改良Fontan手术术后多普勒超声随访分析

改良Ｆｏｎｔａｎ手术是纠治各种复杂先天性心脏病的重要手术方式之一〔1〕,为评价该手术的远期效果 ,我们利用多普勒超声技术 ,对 2 0例改良Ｆｏｎｔａｎ手术术后病儿进行随访 ,现报告如下。临床资料　自 1996年 11月至 1998年 11月 ,我们对Ｆｏｎｔａｎ手术术后 1周～ 8年的病儿 2 0例进行超声随访 ,其中男 16例 ,女 4例 ;年龄 1～ 14岁 ,平均 8 5岁。包括三尖瓣闭锁合并室间隔缺损、肺动脉狭窄 6例 ,单心室合并室间隔缺损、肺动脉狭窄 6例 ,右室双出口合并室间隔缺损、肺动脉狭窄 4例 ,完全性大血管错位合并室间隔缺损 3例 ,三尖瓣狭窄…     

双调转术(Double-Switch手术)的临床应用

目的 探讨Double-Switch手术方法的临床应用。方法 采用Double-Switch手术方法纠治房室连接和心室大动脉连接不一致的复杂先天性心脏病病儿3例，其中1例为纠正型大血管错位、室间隔缺损、肺动脉狭窄伴轻度三尖瓣反流，2例为房室连接不一致、右心室双出口、肺动脉狭窄、室间隔缺损、三尖瓣轻一中度反流。心房内采用Senning术，心室内采用Rastelli术的方法，从解剖上彻底予以纠治。结果 手术均取得成功，恢复顺利，分别术后11d、18d和21d出院。术后随访心电图窦性节律，超声检查示心功能正常，腔静脉、肺静脉血流通畅，同种带瓣管道和心内隧道血流无梗阻。结论 对房室连接和心室大动脉连接不一致病例，特别是解剖右心室功能不全和三尖瓣反流者，应首选Double-Switch手术。     

主动脉缩窄合并心内畸形的治疗进展

主动脉缩窄 (coarctation ,CoA)占先天性心脏病的 5 %～10 % ,主要合并心内畸形包括室间隔缺损 (VSD)、房间隔缺损(ASD)、房室隔缺损 (AVSD)等。其中VSD发生率最高 ,可达5 5 % 〔1,2〕。目前对其治疗原则的争论主要在于手术方法和手术时机的选择。手术时机的选择目前 ,随着手术技术、体外循环及重症监护的进步 ,主动脉缩窄合并心内畸形的治疗效果已非常满意 ,但对一期根治或者二期纠治的选择仍存在很大争论〔3〕。1.分期手术的特点和结果传统的分期手术指在第一次手术时纠治主动脉缩窄或同时行肺动脉环缩术 ,后期再行心内畸形的治疗…     

小婴儿危重复杂先天性心脏病的外科治疗

目的　评估小婴儿危重复杂先天性心脏病体外循环心内直视手术的治疗效果。方法　回顾分析我院 2 0 0 0年 1月至 2 0 0 4年 1月外科治疗 187例年龄小于 6个月的危重复杂先天性心脏病的临床资料。病儿年龄 3d～ 6个月 ,平均 (4 15± 2 32 )个月 ;体重 3～ 7kg ,平均 (4 4 2± 0 5 6 )kg。包括室间隔缺损合并肺动脉高压、完全性大血管错位、主动脉缩窄合并心内畸形、完全性肺静脉异位引流、法洛四联症 ,完全性房室隔缺损、室间隔完整的肺动脉闭锁、三房心、重度肺动脉狭窄、右旋心合并心内畸形、新生儿心脏横纹肌瘤等。结果　术后死亡 9例 ,死亡率 4 81%。术后并发低心排出量综合征、残余分流、肺炎肺不张、肺出血等。平均机械通气 (17 5± 9 7)h。平均术后ICU停留时间为 (4 9± 1 5 )d。随访 4～ 4 8个月 ,生存病儿心功能良好 ,生长发育明显加快。结论　随着小儿心脏外科技术的发展 ,小婴儿危重复杂先天性心脏病的体外循环手术是可行的。     

右上腔静脉缺如伴永存左上腔静脉合并心内畸形2例

例1 男，28岁。发现心脏杂音20余年。10年前曾在本院诊断为先天性心脏病室间隔缺损伴重度肺动脉高压，病人拒绝手术。查体：胸骨左缘第3肋间可闻及3／Ⅵ级收缩期杂音。心电图示P波I导联倒置，aVR导联直立，电轴右偏，右心室增大，左房心律。胸部X线检查示肺动脉呈瘤样突出，心胸比率0．68。超声心动图检查示先天性心脏病，嵴下型室间隔缺损2．5cm，肺动脉增粗5．0cm，右室壁增厚0．8～1．0cm。动     

复杂先天性心脏病右心室流出道重建困难的处理

目的探讨复杂先天性心脏病在纠治过程中右心室流出道(RVOT)重建困难的处理方法和其适应证、手术方法、应用中的注意要点。方法回顾性分析2008年1月至2010年12月广州市妇女儿童医疗中心26例复杂先天性心脏病患者行手术治疗的临床资料。其中男14例,女12例;年龄(2.9±2.0)岁;体重(12.2±6.0)kg。所有患者在纠治过程中RVOT重建困难,分别采用以下方法进行处理:(1)肺动脉从根部切断与主动脉交叉后吻合于右心室切口;(2)肺动脉从根部切断直接下拉吻合于右心室切口;(3)肺动脉切断后远心端与右心室切口间的后壁用左心耳或右心耳做后壁,人工补片做前壁,重建RVOT;(4)肺动脉与右心室切口间用自体心包做成的心包卷管道连接。结果无手术死亡,无Ⅲ°房室传导阻滞。10例患者因尿少行腹膜透析治疗,心脏压塞和/或胸腔出血开胸止血4例。所有患者随访0.5～3.0年复查:应用肺动脉从根部切断与主动脉交叉后吻合于右心室切口和肺动脉从根部切断直接下拉吻合于右心室切口两种方法的7例患者中肺动脉血流速度加快3例,2.5～3.0 m/s;残留中度以上肺动脉高压5例;中至重度肺动脉反流6例,其中肺动脉血流速度增快2例,残留中度肺动脉高压4例。结论以上4种RVOT重建方法在复杂先天性心脏病RVOT重建中应用得当有良好的手术效果;前2种方法有肺动脉血流速度增快的可能性;肺动脉瓣反流与术后肺动脉高压或肺动脉梗阻有关。     

婴幼儿先天性心脏病伴气管狭窄的一期矫治

目的回顾总结一期纠治婴幼儿先天性心脏病（先心病）伴气管狭窄的体会。方法2001年8月至2005年11月，6例先心病伴气管狭窄的婴幼儿行一期手术矫治。手术年龄24d～3岁；体重4．2～10．0kg，平均（7．98±2．03）kg。其中法洛四联症3例，1例伴肺动脉闭锁；室间隔缺损2例，1例伴主动脉缩窄；肺动脉吊带1例。所有病儿均在低温体外循环下行先心病纠治术，同时处理气管狭窄。结果术后早期1例左、右支气管均匀性狭窄，不能脱离呼吸机死亡。1例肺动脉吊带者术后顺利出院，3个月后出现气管内肉芽增生，家属放弃治疗。余4例术后恢复良好，随访6个月至4年，临床上无气促表现。超声复查心功能良好，其中3例经CT复查，显示气管吻合口通畅，无明显狭窄。结论先心病伴先天性气管狭窄，以一期手术治疗为好，不但术中在体外循环下纠治气管狭窄较安全，而且同时纠治先心病有利于手术后病儿康复。     

肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道

目的明确肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道后肺动脉发育、瓣膜反流及右心功能情况。方法回顾性分析2002年11月至2012年9月广东省人民医院31例肺动脉闭锁合并室间隔缺损患者的临床资料,其中男20例,女11例;患儿手术时14d～14．50岁（47．90±53．84）个月,均采用肺动脉下拉重建右心室流出道。随访评估其吻合口及肺动脉发育情况、肺动脉与三尖瓣反流及其与随访时间的相关关系;采用右心室应变等指标评估右心功能。结果术后早期死亡3例。随访27例（27／31,87．1％）,随访时间4～129（35．97±28．24）个月。随访期间无死亡。根治术后元吻合口再狭窄,姑息术后吻合口相对狭窄4例。吻合口及左、右肺动脉的直径均明显大于术后早期;肺动脉反流及i尖瓣反流加重,但肺动脉反流量与随访时间无相关关系。远期右心功能整体良好。结论肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道效果良好,吻合口及肺动脉分支可生长发育,术后中远期右心功能良好。     

自体肺动脉连接右心室纠治婴儿永存动脉干

目的　评估自体肺动脉连接右心室方法纠治婴儿永存动脉干的疗效。方法　 2 0 0 1年 11月至 2 0 0 2年 7月 ,5例病婴采用自体肺动脉连接右心室方法纠治永存动脉干。 5例病婴为 2～ 9月龄 ,体重 3 5～ 8 0kg ,平均 (5 5± 1 6 )kg。永存动脉干I型 3例 ,II型 2例。结果　手术无死亡。术后随访 10～18个月 ,超声心动图显示室间隔缺损修补后无残余分流 ,肺动脉至右室流出道无残余梗阻 ,除 1例共同瓣轻度反流 ,余 4例均无明显反流。结论　自体肺动脉直接连接至右心室切口 ,完全由自体组织连接 ,使其能生长 ,不引起远期梗阻或狭窄 ,避免了再次手术的痛苦和危险 ,是较为理想的手术方法。但手术时间尚短 ,仍需进一步随访。     

11／2心室修补手术

目的 应用11／2心室修补手术治疗小儿复杂先天性心脏病,探讨11／2心室修补手术指征。方法 自1996年3月－2000年5月,对11例患者进行了11／2心室修补术。其中包括三尖瓣闭锁（1a型）、三尖瓣狭窄、室间隔完整型肺动脉闭锁、肺动脉狭窄和室间隔缺损等合并右心发育不良6例;埃布斯坦综合征（Ebstein anomaly C 型）、肺动脉闭锁伴Ebstein综合征等右心室功能不良2例以及合并左侧上腔静脉异位引流至左心房3例。所有患者在心内修补手术后进行双向腔静脉肺动脉吻合术,其中房间隔开窗3例。结果 手术死亡1例,存活10例,术后血流动力学稳定,随访结果满意。结论 对复杂先天性心脏病某些选择性病例,11／2心室修补手术是一种安全、可靠、有效的手术方法。     

Surgical treatment of interrupted aortic arch in infants under three months of age

From March, 1982, through March, 1988, 8 infants less than 3 months of age underwent surgical treatment of interrupted aortic arch. Five patients had IAA type A and 3 patients had type B. Seven patients with associated VSD underwent staged operations and 1 infant with aortopulmonary window underwent primary repair off cardiopulmonary bypass. Aortic arch reconstruction was by subclavian aortic anastomosis (6) or large tube graft (8 mm) interposition (2) combined with pulmonary artery banding (5). All seven patients with associated VSD survived the first-stage operation. One patient subsequently underwent pulmonary artery banding, two underwent patch aortoplasty and four patients underwent intracardiac repair and removal of a pulmonary artery band, with six survivors (86%). The operative death occurred in an infant in whom modified Damus-Kaye-Stansel operation was carried out for severe subaortic stenosis, which rerouting all left ventricular blood through VSD and the main pulmonary artery into the ascending aorta and reconstructing right ventricular-distal pulmonary artery connection by a valved external conduit. All six surviving patients are doing well at present (mean follow up of 3 years) without any significant pressure gradient between the ascending and thoracic aorta. Based on these data we conclude: (1) Aortic arch reconstruction and pulmonary artery banding can be reliably performed even in critically ill infants with IAA and isolated VSD. (2) The primary repair will provide better chance of survival in infants with IAA associated with significant LVOTO, truncus arteriosus or aortopulmonary window that do not readily lend themselves to pulmonary artery banding.     

Pulmonary artery sling with tracheal stenosis —Primary repair in infancy—

Between 1984 and 1996 five infants underwent surgical repair of pulmonary artery sling associated with severe congenital tracheal stenosis. All infants had symptoms of severe respiratory distress and three of them required ventilator support preoperatively. ages ranged from 2 to 11 months (mean age 6 months). Complete tracheal rings were present in all patients as an associated lesion and right upper lobe tracheal bronchus in 3 patients. The length of tracheal stenosis ranged from 18 to 45 mm (median 40 mm). Three had associated intracardiac anomalies (Scimitar sydrome (1), VSD (1), double-outlet right ventricle with VSD (1), double-outlet right ventricle with pulmonary hypertension (1)). Surgical intervention was carried out through a right thoraco tomy (1) or median sternotomy (4). Cardiopulmonary bypass (CPB) was used in 3 patients and extracorporeal membrane oxgenator (ECMO). in 1. All infants had reimplantation of the left pulmonary artery into the main pulmonary artery left anterior to the trachea. Four patients underwent simultaneous tracheoplasty using costal cartilage grafts and one had complete resection of obstructed trachea between the right upper lobe tracheal bronchus and carina. The length of resected trachea was about 30% of the entire length of the trachea. Three infants underwent simultaneous intracardiac repair. There was no hospital death. All were weaned from ventilatory support and extubated on 1 to 16 months (mean 4, 5 months) postoperatively. AS an additional procedure, aortopexy, removal of granulation tissue or balloon dilatation of the trachea were carried out in one patient each following tracheoplasty using cartilage grafts. There was one late death at 1 year postoperatively. Three of 4 survivors are doing well with no stridor. We adono stridor. We adovocate 1) early aggressive primary repair of pulmonary artrey sling with tracheal stenosis, 2) concomitant repair of tracheal lesion and intracardiac anomalies whenever possible, 3) application of CPB or ECMO to avoid cumbersome intubation technique, and 4) utmost effort to perform tracheal resection and end-to-end anastomosis.     

冠状动脉畸形对复杂先心病右室流出道重建的影响

目的探讨冠状动脉畸形对复杂先心病右室流出道（RVOT）重建的影响。方法自1989年4月至2004年5月治疗18例合并冠状动脉畸形的法洛四联症（14例）和右室双出口（4例）患者。右冠状动脉开口缺如7例,左冠状动脉开口缺如4例,左前降支起自右冠状动脉6例,左右冠状动脉交通支形成蔓状血管丛1例。均在全身麻醉低温体外循环下手术,1例行姑息手术,17例行根治术,RVOT重建的方法：游离畸形冠状动脉从其下方做切口,RVOT心包补片扩大术4例;3例在异常交通支下方平行切口修补室间隔缺损（VSD）,交通支上方纵切口加宽RVOT和肺动脉瓣环;4例行右心房切口修补VSD,RVOT小切口和肺动脉切口心包补片扩大术;3例肺动脉主干与RVOT直接吻合;3例同种带瓣主动脉重建RVOT。结果术后早期死于顽固性低心排血量综合征1例,无严重出血并发症和呼吸道并发症,姑息手术1例,血氧饱和度由术前的68．0％升至82.0％。16例随访10个月～8年,1例术后1年不明原因猝死;超声心动图示轻度残余漏3例,残余梗阻3例。结论复杂先天性心病并冠状动脉畸形RVOT重建时,应根据冠状动脉畸形特点选择手术方式。     

Surgical treatment of complex cardiac anomalies: the 'one and one half ventricle repair'.

OBJECTIVE: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. METHODS: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one. Four patients underwent previous surgery which included: main pulmonary artery (MPA) banding in two patients, pulmonary valvotomy, central shunt and right ventricular outflow tract reconstruction in one, pulmonary artery separation from truncus arteriosus and modified Blalock-Taussig shunt in one, and MPA closure in one. Two patients underwent a bidirectional cavo-pulmonary shunt before the one and a one half ventricle repair. Associated cardiac lesions were treated simultaneously. RESULTS: There were no hospital deaths. All the patients were discharged home in good clinical conditions. There were no late deaths or reoperations. At mean follow-up of 29.8 months (range 8 months to 7.3 years) all the patients are alive and in good general conditions. MPA percutaneous balloon dilation was performed in two patients at 33 and 4 months, respectively, both after MPA reconstruction (which was previously ligated) and dilation of the left pulmonary artery branch in one patient, repeated twice at 10 and 14 months from repair, for a hypoplastic left pulmonary artery after truncus arteriosus repair. CONCLUSIONS: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic and or a dysplastic RV by means of one and one half ventricle repair has the advantage of reducing the surgical risk for biventricular repair, and compared to the Fontan circulation, it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation. Short and medium-term results are promising. Longer follow-up is needed, to prove the efficacy of such a repair, in the long term.     

Surgical treatment of complex cardiac anomalies: the ‘one and one half ventricle repair’

Objective: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. Methods: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one. Four patients underwent previous surgery which included: main pulmonary artery (MPA) banding in two patients, pulmonary valvotomy, central shunt and right ventricular outflow tract reconstruction in one, pulmonary artery separation from truncus arteriosus and modified Blalock–Taussig shunt in one, and MPA closure in one. Two patients underwent a bidirectional cavo-pulmonary shunt before the one and a one half ventricle repair. Associated cardiac lesions were treated simultaneously. Results: There were no hospital deaths. All the patients were discharged home in good clinical conditions. There were no late deaths or reoperations. At mean follow-up of 29.8 months (range 8 months to 7.3 years) all the patients are alive and in good general conditions. MPA percutaneous balloon dilation was performed in two patients at 33 and 4 months, respectively, both after MPA reconstruction (which was previously ligated) and dilation of the left pulmonary artery branch in one patient, repeated twice at 10 and 14 months from repair, for a hypoplastic left pulmonary artery after truncus arteriosus repair. Conclusions: Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic and or a dysplastic RV by means of one and one half ventricle repair has the advantage of reducing the surgical risk for biventricular repair, and compared to the Fontan circulation, it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation. Short and medium-term results are promising. Longer follow-up is needed, to prove the efficacy of such a repair, in the long term.     

自制带单瓣管道重建右心室-肺动脉连接治疗复杂先天性心脏病

目的总结自制带单瓣管道重建右心室-肺动脉连接治疗复杂先天性心脏病的经验,探讨术中操作要点,评估该疗法的近中期结果。方法回顾性分析我科2006年1月至2018年6月利用自制带单瓣管道外科治疗的65例复杂先天性心脏病患者的临床资料。其中男42例、女23例,年龄5~23(9.9±4.2)岁,体重15~65(26.2±9.9)kg。根据所使用带单瓣管道将患者分为两组:带单瓣人工血管组19例,带单瓣牛心包管组46例。其中室间隔缺损(VSD)合并肺动脉闭锁(PA)48例,矫正性大动脉转位合并肺动脉狭窄10例,法洛四联症合并单支冠状动脉畸形5例,右室双出口合并肺动脉狭窄及单支冠状动脉畸形2例。结果术后早期死亡2例,均为VSD/PA行人工血管外管道根治患者。术中测右室流出道压差:人工血管组13~37(25.2±4.9)mm Hg,牛心包管组5~23(10.5±3.3)mm Hg。出院时心脏彩超显示外管道内无明显反流。术后失访1例,随访时间8个月至13年。随访期间,三尖瓣中度反流5例,管道内中度反流32例,右室流出道重度梗阻7例,中度梗阻11例,轻度梗阻25例。无中晚期死亡。心功能分级(NYHA)Ⅰ~Ⅱ级,活动耐量良好。结论自制带单瓣外管道重建右心室-肺动脉连接临床效果良好,牛心包管道术中流出道压差小于人工血管,我们更推荐牛心包管用于低龄、低体重患者。     

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.     

改良Norwood手术临床应用

目的总结改良Norwood手术的临床应用经验。方法2004年7月和2005年3月分别为1例7月龄单心室伴左心室流出道梗阻女婴,采用主肺动脉和主动脉弓下缘直接吻合、主动脉肺动脉人工血管分流完成改良Norwood手术;1例出生13 d左心发育不良综合征新生儿,采用主肺动脉和主动脉弓下缘直接吻合、右心室肺动脉人工血管分流完成改良Norwood手术。结果例1术后平稳,动脉压为85～100/25～40mmHg（1 mm Hg=0．133kPa）,动脉血氧饱和度为0．75～0．85,术后第3d撤离辅助呼吸,第11 d出院。例2术后平稳,动脉压为65～80／40～60mmHg,动脉血氧饱和度为0．67～0．89,术后第12 d撤离辅助呼吸,第28 d出院。结论改良Norwood手术的先进方法和技术,值得在我国推广应用。     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.