2186例小儿法乐四联症外科治疗分析

目的 总结先天性心脏病法乐四联症(TOF)的外科治疗经验。方法 1973年4月～2003年7月2186例TOF病人，手术采用体外循环下行根治和姑息手术。结果 术后死亡146例(6．7％)，近3年手术死亡率降至1．6％。术后并发症主要是出血低心排、残余梗阻和分流、Ⅲ度房室传导阻滞。结论 根治术以大于1岁为宜，对缺氧发作症状明显的患儿则不受年龄限制。根治术中右室流出道小切口修补室间隔缺损和疏通右室流出道效果佳，自身心包单叶瓣补片扩大右室流出道对术后早期右心功能保护效果良好。应视解剖条件和年龄选择手术方法以及右室流出道疏通方法。     

一氧化氮和米力农联合应用改善Fontan术后肺循环阻力的疗效

Objective Inhaled nitric oxide(iNO) has been successful used for patients with increased pulmonary vascular re- slstance (PVR) shortly after Fontan operation, but its withdrawal rebound remains a major concem. We hypothesized that iNO associ- ated with milrinone could provide additive benefits for pulmonary hemodynamics and reduce the occurrence of iNO withdrawal rebound. Methods Thirty-one patieats with high transpulmonary pressure gradient(TPG, >10mmHg) and arterial oxygen saturation(SatO2, 0.85) after modified fenestrated Fontan operation were prospectively randomized into two groups, I.e. , group iNO (iNO at 10 ppm, dynamics, arterial blood oxygenation and occurrence of withdrawal rebound were compared between the two groups.Results Com- bined application achieved following improvenents including (1) decrement of CVP [(19.6±3.5)% vs. (15.2±4.6)% in group iNO, P<0.05] and TPG [(18.2±4.8)% vs. (15.3±2.6)% in group iNO, P<0.05); and increment of systemic aterial pres- sure [(8.7±2.7)% vs. (5.2±3.1)% in group iNO, P<0.05].(2)improvement of arterial oxygen saturation[(9.3±3.2)% vs. (6.8±2.8)% in group iNO, P<0.05)]. (3)occurrence of withdrawal rebound were significantly lower in group iNO+ Mil (1 patient vs. 6 patients in group iNO, P<0.05). Conclusion Combined uae of iNO and milrinone provided additional benefits as compared with exclusive use d iNO for patients with elevated PVR after Fontan procdure.     

永存第五对主动脉弓狭窄二例

永存第五对主动脉弓（persistent fifth aortic arch,PFAA）狭窄是罕见的主动脉弓畸形，我院自1988年7月至2004年8月收治2例PFAA狭窄同时合并主动脉弓离断（interrupted aortic arch,IAA）的患儿，手术治疗效果满意。     

室间隔缺损远离大动脉开口的右室双出口外科治疗与适应证选择

目的回顾室间隔缺损（VSD）远离2个大动脉开口的右室双出口（double—outlet right ventricle,DORV）的病理解剖特点、手术方法选择和治疗结果,对手术适应证和方法提出优化意见。方法1984年4月至2005年4月共有37例VSD远离2个大动脉开口的DORV患儿接受外科手术,年龄5个月～12岁,根据不同手术方法将病人分3组比较,其中心室内隧道修补术（intraventricular tunnel repair,IVR）组15例,Rastalli手术组10例,Fontan手术组12例。对限制性VSD,同时扩大VSD直径。结果手术住院死亡10例,死亡率27．0％（10／37例）,其中1999年4月后死亡2例,死亡率10．5％（2／19）。IVR的手术死亡率（46．7％,7／15例）明显高于Rastalli组（20％,2／10例）和Fontan组（8．3％,1／12例）。结论三尖瓣与肺动脉之问距离是否大于主动脉瓣口直径是区别选择IVR手术和Rastalli手术的主要依据。共同房室瓣、三尖瓣附属组织跨越或嵌入到VSD和主动脉之间的区域、两个心室发育不平衡是我们选择改良Fontan的主要适应证。     

小于6个月先天性心脏病患儿的外科治疗

目的 回顾总结小于6个月婴幼儿先天性心脏病的外科治疗.方法 2000年1月～2006年12月,手术纠治年龄小于6个月先天性心脏病1831例.包括:室间隔缺损并肺动脉高压453例,完全性大动脉错位214例、法乐四联症119例、完全性肺静脉异位引流106例、右室双出口69例、肺动脉瓣狭窄65例,完全性房室通道55例、动脉导管未闭53例、主动脉缩窄伴室间隔缺损46例、肺动脉闭锁伴室间隔缺损36例、肺动脉闭锁室间隔完整型31例、主动脉弓中断24例、右室双出口伴肺动脉瓣下室间隔缺损22例、其他538例.根据不同病种采取相应的手术方法纠治.结果 手术死亡98例,手术死亡率5.35%.随着手术方法不断改进,手术总死亡率从2000年的8.23%降至2006年的4.91%.随访:完全性大动脉错位术后发生室间隔残余漏1例,肺动脉瓣上狭窄5例,主动脉瓣上狭窄2例,主动脉瓣下狭窄1例,其中再次手术4例,远期死亡1例;完全性肺静脉异位引流心内型4例在术后出现肺静脉回流梗阻,2例死亡,2例再次手术解除梗阻.早期室间隔缺损术后发生残余漏5例,分流量小,不需再次手术;其余病例随访不完整.结论 婴幼儿先天性心脏病的手术处理时间非常重要,危重复杂型先天性心脏病如不早期手术,将失去手术机会,增加术后危险性和死亡率.     

先天性心脏病Taussig-Bing畸形的外科治疗

目的总结一期手术治疗Taussig-Bing畸形的经验。方法2000年5月～2006年12月上海儿童医学中心手术治疗Taussig-Bing畸形43例,其中合并主动脉弓病变12例;采用大动脉换位手术(ASO)矫治Taussig-Bing畸形42例,采用Kawashima手术1例,主动脉弓病变的矫治主要是将胸主动脉与主动脉弓下缘端端吻合或与升主动脉端侧吻合。结果住院死亡8例,总死亡率18.6%。合并主动脉弓病变者并发大动脉侧侧位和冠状动脉畸形比较常见,手术死亡率(25.0%,3/12例)比单纯Taussig-Bing畸形(16.1%,5/31例)者高,但差异无统计学意义。结论Taussig-Bing畸形病理解剖比较复杂,须根据解剖条件的不同选择不同的手术方法才能降低手术死亡率。     

完全性肺静脉异位引流病理谱及个体化手术治疗

目的 描绘完全性肺静脉异位引流(TAPVC)患儿肺静脉走行"路线图"和形态"变异图",阐明肺静脉病理谱的变化规律,促进有效个体化手术.方法 2006年4月至2009年9月,139例TAPVC患儿进行手术,心上型61例,心内型55例,心下型6例和混合型17例.病理诊断依据超声心动图、核磁共振、计算机断层扫描或心导管和心血管造影检查以及术中解剖,根据患儿TAPVC类型和解剖特点选择个体化手术方法.结果 1.病理谱:①肺静脉走行连接"路线图":心上型按照垂直静脉(VV)走行方式分为4种,左行(47例)、右行(9例)、后行(2例)和双行(3例);心内型按照肺静脉回流部位分为3种,汇入冠状静脉窦(49例)、汇入右心房(5例)和同时汇人CS和右心房(1例),再依据肺静脉开口数目分为4个开口、2个开口和1个开口三种亚型;心下型按照VV汇入体静脉方式分为4种:汇入门静脉(1例)、肝静脉(3例)、同时汇入门静脉和肝静脉(1例)以及汇入下腔静脉(1例);混合型按照肺静脉回流双侧是否对称分为双侧对称连接的"2+2"型(5例)、双侧不对称连接的"3+1"型(10例)和"怪异型"(2例).②肺静脉形态"变异图":肺静脉入口狭窄、发育不良或多分支(11例)、共汇肺静脉发育不良或伴内膜增生(4例)、VV扭曲、短小和狭窄(9例).2.手术结果:本组早期死亡6例(4.3%),其中低心排1例,肺静脉梗阻5例.中期随访因肺静脉梗阻再手术6例,术后轻微梗阻2例.结论 TAPVC患儿肺静脉病理谱广,个体差异大;按照肺静脉走行"路线图"和形态"变异图"有利术中肺静脉解剖的探查和个体化手术设计.

Abstract：

Objective To delineate the morphological spectrum of total anomalous pulmonary venous connection (TAPVC) by building the "road map" and the " variation chart" of pulmonary veins,and to advance individualized surgical treatment Methods Between April,2006 to June,2009, 139 consecutive patients with TAPVC underwent operations. There were 61 supracardiac,55 intracardiac,6 infracardiac and 17 mixed types. Pathological diagnosis was made by echocardiogram,magnetic resonance imaging, computerized tomography,or the cardiac catheterization and operative findings during operation. The option of procedure was determined by findings. Results (1) Pathological spectrum:①" the road map"of the pulmonary veins: the patients with supracardiac type were divided into 4 subtypes according to the course of vertical veins:left course (47 cases),right course (9 cases),posterior course(2 cases) and double courses (3 cases). The patients with intracardiac type were divided into 3 subtypes according to the draining site of pulmonary veins: to coronary sinus (49 cases) ,to right atrium (5 cases) or to coronary sinus and right atrium (1 case),and the openings of pulmonary veins may be 4,2 or 1 in each subtype. The patients with infracardiac type were divided into 4 subtypes according to the draining site of vertical vein:to portal vein (1 case),to hepatic vein(3 cases),to portal vein and hepatic vein (1 case) and to inferior vena cava (1 cases). The patients with mixed type were divided into 3 subtypes: bilateral and symmetrical connections "2 + 2" pulmonary venous drainage pattern; bilateral and asymmetrical connections"3 +1" pulmonary venous drainage pattern and bizarre anatomic variants. ② The morphological "variation chart" of pulmonary veins individual pulmonary vein stenosis or excessive tributary veins (11 cases) showed: hypoplastic confluence veins (4 cases) and vertical veins distortion or elongation or forming hemodynamic vise or common pulmonary vein that drained to coronary sinus or right atrium through a narrowed short vertical vein or a small window(9cases).(2)Surgical results：early death was encountered in 6 cases(4.3％).The causes included 10W cardiac output syndrome in 1 case,and pulmonary veins stenosis and associated complications in the other 5 patients.Six patients with pulmonary restenosis underwent reoperation.All survived.Two patients had mild residual obstruction during Intermediate-term follow-up.Conclusions The patients with TAPVC had a wide spectrum of pulmonary veins with high inter-individual variation.It was useful to delineate the anatomy of pulmonary veins and to plan personalized procedures during operations according to the"road map"and"variation chart"of pulmonary veins.     

改良Fontan手术加共同房室瓣置换术治疗心脾综合征

目的:总结对心脾综合征患儿同时进行Fontan手术和共同房室瓣(CAVV)置换术的方法和经验。方法:对3例伴有CAVV中到重度返流的心脾综合征患儿进行Fontan手术的同时置入29～31mm的ATS机械二尖瓣,观察治疗效果。结果:无手术死亡,1例并发完全性房室传导阻滞植入心外膜永久埋藏式起搏器,随访1～6年心功能均为Ⅰ级。结论:心脾综合征患儿的CAVV存在明显的结构缺陷,CAVV成形术效果差,Fontan手术加CAVV置换术是有效可行的手术方案;在行CAVV置换术时,应尽量保留CAVV瓣下组织结构,对Fontan术后心功能的稳定尤为有利;褥式缝合时应浅缝缝针尽量置于瓣环靠近瓣叶处,避免损伤房室传导系统;术后须及早抗凝治疗,维持INR在1.5～2.5,可避免抗凝并发症的发生。     

法洛氏四联症缺氧发作的急诊手术

目的对１４例法洛氏四联症伴严重缺氧发作患儿进行急诊或亚急诊手术,旨在探讨一种重症四联症的有效治疗方法。方法根治手术１３例,姑息手术１例;开始发作距手术间隔时间５～７２ｈ。结果无住院病死率,根治术后低心排７例。结论法洛氏四联症缺氧发作急诊或亚急诊手术在救治危重患儿中是可行的及必要的。