手术时机是成功的重要因素，重建通畅良好的左或右室流出道是提高手术成功率的关键因素。     

右心室双出口的外科治疗

右心室双出口是少见而又复杂的先天性心脏病，其解剖形态有较多的变异，因而手术治疗方法和效果也因病理类型而异。现报道我们右心室双出口的外科治疗及其效果。     

双心室矫治术治疗右心室双出口合并完全性房室间隔缺损

目的总结右心室双出口(DORV)合并完全性房室间隔缺损(AVSD)的双心室解剖矫治经验。方法回顾性分析1996年1月至2010年12月阜外心血管病医院14例DORV-AVSD患者施行双心室解剖矫治术的临床资料,其中男9例,女5例;年龄6个月～31岁。患者均行双心室解剖矫治术,经右心房和右心室切口,疏通右心室流出道,分隔并成形房室瓣,采用"逗号状"补片修补室间隔缺损,同时构建通畅的左心室流出道,自体心包闭合Ⅰ孔房间隔缺损,用心包或跨瓣环补片加宽右心室流出道。结果 1996年1月至2008年12月收治的10例患者中,住院死亡5例,其中术中不能脱离体外循环3例,不能脱离呼吸机2例;住院时间23～105 d,住ICU时间5～90 d,机械通气时间1～52 d。2009年1月至2010年12月收治的4例患者中,无住院死亡,术后未发生并发症;住院时间21～41 d,住ICU时间4～21 d,机械通气时间1～7 d。随访9例,随访时间6～26个月,随访期间无死亡,无流出道残余梗阻。结论 DORV-AVSD患者可一期行双心室矫治术,近年来手术效果明显提高。     

Rastelli手术治疗大动脉转位伴室间隔缺损

目的介绍Rastelli手术治疗大动脉错位伴室间隔缺损的经验.方法全组49例中男29例,女20例.平均年龄5.6岁;平均体重17.2kg.完全性大动脉转位31例,纠正性大动脉转位18例;伴肺动脉狭窄45例,伴肺动脉高压4例.均在低温体外循环下行Rastelli手术.二期根治5例.体外循环灌注(178.5±52.5)min;主动脉阻断(109.2±38.3) min.结果手术早期死亡6例,死亡率12.2%.死因为肺动脉高压危象、肾衰、心律紊乱和严重低心输出量综合征.术后并发症有心律紊乱、肺动脉高压危象、蛛网膜下腔出血、脑功能紊乱、肾衰及多脏器功能衰竭、心包或胸腔积液、感染等.CICU平均监护7.3 d.随访中因同种带瓣大动脉(VHC)感染死亡1例.结论完全性大动脉转位手术中,右室流出道直切口有利于心内隧道修补室间隔缺损;纠正性大动脉转位手术中,解剖右室径路显露缺损较好且易避开传导系统,但不利于术后心功能恢复.而解剖左室径路修补室间隔缺损的房室传导阻滞发生率高;大于4岁者手术宜选择大号VHC可减少再次手术几率;对无长段左室流出道狭窄的完全性大动脉转位病婴,可在动脉换位术基础上行肺动脉瓣叶交界切开或Konno术以解除左室流出道梗阻.为防止术后功能性二尖瓣反流,对伴肺动脉狭窄的纠正性大动脉转位病儿,提倡心房-大动脉双换位手术.     

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。     

改良Fontan手术的疗效分析

目的探讨改良Fontan手术后的早期效果[以住院期间或1个月(住院超过1个月者)统计]及远期效果(以手术后5年统计),为其临床应用提供理论依据.方法1990年9月至1996年5月行改良Fontan手术病儿52例,年龄2.5～16岁,随访49例(93%),平均随访时间(5.2±0.4)年.分析包括死亡、手术失败、心律失常、血栓形成、胸腔渗液等参数.结果3例早期死亡,4例远期死亡;1例早期再次手术,1例远期再次手术.术后早期生存率94%,成功率(不包括术后疗效不佳且危及生命,需二次手术者)92%,室上性心动过速6%,心动过缓10%,胸腔渗液38%;术后远期生存率87%,成功率83%,室上性心动过速10%,心动过缓13%,胸水渗出6%.结论在复杂先天性心脏病的治疗中,即使应用于不同的解剖诊断病人,改良Fontan手术仍有良好的术后早期及远期效果.     

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.     

A case report: Rastelli operation for double outlet right ventricle associated with a chordae insertion to the infundibular septum]

A successful Rastelli operation for double outlet right ventricle (DORV) with a chordae insertion of the tricuspid valve to the infundibular septum was reported. A patient was a 6-year-old boy and the diagnosis was DORV, d-malposition of the aorta and pulmonary stenosis. The infundibular septum was not resected but mobilized with two incision, one anterior and vertical, and other one subaortic. This procedure allowed the construction of the tunnel similar to the closure of a large, subarterial VSD and of the straight unobstractive left outflow tract. The postoperative echocardiographic and angiographic examination revealed neither the pressure gradient in the left ventricular outflow tract nor the tricuspid regurgitation.     

右室双出口的外科治疗

目的：总结手术纠治右室双出口６７ 例的经验。资料与结果：手术年龄４ 个月～１２ 岁,平均（４ ．９８ ±２ ．９６） 岁;体重４ ．１ ～３６ ．０ｋｇ,平均（１５ ．１３ ±５ ．５４）ｋｇ 。其中伴右室流出道梗阻４８ 例,肺动脉高压１８例,肺动脉瓣闭锁１ 例。１３ 例行姑息手术者无死亡。５４ 例行根治术,其中４ 例又行ＩＩ期根治,６ 例行Ｆｏｎｔａｎ 纠治术;根治手术死亡５ 例,病死率８ ．６２ ％ 。本组总病死率７ ．４６ ％ 。结论：右室双出口伴肺动脉高压者,必须早期手术,防止肺血管病变发生。对肺动脉瓣下型室缺的手术纠治较困难,死亡率高。心内隧道补片方法,术后需定期随访,及时发现左室流出道梗阻,必要时需再次手术解除。     

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.     

完全性心内膜垫缺损合并右室双出口的双心室修复

Objective Double-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.Methods From July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.Results One death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.     

完全性心内膜垫缺损合并右室双出口的双心室修复

目的 总结完全性心内膜垫缺损合并右室双出口双心室修复的经验.方法 2006年7月至209年11月对6例完全性心内膜垫缺损合并右室双出口病人采用双片法修补完全性心内膜垫缺损,人工血管补片作右心室内隧道,连接室间隔缺损与主动脉口,引导左心室血经内隧道进入主动脉;应用牛心包补片扩大右室流出道;肺动脉瓣环小者用带瓣补片跨环修补右室流出道;心室内隧道有可能阻塞右室流出道造成重度梗阻者,右室流出道与主肺动脉之间安置带瓣管道(Rastelli手术).结果 术后死亡1例,占16.6%,其余病例恢复良好.结论 完全性心内膜垫缺损合并右室双出口一期双心室修复可获得良好效果.

Abstract：

Objective Double-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.Methods From July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.Results One death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.     

完全性心内膜垫缺损合并右室双出口的双心室修复

Objective Double-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.Methods From July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.Results One death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.