同种主动脉治疗复杂先天性心脏病

目的　总结用同种带瓣主动脉和同种主动脉治疗 11例复杂先天性心脏病的经验。　方法　对 11例法洛四联症合并肺动脉闭锁和动脉导管未闭 ,矫正型大动脉转位合并室间隔缺损和肺动脉狭窄 ,完全型大动脉转位合并室间隔缺损和肺动脉狭窄 ,右心室双出口合并完全性房室通道 , 型永存动脉干 ,主动脉弓中断合并动脉导管未闭 ,主动脉缩窄合并动脉导管未闭和二尖瓣关闭不全患者施行了手术治疗 ;其中 Rastelli手术 7例 ,全腔静脉与肺动脉连接 (TCPC) 2例 ,升主动脉至降主动脉旁路移植 2例。　结果　手术死亡 1例 ;慢性心力衰竭 1例 ,内科治疗 1年后心功能 级。随访 1个月～ 7.2年 ,无远期死亡。　结论　同种主动脉是矫正复杂先天性心脏病的理想材料     

同种带瓣大动脉重建右心室流出道术后护理

对164例复杂先天性心脏病患者采用同种带瓣大动脉（VHC）重建右心室流出道术。结果术后8～42h拔除气管插管，ICU滞留时间3～80d，除20例死亡外，其余均康复出院，出院时心功能均为Ⅰ级。随访3个月至10年，仅1例因。VHC感染死亡，其余心功能良好。表明术后对呼吸、循环、泌尿和神经系统的严密监护是手术成功的重要环节。     

787例小于6月龄先天性心脏病患者外科治疗的临床分析

目的回顾性总结婴幼儿先天性心脏病的手术时机和手术治疗方法,以进一步提高手术成功率和远期疗效。方法1988年1月～2003年6月,手术纠治年龄小于6个月的先天性心脏病患者787例。主要病种包括完全性大动脉错位109例,完全性肺静脉异位引流51例,肺动脉闭锁16例,主动脉缩窄33例,室间隔缺损伴肺动脉高压299例,法洛四联症44例,右心室双出口23例,室间隔完整型肺动脉闭锁9例等;对787例患者根据不同病种采取相应的手术方法纠治。结果手术死亡77例,手术死亡率9.78%(77/787)。随着手术方法的不断改进,手术总死亡率从1988～1995年的25%降至2003年的4.11%。随访完全性大动脉错位患者中发生VSD残余漏1例,术后3个月再次手术治愈;随访中发生肺动脉和主动脉瓣上狭窄2例。完全性肺静脉异位引流心内型患者中2例分别在术后4d和2个月出现肺静脉回流梗阻,1例死亡,1例再次手术解除梗阻。室间隔缺损患者中发生残余漏5例,分流量小,不需再次手术。其余病例随访资料不完整。结论对婴幼儿先天性心脏病施行手术治疗的时间非常重要,危重复杂型先天性心脏病患者如不早期手术,将失去手术机会,增加术后危险性和死亡率。     

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。     

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。     

同种带瓣心外管道的临床应用

目的　探讨同种带瓣主动脉和肺动脉心外管道治疗复杂先天性心脏病的临床效果。　方法　应用深低温保存同种带瓣主动脉或肺动脉心外管道重建右心室流出道治疗复杂先天性心脏病 31例 ,其中 ,右心室双出口 6例 ,矫正型大动脉转位 2 1例 ,完全型大动脉转位 2例 ,三尖瓣闭锁 1例 ,感染性心内膜炎合并主动脉瓣关闭不全 1例。应用同种主动脉 2 7例 ,同种肺动脉 4例。　结果　术后同种管道吻合口通畅 ,无压差、无扭曲和受压 ,同种瓣膜活动良好。　结论　同种带瓣主动脉和肺动脉心外管道具有生物活性和完整的瓣膜功能 ,可从解剖学和血流动力学上矫正心脏畸形     

同种带瓣大动脉重建右心室流出道术后护理

对164例复杂先天性心脏病患者采用同种带瓣大动脉(VHC)重建右心室流出道术.结果 术后8～42 h拔除气管插管,ICU滞留时间3～80 d,除20例死亡外,其余均康复出院,出院时心功能均为I级.随访3个月至10年,仅1例因VHC感染死亡,其余心功能良好.表明术后对呼吸、循环、泌尿和神经系统的严密监护是手术成功的重要环节.     

同种带瓣大动脉在重建右室流出道中的应用

应用自行采集和冷冻保存的同种带瓣大动脉（ＶＨＣ）为５０例复杂先心病病儿重建右室流出道，观察其疗效及存在的问题。本组５０例，年龄１４个月～１６岁（５．９±３．２岁）。包括１７例完全性大动脉错位、７例右室双出口、５例永存动脉干和２１例法乐四联症。主动脉ＶＨＣ应用于４２例（裁剪成２瓣ＶＨＣ４例），肺动脉ＶＨＣ应用于２例，单瓣肺动脉作为补片扩大ＲＶＯＴ５例，同种无名动脉架桥于ＲＶＯＴ１例。结果：术后住院死亡７例。３５例随访１个月～７年，晚期死亡２例，均为感染。１例ＩＩ型ＰＴＡ病儿术后３年半发生心内膜炎，手术清除ＶＨＣ瓣膜处赘生物，仍存活。３３例长期生存者中５例Ｘ线胸片示ＶＨＣ有钙化，均为主动脉材料，仅１例有轻度压力阶差。结论：ＶＨＣ可广泛应用于复杂先心病。ＶＨＣ的长期通畅比合成管道好，肺动脉ＶＨＣ优于主动脉ＶＨＣ。为预防植入后ＶＨＣ的感染，应重视和改进ＶＨＣ的收集、保存和应用过程中的预防感染措施     

应用系列改良Fontan手术治疗复杂先天性心脏病

目的探讨系列改良Fontan手术的特点和治疗复杂先天性心脏病的效果，进一步提高对其临床应用的认识。方法回顾性分析1992年9月～2006年6月期间77例复杂先天性心脏病患者行改良Fontan手术治疗的临床资料，其中行右心房肺动脉吻合术21例，心房内板障或管道全腔静脉-肺动脉连接术28例，心外人工管道全腔静脉-肺动脉连接术24例，自体右房壁管道全腔静脉肺动脉连接术2例，自体带蒂心包心外管道全腔静脉-肺动脉连接术1例，主肺动脉与下腔静脉吻合全腔静脉-肺动脉连接术1例。结果术后早期死亡5例，其中死于心力衰竭3例，突发心律失常1例，脑出血1例。再次手术1例，术后早期生存率93．5％（72／77），手术成功率92．0％。左心室舒张期末内径（LVEDD）较术前减小（52．5±7．8mm vs．62．5±11．0mm，P=0．013），左心室射血分数（LVEF）较术前增加（68．5％±4．0％vs ．62．0％±4．5％，P=0．032）。随访63例，随访率87．5％（63／72），随访时间1～15年。随访期间死亡4例，远期再次手术1例，远期生存率88．3％，手术成功率86．0％。结论在复杂先天性心脏病的治疗中，改良Fontan手术有良好的疗效；同时根据具体解剖结构可选择不同的手术方式。     

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。     

Aortic allografts: reconstruction of right ventricle-pulmonary artery continuity

The use of aortic allografts in the repair of congenital cardiac lesions has increased as a result of both the advent of cryopreservation and the effects of increased donor availability secondary to infant transplantation. During the period 1986 through 1987, 38 cryopreserved aortic allografts were placed for right ventricle-pulmonary artery discontinuity. Size of the allografts ranged from 11 to 26 mm (mean size, 19 mm), and age of the patients ranged from 6 weeks to 26 years (mean age, 5 years). Twenty-one patients had primary placement of aortic allografts, and 17 underwent replacement of previous conduits. There were 5 hospital deaths (13%) overall, only 1 among the 10 patients younger than 6 months of age with truncus arteriosus, and none among the 17 patients having conduit replacement. A large conduit could be placed with a low incidence (10.5%) of postoperative hemorrhage related to the conduit. The aortic allograft is our conduit of choice for both conduit replacement and primary repair of right ventricle-pulmonary artery discontinuity.     

Ascending Aortic Extension for Right Pulmonary Artery Stenosis Associated With Ventricular-to-Pulmonary Artery Conduit Replacement

A bstract Background : Ventricular-to-pulmonary artery conduits in growing patients with congenital heart disease will require replacement from time to time due to somatic growth, neointimal hyperplasia, and pulmonary artery stenosis. The purpose of this article is to review our experience with ascending aortic extension for significant long-segment pulmonary artery stenosis in patients undergoing reoperation for right ventricular-to-pulmonary artery conduit replacement. Methods : From 1989 to 1997, 8 patients had aortic transection, right pulmonary artery augmentation arterioplasty, and aortic interposition graft (Hemashield in 7 and Gore-tex in 1) in association with right ventricular-to-pulmonary artery conduit replacement in 7 patients and completion Fontan operation in 1 patient. Aortic cross-clamp time was 90 ± 34 minutes, and the cardiopulmonary bypass time was 205 ± 37 minutes. Results : All patients survived. In those 7 patients who had conduit replacement, the RV/LV ratio declined from 0.78 ± 0.15 to 0.45 ±; 0.05 postoperatively (P < 0.05). Average length of stay was 8.9 ± 7.2 days. Follow-up range is 18 months to 8 years (mean 4 years). Two complications included cardiac transplantation for pre-existing poor left ventricular function and accelerated conduit stenosis leading to conduit re-replacement. Conclusion : Ascending aortic extension facilitates long-segment pulmonary artery augmentation arterioplasty and enlarges the retroaortic space, preventing future compression restenosis.     

Reconstruction of right ventricular outflow tract with a valved conduit in 75 cases of congenital heart disease.

Reconstruction of the right ventricular outflow tract with an aortic homograft conduit was performed in 75 patients from 1966 to 1974. The types of congenital heart disease were as follows: pulmonary atresia, 35 cases; severe tetralogy of Fallot, 22 cases; truncus arteriosus, 6 cases; transposition of the great arteries (TGA), 3 cases; single ventricle, 2 cases; and tricuspid atresia, 7 cases. Ninety per cent of the patients had one or more previous shunts, and this was a factor affecting the mortality rate. Other factors included age, pulmonary vascular resistance, surgical anatomy, and technical problems such as bleeding, prolonged bypass, coronary artery injury, and compression of the conduit by the sternum. Our present approach is to avoid shunts, define the anatomy precisely by angiography, and to attempt total correction when severe hypoxia or effort intolerance occurs or before increased pulmonary vascular resistance develops.     

Aortic valve homografts in the surgical treatment of complex cardiac malformations

From April of 1968 to March of 1983, the surgical treatment of complex congenital cardiac malformations requiring an extracardiac conduit for their correction was performed with aortic valve homografts or aortic valved homograft conduits sterilized and preserved in our hospital. Our experience concerns 93 patients in whom a total of 103 aortic valve homografts were implanted. Ages of the patients ranged from 7 months to 36 years (mean 11.6 years). The aortic valve homografts were used from the right atrium to the pulmonary arteries or right ventricle (right atrium-dependent conduit), from the venous ventricle to the pulmonary arteries (ventricle-dependent conduit), or in the pulmonary orifice and in the superior and/or inferior venae cavae. There were 25 early and nine late deaths (36.5%), none of them related to the aortic valve homograft. The clinical follow-up of the 59 survivors (1 month to 15 years, mean 4.3 years) evidenced neither dysfunction of the aortic valve homograft nor thromboembolism or hemolysis; 93% of the patients are in New York Heart Association Class I or II. Control cardiac catheterization in 53 patients evidenced a pressure gradient in only 14 ventricle-dependent conduits. In seven patients with serial control catheterizations after 5 to 10 years, the pressure gradient had not increased.     

自体肺动脉瓣移植术治疗先天性主动脉瓣病变

目的　总结自体肺动脉瓣移植手术 (Ross手术 )治疗先天性主动脉瓣病变的疗效。方法　自 1994年 10月至 2 0 0 3年 11月 ,共收治 2 0例先天性主动脉瓣病变患者行Ross手术治疗 ,其中男 15例 ,女 5例 ,平均年龄 2 5岁 ;术前诊断 :主动脉瓣二瓣畸形 12例 ,主动脉瓣叶脱垂 5例 ,瓣叶发育不良 3例 ,合并亚急性细菌性心内膜炎 4例 ,合并室间隔缺损 2例。术前超声心动图检查 (UCG)示所有患者均存在主动脉瓣狭窄或 /并关闭不全 (中重度 )。左心室舒张末内径 (LVDD) (6 0 5 1±11 87)mm ,主动脉瓣跨瓣压差 (2 7 0 4± 6 80 )mmHg。心功能 (NYHA分级 )Ⅱ级 17例 ,Ⅲ级 3例。所有病例均在全麻体外循环中度低温下进行 ,手术分三步进行 :(1)采取自体肺动脉瓣 ;(2 )切除病变的主动脉瓣并移植自体肺动脉瓣于主动脉位 ;(3)利用同种动脉瓣重建右心室流出道。结果　全组患者无手术死亡 ;左心室舒张末内径明显缩小 ,为 (46 38± 9 17)mm (t=3 4 0 0 7,P =0 0 0 0 8) ,术后主动脉跨瓣压差降至正常范围 (6 80± 0 19)mmHg。术后随访 3个月至 9年 ,所有患者的主动脉瓣、肺动脉瓣结构及功能正常。结论　自体肺动脉瓣移植手术是一种临床疗效好的治疗先天性主动脉瓣病变的手术方法 ,近中期效果良好。     

De BakeyⅠ型主动脉夹层的外科治疗

目的总结68例De BakeyⅠ型主动脉夹层的外科治疗经验,探讨手术方式和脑保护方案,以提高手术疗效。方法回顾性分析2004年5月至2010年4月沈阳军区总医院收治的68例De BakeyⅠ型主动脉夹层患者的临床资料,男45例,女23例;年龄29~72岁（44.5±17.2岁）。急性主动脉夹层（发病〈2周）57例,慢性主动脉夹层11例;主动脉夹层破口位于升主动脉45例,主动脉弓12例,主动脉弓降部11例。急诊手术35例,择期或限期手术33例。手术采用深低温停循环＋选择性顺行性脑灌注或右上腔静脉逆行灌注脑保护。行主动脉全弓置换＋支架象鼻手术25例,Bentall手术＋主动脉全弓置换＋支架象鼻手术16例,单纯右侧主动脉半弓置换术15例,主动脉全弓置换术7例,右侧主动脉半弓置换＋支架象鼻手术3例,升主动脉带瓣管道＋Cabrol手术＋主动脉全弓置换＋支架象鼻手术2例。结果手术死亡5例（急性夹层4例、慢性夹层1例）,手术死亡率7.4%（5/68）;死于术中吻合口顽固性渗血1例,术后低心排血量综合征和恶性心律失常2例,并发急性肾功能衰竭1例,脑部并发症1例。围术期出现精神异常5例,心包积液2例,声音嘶哑6例,切口愈合不良1例,均经相应的治疗治愈。随访60例（95.2%,60/63）,随访时间2个月~6年,失访3例。随访期间猝死1例,1例出现心包积液,经治疗好转;其余患者生活质量良好,心功能均明显改善,心功能分级（NYHA）I级54例,Ⅱ级5例。结论对De BakeyⅠ型主动脉夹层患者应积极手术治疗,术中根据不同病情选择最佳的手术方式和合适的脑保护方法,术后及时处理并发症,可取得良好的手术效果。     

同种带瓣管道应用的远期效果

目的　评价同种带瓣管道应用后的远期效果。方法　1988年1月至1997年12月的10年间应用同种带管道瓣治疗165例多种复杂先天性心脏病(先心病)，其中主动脉带瓣管道136例、肺动脉带瓣管道29例。病人年龄1.3～22.0岁，平均7.6岁。体重9.5～58.0kg，平均22.0kg。管道直径17～22mm，平均19mm。结果　术后早期（30d内）死亡40例，死亡率为24%。术后随访10个月～12.1年，平均(37.0±8.6)个月。病人术后5、10年生存率分别为94.6%、83.8%，带瓣管道完好率分别为83%、58%。结论　应用同种带瓣管道治疗复杂先心病，远期疗效比较满意。     

心外管道全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的总结心外管道全腔静脉-肺动脉连接术（ECTCPC）治疗复杂先天性心脏病的临床经验,并就手术适应证、手术方法及手术效果进行讨论。方法1998年6月至2006年12月,68例先天性心脏复杂畸形的患者接受了ECTCPC。包括单心室伴有大动脉转位、肺动脉瓣狭窄45例：三尖瓣闭锁、右心室发育不良19例;三尖瓣下移畸形并右心室发育不良4例。其中合并永存左上腔静脉6例,双向Glenn术后行全腔静脉-肺动脉连接术18例（其中包括单心室、肺动脉闭锁、左肺动脉狭窄双向Glenn术后1例）,单心房、单心室、心上型完全性肺静脉异位引流、多发粗大体肺侧支1例。全组采用体外循环下手术共57例,其中8例患者因需要矫正心内畸形在主动脉阻断下手术外,其余49例均在全身麻醉并行体外循环心脏跳动下进行;非体外循环下手术11例。结果术后早期死亡2例,病死率为2．9％。其中1例死于术后反复肺内出血,1例死于上消化道反复大出血。66例痊愈出院,术后随访1个月至8年,无晚期死亡。所有患者症状消失,血氧饱和度90％～96％,恢复良好。结论ECTCPC方法简便易行,术后并发症较少,效果好,较其他术式有较大优点。     

Right ventricular aneurysm following modified Norwood-Sano operation for hypoplastic left heart syndrome

Hypoplastic left heart syndrome is a rare congenital heart defect characterized by underdevelopment of left-sided heart structures, including the aortic arch. The contemporary surgical management of this anomaly includes the Norwood procedure and provision of pulmonary blood flow by either a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery (RV-PA) conduit, commonly referred to as the Sano shunt. We report on an unusual complication of the Sano shunt, that of a giant right ventricular pseudoaneurysm occurring at the shunt insertion site.     

Mechanical Aortic Valve Replacement in Children and Adolescents After Previous Repair of Congenital Heart Disease

Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown‐ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart defects. Between July 2002 and November 2008, 15 (10 male and 5 female) consecutive patients (mean age 14.5 ± 10.5 years) underwent mechanical AVR. Hemodynamic indications for AVR were aortic stenosis in four (27%), aortic insufficiency in eight (53%), and mixed disease in three (20%) after previous repair of congenital heart defects. All patients had undergone one or more previous cardiovascular operations due to any congenital heart disease. Concomitant cardiac procedures were performed in all of them. In addition to AVR, in two patients, a mitral valve exchange was performed. One patient received a right ventricle‐pulmonary artery conduit replacement as concomitant procedure. The mean size of implanted valves was 23 mm (range 17–29 mm). There were neither early deaths nor late mortality until December 2008. Reoperations were necessary in five (33%) and included implantation of a permanent pacemaker due to complete atrioventricular block in two (15%), mitral valve replacement with a mechanical prosthesis due to moderate to severe mitral regurgitation in one (7%), aortocoronary bypass grafting due to stenosis of a coronary artery in one (7%), and in one (7%), a redo subaortic stenosis resection was performed because of a secondary subaortic stenosis. At the latest clinical evaluation, all patients were in good clinical condition without a pathological increased gradient across the aortic valve prosthesis or paravalvular leakage in echocardiography. Mechanical AVR has excellent results in patients after previous repair of congenital heart defects in childhood, even in combination with complex concomitant procedures. Previous operations do not significantly affect postoperative outcome.