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.     

改良Rastelli手术治疗右心室双出口合并左前位主动脉

目的 探讨右心室双出口合并左前位主动脉患者采用改良Rastelli的手术效果。方法 对10例右心室双出口患者采用改良Rastelli手术,9例为右心室双出口合并右前位主动脉,1位为镜面右位心型右心室双出口。除1例外,9例患者均有肺动脉瓣及瓣下狭窄。室间隙缺损位于肺动脉下或远离两大动脉。体外循环下行改良Rastelli手术,经右心室切口用涤纶片或人工血管片修补空间隔缺损,形态左心室至主动脉的内隧道。切断主肺动脉,闭合近端。用带瓣同种肺动脉或主动脉及人造血管,建立右心室流入道三关瓣环下方至主肺动脉的外管道。结果 10例患者术后恢复良好,超声心去科显示所有内隧道及外管道功能正常,无血流梗阻征象。结论 改良Rastelli手术是治疗右心室双出口合并左前位主动脉的满意术式,它可以彻底矫治肺动脉瓣及瓣下狭窄和右向左分流。     

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。     

右室双出口室间隔缺损远离两大动脉开口的外科治疗

自１９９１年１２月至１９９３年９月外科治疗右室双出口、室间隔缺损远离两大动脉开口５例。房室顺序一致４例，房室顺序不一致１例，其中伴有肺动脉狭窄３例。手术经右室切口用Ｇｏｒｅ－Ｔｅｘ补片建立室间隔缺损－主动脉内隧道３，中室注出道切口直接缝合２３例，跨肺动脉瓣环自身心包补片扩大１例；经右房切口修室间隔缺损，右室－肺动脉同种异体主动脉外管道１例；改良Ｆｏｎｔａｎ纠治术１例。手术４例成功，１例死亡。作者认     

Lecompte术治疗小儿心室大动脉连接异常

目的 探讨和评价近年来Lecompte术在小儿心室大动脉连接异常的先天性心脏病（先心病）中的临床应用价值。方法 2000年1月至2006年4月18例心室大动脉连接异常的先心病病儿行Lecompte手术治疗。年龄3个月～9．2岁；体重4．5～27．0kg。大血管转位（D-TGA）、室间隔缺损（VSD）、肺动脉狭窄6例，右心室双出13（DORV）S例，永存动脉干7例。6例D-TGA中主动脉和肺动脉呈前后位4例，另2例与4例DORV主肺动脉呈右前左后关系，1例DORV主动脉动脉呈左前右后关系。7例因位于两半月瓣之间的漏斗（圆锥）隔妨碍建立VSD和主动脉之间隧道，遂切除漏斗隔。8例行经典Lecompte术。10例进行改良Leeompte术，前壁再用心包补片扩大。术毕病儿右心室和左心室收缩峰压比值（PRwPLV）为0．20～0．45。结果所有病儿均生存，5例表现为右心功能不全，术后彩色超声检查，2例右室流出道压差30．0—34．51mmg Hg／（1 mm Hg=0．133kPa）；2例行右肺动脉改良Leeompte术病儿压差37．5～47．3 mm Hg.分别随访3个月、2年，此2例病儿右肺动脉残余压差24．0—29．31／mm Hg。结论Lecompt手术可以减轻左心室流出道梗阻，避免使用人工管道，采用自体肺动脉重建肺动脉流出道具有生长潜能，特别适用于婴幼儿、甚至新生儿。     

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.     

右室双出口15例治疗体会

自 1991年 8月至 2 0 0 0年 5月 ,针对不同病理解剖特点 ,我们采用相应的不同手术方案行右室双出口 (ＤＯＲＶ)矫治术15例 ,取得满意的效果 ,现报道如下。临床资料　 15例病人中男 8例 ,女 7例 ;年龄 3～ 19岁。按ＶａｎＰｒａａｇｈ对先心病的三节段命名法及Ｓｔｅｗａｒｄ对该畸形室间隔缺损 (ＶＳＤ)位置的分类见表 1。表 1　 15例ＤＯＲＶ〔1〕分类ＳＤＤＩＬＬＳＤＬＩＬＤ主动脉下 (ＳＡ)ＶＳＤ肺动脉下 (ＳＰ)ＶＳＤ靠近两大动脉开口 (ＤＣ)ＶＳＤ远离两大动脉开口 (ＮＣ)ＶＳＤ712—1———3——————1总　计 10 131注 :ＳＤＤ -…     

心尖部室间隔缺损修补临床分析

目的探讨心尖部室间隔缺损(VSD)外科治疗方法的选择,以提高治疗水平。方法回顾性分析1999年1月~2004年7月间29例存在心尖部VSD行室间隔修补术患者的临床资料,其手术切口入路共有4种:右心房切口、右室心尖部切口、左室心尖部切口和右心室流出道联合左室心尖部切口;并分析比较不同入路的手术效果。结果本组患者手术死亡2例(6.9%),1例为左室心尖部切口术后发生低心排血量死亡,1例术中不能停机,行心肌活检示心内膜弹力纤维增生症死亡;发生残余漏4例(13.8%),直径3~5mm,均为右心房切口,不需再次手术治疗;其余患者均治愈出院。术后11例患者随访2~34个月,超声心动图显示均未见室壁瘤形成。结论心尖部VSD患者手术结果良好,但残余漏的发生率仍较高,术终应常规行食管超声心动图检查。手术切口选择应根据术中VSD的实际位置,尽可能采用右心房切口,必要时可经右室心尖部切口,避免左心室切口。     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

目的 分析和评估纠正型大血管错位伴肺动脉狭窄病儿采用双调转(Double-Switch)手术的治疗效果.方法 2001年8月至2008年12月采用Double-Switch手术行纠正型大血管错位伴肺动脉狭窄21例.其中男11例,女10例;年龄3.5个月至6.3岁,平均(31±18)个月;体重6～28 kg,平均(13.1±6.5)kg.室间隔缺损(VSD)为膜周型15例,远离大动脉1例,肺动脉下5例.轻度二尖瓣反流2例,中度反流2例.采用Senning+Rastelli手术方法 .13例右心室与肺总动脉的连接采用牛颈静脉管道.结果 全组均生存.体外循环转流156～287 min,平均(202.0±35.8)min;主动脉阻断93～161min,平均(138.0±19.8)min.均无完全性房室传导阻滞.随防2～5年,上腔静脉流速增快3例(1.8～2.2)m/s,其中1例术后2.6年再次手术解除上腔静脉梗阻,余2例在随防中;肺静脉回流均无明显梗阻;牛颈静脉管道通畅,解剖右室流出道连接处梗阻2例,压力阶差在30～45 mm Hg(1 mm Hg=0.133kPa),尚在随防中.结论 Double-Switch手术纠治纠正型大血管错位伴肺动脉狭窄的复杂型先心病,取得了较好效果,但仍需进一步随访,以观察这类复杂手术方法 的长期疗效.     

303例右室双出口手术后早期转归

目的　总结右室双出口 (DORV)病例的术后处理及不同外科术式的早期转归。方法　 1988年 1月至 2 0 0 3年 12月期间收治 30 3例DORV病儿 ,按室间隔缺损 (VSD)的部位分为主动脉下VSD ,肺动脉下VSD ,双动脉下VSD和远离大动脉下VSD 4组。总结比较不同外科术式术后的并发症和死亡率及其原因。结果　再手术 18例 ,死亡 2 9例 ,死亡率为 9 6 % ,其中肺动脉下VSD死亡率最高 ,为 2 4 . 4 % (χ2 =14 . 12 ,P <0 . 0 1) ;根治术和姑息术的死亡率分别为 10 . 6 %和 6 . 6 % (χ2 =1. 0 3,P >0 .0 5 )。死亡原因主要是低心排血量综合征 (LCOS) ,占 82 . 6 % ;其也是术后主要并发症 ,占 31 .0 %。结论　LCOS是术后主要并发症和死亡的高危因素。有效预防和控制LCOS是取得治疗成功的关键。     

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。     

房室连接不一致右心室双出口根治手术的选择与适应证优化

目的　回顾性分析房室连接不一致右心室双出口(doubleoutlet right ventricle,DORV)的病理解剖特点、手术方法选择和治疗结果,探讨手术适应证和手术方法的优化。　方法　1990年9月至2 0 0 4年8月对7例房室连接不一致的DORV患者施行根治手术治疗,年龄3～7岁,其中形态学左心室与肺动脉心外管道连接和室间隔缺损修补术3例,double- switch(双调转)手术2例,改良Fontan手术2例。　结果　手术早期死亡1例(14 .3% ,1/ 7) ,为手术后2个月并发Hom ograft感染。 度房室传导阻滞1例,安装永久心外膜起搏器;1例改良Fontan手术后并发上腔静脉回流梗阻和心包积液,症状改善后出院。Double- switch手术后平均监护时间最短为术后6 d。　结论　房室连接不一致DORV根治手术应首选double- switch手术,尤其对形态学右心室功能不全和三尖瓣关闭不全者,如果合并多种复杂畸形解剖无法矫治,可选择单个心室的修补方案。     

Successful surgical treatment of a case of corrected transposition in situs inversus with associated anomalies

A 33-year-old female with corrected transposition (situs inversus) with ventricular septal defect (VSD), atrial septal defect (ASD), and pulmonary stenosis (valvular and subvalvular) was operated successfully. Closure of ASD and infracristal VSD was performed through right atrial approach. In order to get complete exposure of VSD, a part of anterior leaflet of mitral valve was detached temporally. An external conduit was interposed between the morphologic left ventricle and the main pulmonary artery to relieve pulmonary stenosis. Postoperative course was uneventful except a transient complete heart block for a day. Several technical aspects of the radical operation were discussed including VSD closure method to avoid damaging the atrioventricular conducting tissues and reconstructive procedure of morphological left ventricular outflow.     

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

BACKGROUND: The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. METHODS: Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. RESULTS: There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. CONCLUSIONS: The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.     

左胸微创切口在儿童干下型室间隔缺损直视修补术中的应用

目的总结左胸微创切口在儿童干下型室间隔缺损(ventricular septal defect,VSD)直视修补术中的应用方法,并评价临床效果。方法回顾性分析2015年10月至2019年4月上海交通大学医学院附属新华医院心胸外科21例干下型VSD儿童的临床资料,其中男13例、女8例,年龄5~13(9.1±2.2)岁,体重22~55(35.6±9.5)kg。VSD最大直径4~15(9.1±3.3)mm,术前合并主动脉瓣右冠瓣轻度脱垂8例,主动脉瓣轻到轻中度关闭不全4例。取左侧第2或第3肋间胸骨旁横切口进胸,股动、静脉插管建立体外循环,阻断升主动脉后,在肺动脉瓣上横行切开肺动脉,根据VSD大小、形态直接缝合或补片修补干下型VSD。出院前及随访常规复查经胸超声心动图评价VSD修补效果及主动脉瓣开闭情况。结果全组患儿均顺利经左胸微创切口完成VSD直视修补术,无转为正中开胸手术者。VSD修补方法:补片修补(n=15),直接缝合(n=6)。体外循环时间45~68(57.1±6.3)min,主动脉阻断时间23~40(32.6±4.7)min,术后呼吸机辅助时间5~9(6.3±1.3)h,术后24 h胸腔引流量33~105(57.5±17.7)mL,术后住院时间5~8(5.7±1.0)d。出院前及随访复查经胸超声心动图提示VSD闭合良好,无VSD残余漏;主动脉瓣轻度关闭不全1例。围术期及随访无死亡病例,无房室传导阻滞、切口愈合不良、胸廓畸形等并发症发生。结论左胸微创切口可安全、有效地应用于儿童干下型VSD直视修补术中,早、中期治疗效果满意。     

Successful closure of ventricular septal defect through a left-sided ventriculotomy in corrected transposition of the great vessels.

Corrected transposition of the great vessels is often associated with other cardiac anomalies. Ventricular septal defect (VSD) is the most common among them. Closure of a VSD is usually performed through a right-sided ventriculotomy. We had previously closed the VSD through the right-sided ventricle in 2 patients with corrected transposition of the great vessels and in 2 others with double-outlet right ventricle with ventricular inversion, which resulted in complete heart block. Recently we have closed the VSD through the left-sided ventricle in 3 patients with corrected transposition, and no block resulted.     

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.     

Ventricular morphology and coronary arterial anatomy in hearts with isometric atrial appendages.

BACKGROUND: Knowledge of the precise anatomy can be advantageous when striving to improve surgical results in patients with visceral heterotaxy. METHODS: We studied the ventricular mass, and its coronary arterial supply, in 125 specimens with isomeric right and 58 with isomeric left appendages. RESULTS: The situation in which each atrium connected to its own ventricle was the most common arrangement in hearts with isomeric left appendages. The pattern with both atriums connecting to the same ventricle was more frequently seen in those with isomeric right appendages. Concordant ventriculoarterial connections were seen in only 4% of cases with isomeric right appendages, but were seen in 45% of those with isomeric left appendages. Abnormal patterns in branching of the coronary arteries were commonly associated with abnormal ventricular architecture. The morphologically right or left ventricular arteries were frequently lacking in those hearts with a dominant ventricle and a rudimentary and incomplete ventricle. A solitary coronary artery was seen in 13%. CONCLUSIONS: Recognition of these abnormalities is of clinical importance if optimal surgical strategies are to be established for patients with visceral heterotaxy.