2例半旋转动脉干调转术治疗完全大动脉转位合并室间隔缺损和肺动脉狭窄的术后护理

半旋转动脉干调转术是治疗完全大动脉转位合并室间隔缺损和肺动脉狭窄的一种新方法。以往治疗完全大动脉转位合并室间隔缺损和肺动脉狭窄方法是Rastelli或Lecompte手术。Rastelli术后易存在心内隧道狭窄,发生左室流出道梗阻,同种异体心外管道易远期钙化,使右室流出道狭窄和梗阻,Lecompte手术还易存在肺动脉关闭不全。     

完全性大动脉转位合并室间隔缺损、肺动脉狭窄患者行Rastelli术后早期处理(附2例报告)

目的:总结Rastelli术[1]治疗合并心室间隔缺损和肺动脉狭窄的完全性大动脉转位术后早期处理经验。方法:2例完全性大动脉转位合并心室间隔缺损和肺动脉狭窄,年龄15岁和17岁。2例均在中低温(22℃~25℃)、低流量体外循环下,应用Rastelli术对其进行解剖矫正。术后严密监测血流动力学指标,及时补充胶体溶液,维持适当的过度通气,应用正性肌力药物及降低肺血管阻力药物。结果:术后无死亡。术后早期并发症包括1例发生低心排血量综合征,房性心动过速1例,低氧血症1例,二次开胸止血1例。结论:术后维持足量的前负荷,充分镇静镇痛,降低氧耗,有效降低肺循环阻力,及时纠正低心排血量综合征、低氧血症、心律失常等并发症,可有效提高手术成功率。     

手术治疗矫正型大动脉转位六例报告

目的；探讨手术治疗矫正型大动脉转位的临床效果．合并畸形的矫治原则及手术方法。方法：6例矫正型大动脉转位患者室间隔缺损均经右心房-二尖瓣径路补片修补，3例肺动脉流出道狭窄分别行瓣交界切开(2例)和左心室-肺动脉心外管道重建术(1例)。结果：6例患者均治愈出院，无完全性房室传导殂滞及低心排血量综合征发生。结论：矫正型大动脉转位解剖复杂，应根据不同的合并畸形选择手术方式，避免房室传导阻滞的发生和最大限度保护心功能。     

1例右心室至肺动脉带瓣管道连接术治疗完全性大动脉转位患者的护理

完全性大动脉转位（TGA）是复杂型先天性心脏病的一种,主要特征是心室与大动脉连接畸形,从而造成2个独立的循环。该病患儿多伴有发绀,并进行性加重。右心室至肺动脉带瓣管道连接术（Rastelli术）是用于治疗TGA合并室间隔缺损（VSD）和左心室流出道梗阻的常用手术方法。     

一例Rastelli术后气道大量出血的抢救与护理

右室至肺动脉带瓣管道架接术(Rastelli)适用于大动脉错位同时伴有大型室间隔缺损和肺动脉狭窄的病例,或用于做过肺动脉环缩术的大动脉错位病例并有解除环缩后肺动脉存在瘢痕不能达到满意松解者[1].     

Rastelli术治疗合并室间隔缺损、肺动脉狭窄、完全性大动脉转位的麻醉管理

目的:采用Rastelli术治疗合并室间隔缺损、肺动脉狭窄、完全性大动脉转位病人,探讨围手术期麻醉管理特点。方法:选用大剂量芬太尼静脉复合麻醉方案,在深低温、低流量体外循环下实施Rastelli手术并进行多指标监测和临床观察。结果:麻醉经过平稳,围手术期患者生命体征稳定。结论:选择恰当的麻醉方案,有效的心脏保护,保持酸碱平衡和水、电解质稳定是成功进行麻醉管理的必要条件。     

改良Nikaidoh术治疗大动脉转位1例术后护理

近年来,我科为1例完全性大动脉转位(D-TGA)合并房间隔缺损(ASD)、室间隔缺损(VSD)和肺动脉狭窄(PS)患儿成功实施了改良Nikaidoh术,效果满意。现报告如下。1临床资料患儿女,4岁,体重17 kg,因活动后气喘,外院心脏超声提示"先天性心脏病、完全性大动脉转位、房间隔缺损、室间隔缺损、动脉导管未闭"入院。入院体检:口唇及甲床发绀明显,SaO276%,杵状指,心前区闻及收缩期杂音,心律不齐。心脏     

镜像右位心及合并畸形彩色多普勒超声心动图特征和规律性研究

目的 探寻镜像右位心及合并畸形彩色多普勒超声心动图(CDE)特征和规律性.方法 应用CDE检查100例镜像右位心及合并畸形,寻找CDE特征和规律性,95例经心导管对照,76例手术证实.结果 根据CDE特征对92例做出正确诊断,诊断准确率92%,误诊8例.本组镜像右位心及合并畸形CDE特征和规律性明显:(1)镜像右位心合并12种先天性心脏病,其中ILL型右心室双出口(27%)最多,IDD型矫正性大动脉转位(17%)、法洛四联症(16%)和室间隔缺损(12%)较少,心室双入口(7%)、三尖瓣闭锁(5%)、两腔心(5%)、ILL型完全性大动脉转位(4%)、二尖瓣闭锁(2%)、孤立性右室流出道狭窄(2%)、完全性肺静脉异位引流(2%)、孤立性二尖瓣前叶裂隙(1%)更少.(2)镜像右位心无论合并那种先天性心脏病均为心房反位.(3)镜像右位心合并右心室双出口和完全性大动脉转位为左转位,合并矫正性大动脉转位为右转位.(4)镜像右位心合并肺动脉狭窄(80%)多见,肺动脉压正常(12%)和肺动脉高压(8%)少见.(5)合并房间隔缺损和室间隔缺损,彩色多普勒血流显像显示过房间隔或室间隔左向右或双向五彩镶嵌分流束血流信号.结论 镜像右位心及合并畸形CDE特征和规律性明显,应用CDE对镜像右位心及合并畸形可做出正确诊断,但检查者必须熟练掌握本病解剖.在探查手法上与正常位心脏明显不同,检查前阅读X线正位胸片对CDE正确诊断有帮助,并可提高检查效率.     

产前超声诊断矫正型大动脉转位

目的 探讨产前超声对矫正型大动脉转位的诊断价值.方法 回顾性分析10例在我院诊断的胎儿矫正型大动脉转位的超声病例资料.利用二维超声心动图获取四腔心切面、主动脉根部短轴切面、主动脉弓切面、双房切面及左、右室流出道切面,并采用心脏三节段分析法进行分析.在四腔心切面上判定心房位置及房-室连接有无异常,在左、右室流出道切面的基础上追踪显示2条大动脉的走行,判定心室-大动脉连接有无异常.结果 10例矫正型大动脉转位的胎儿中,经尸检或生后超声心动图证实有2例为单纯矫正型大动脉转位,4例为矫正型大动脉转位合并室间隔缺损,2例为矫正型大动脉转位合并室间隔缺损及肺动脉狭窄,1例为矫正型大动脉转位合并室间隔缺损、三尖瓣下移畸形,1例为矫正型大动脉转位合并主动脉缩窄.结论 四腔心切面及双流出道切面是诊断矫正型大动脉转位的主要切面.采用三节段分析法及连续追踪法是分析本病的重要方法.     

改良Nikaidoh术患者的护理

有研究[1]报道一种新的手术方式(Nikaidoh术),即通过完整游离主动脉根部移植于左室流出道上,将肺动脉移植于右室流出道出口,修补室间隔缺损后彻底解剖根治大动脉转位.近年来,Nikaidoh术用于治疗完全大动脉转位(TGA)或右室双出口(DORV)合并室间隔缺损(VSD)、肺动脉狭窄(PS)已显露出颇佳的手术效果,术后的形态学上更接近于正常解剖[2].     

Surgical repair of cyanotic cardiac defects in young adults. The Fontan procedure and use of the extracardiac valved conduit

Improvement in the health status of young adults with complex cyanotic congenital cardiac defects has been realized with recent advances in cardiovascular surgical techniques. The Fontan procedure and its modifications have allowed physiologic correction of tricuspid atresia by separating the pulmonary and systemic circulations and creating a direct connection from right atrium to pulmonary artery. The extracardiac valved conduit has been successfully used in the repair of pulmonary atresia with ventricular septal defect, and the triad of transposition of the great vessels, ventricular septal defect, and pulmonary stenosis. Nursing implications for the care of these patients in the postoperative period and their long-term management has been addressed. Advances in surgical techniques have brought these anomalies into the realm of surgical repair and have increased the life expectancy of these patients, and thus more patients with these cardiac defects will be seen in adult cardiology settings.     

Percutaneous implantation of pulmonary valves

Percutaneous implantation of valves is evolving as the most exciting innovation in transcatheter therapeutics. Valve repairs and replacements form an important indication of re-operations after congenital heart operations. Right ventricular outflow tract surgery is common after repair of conditions such as pulmonary atresia with Tetralogy of Fallot, common arterial trunk and Rastelli operation for transposition of great arteries, ventricular septal defect with pulmonary stenosis. Conduit survival is improving, nevertheless, re-operations are common. Although the re-operations can be performed at low risk, they still involve morbidity and mortality. Percutaneous implantation of valves is emerging as an alternative option for these group of patients. Our experience in percutaneous implantation of pulmonary valves has yielded encouraging results. For incompetence, the results are excellent. Evolving technology is making it applicable in wider clinical situations. Similar technology could also be applied to implantation of valves in the aortic position.     

Percutaneous implantation of pulmonary valves

Percutaneous implantation of valves is evolving as the most exciting innovation in transcatheter therapeutics. Valve repairs and replacements form an important indication of re-operations after congenital heart operations. Right ventricular outflow tract surgery is common after repair of conditions such as pulmonary atresia with Tetralogy of Fallot, common arterial trunk and Rastelli operation for transposition of great arteries, ventricular septal defect with pulmonary stenosis. Conduit survival is improving, nevertheless, re-operations are common. Although the re-operations can be performed at low risk, they still involve morbidity and mortality. Percutaneous implantation of valves is emerging as an alternative option for these group of patients. Our experience in percutaneous implantation of pulmonary valves has yielded encouraging results. For incompetence, the results are excellent. Evolving technology is making it applicable in wider clinical situations. Similar technology could also be applied to implantation of valves in the aortic position.     

超声心动图对婴儿完全性大动脉转位的诊断价值

目的 探讨超声心动图对婴儿完全性大动脉转位的诊断价值。方法 将13例婴儿完全性大动脉转位超声心动图特征与手术结果进行对照分析。结果 室间隔完整型大动脉转位5例(合并房间隔缺损、动脉导管未闭2例，单纯合并动脉导管未闭2例，单纯合并房间隔缺损1例)。大动脉转位合并室间隔缺损8例(其中合并室间隔缺损、房间隔缺损、动脉导管未闭3例，房、室间隔缺损1例，单纯合并室间隔缺损3例，1例合并室间隔缺损、右冠状动脉双支)。13例中，12例术前超声诊断与手术结果完全一致，1例冠状动脉畸形超声未检出。结论 超声心动图可早期明确诊断婴儿完全性大动脉转位及其分型，为临床提供准确信息，对婴儿早期进行大动脉转位调转术的筛选及术后随访有重要临床价值。     

右心室双出口的外科治疗

目的 探讨右心室双出口的手术治疗效果。方法 对31例右心室双出口患者手术的临床资料分析。主动脉辨下室间隔缺损(VSD)型23例，肺动脉辨下VSD型2例，两大动脉瓣下VSD型5例，远离两大动脉VSD型1例。有肺动脉狭窄者21倒，肺动脉高压者7例。采用左心室-主动脉心内隧道连接26例，左心室-主动脉心内隧道并右心室．肺动脉心外同种带瓣主动脉管道连接2例，全腔静脉-肺动脉连接1例，双向Glenn手术2例。右心室流出道重建采用直接缝合1例，自体心包片加宽9例，跨瓣补片扩大至左右肺动脉分又或一侧肺动脉14例，同种带瓣主动脉血管片连接2例。结果 手术死亡3例。手术非致死并发症9倒治愈出院。随访无远期死亡，效果良好。结论 根据右心室双出口的解剖特点和不同类型，选择不同的根治或姑息手术方式，保证重建的左、右心室流出道畅通，是手术成功的关键技术。     

体外受精-胚胎移植胎儿先天性心脏病的超声表现及分类特征

目的探讨体外受精-胚胎移植（IVF-ET）妊娠的心脏病胎儿超声心动图特征及分类情况的特殊性。方法对2011年1月至2013年6月孕18-24周在南京军区福州总医院行胎儿超声心动图检查的IVF-ET妊娠孕妇1129例,分析IVF-ET妊娠胎儿先天性心脏病超声表现及分类特征,并对部分检出胎儿心脏病的孕妇选择性进行羊水穿刺术,追踪所有入选孕妇的妊娠过程及胎儿临床结局。结果1129例接受IVF-ET的孕妇中单胎妊娠953例、双胎妊娠176例,共检测胎儿1305个,其中超声心动图诊断胎儿心脏畸形15例（1.1%,15/1305）,临床确诊15例。IVF-ET心脏畸形胎儿超声心动图表现：（1）法洛四联症5例,超声心动图示胎儿主动脉增宽伴骑跨,室间隔缺损,肺动脉狭窄,右心室肥厚。（2）左心发育不良综合征1例,超声心动图示胎儿左心明显缩小,二尖瓣闭锁,右心扩大,肺动脉扩张。（3）完全型房室间隔缺损1例,超声心动图示胎儿心内膜正常十字交叉结构消失,房室共瓣。（4）大动脉共干Ⅰ型1例,超声心动图示胎儿心底部仅见1条大血管,短小的肺动脉主干自大动脉发出,并见室间隔缺损。（5）主动脉狭窄及缩窄各1例,超声心动图示1例胎儿主动脉瓣增厚,升主动脉增宽;1例胎儿主动脉峡部狭窄伴狭窄后扩张。（6）肺动脉狭窄2例,超声心动图示胎儿肺动脉瓣狭窄伴狭窄后肺动脉扩张。（7）室间隔缺损3例,超声心动图示胎儿室间隔连续中断,断端边缘回声增强。超声漏诊胎儿室间隔小缺损1例,为出生后超声心动图检查发现。结论 IVF-ET妊娠胎儿较自然妊娠胎儿先天性心脏病的发生率高,IVF-ET妊娠胎儿与自然妊娠胎儿先天性心脏病的超声心动图表现相同。其中以法洛四联症和室间隔缺损最多见。对IVF-ET妊娠胎儿行超声心动图检查能全面系统评价IVF-ET妊娠胎儿心脏异常的情况,有重要临床指导意义。     

右房同形异构及其伴随的心脏畸形的超声心动图研究

目的 探讨右房同形异构及伴随病变的超声心动图诊断方法，以提高对右房同形异构的认识，为制定手术方案提供详细的资料。方法 收集超声心动图资料完整并经证实的10例患者，对其超声心动图顺序节段分析的结果进行分析。结果 本组右房同形异构患者最常见的心脏畸形组合为肺静脉异位引流，左位上腔静脉，完全型房室隔缺损，共同房室瓣，心室大动脉连接异常(包括右型大动脉转位，右室双出口)，肺动脉狭窄或闭锁。未见肺静脉引流正常，肺动脉系统正常及心室—大动脉连接一致；未见左室流出道狭窄及主动脉梗阻。结论 右房同形异构为复杂的疾病综合症，有其特征性心脏伴随病变，超声心动图可为外科治疗提供重要的诊断。     

大动脉调转术治疗年龄6个月以上重度肺动脉高压患者疗效分析

目的评价大动脉调转术治疗年龄6个月以上重度肺动脉高压伴室间隔缺损、大动脉转位和Taussig-Bing畸形患者的中期疗效。方法对86例年龄6个月以上的重度肺动脉高压伴室间隔缺损、大动脉转位和Taussig-Bing畸形患者行大动脉调转手术,观察术后病死率、并发症发生率、术后肺动脉压力等,分析手术死亡、术后持续性肺动脉高压的影响因素。结果本组住院死亡6例（6.98%）,72例完成随访,死亡2例;存活者心功能NYHAⅡ级2例（2.86%）,心功能NYHAⅠ级68例（97.14%）;术后肺动脉压（30.5±13.0）mm Hg明显低于术前（64.9±13.0）mm Hg,经皮血氧饱和度（99.7±0.7）%明显高于术前（72.0±15.0）%（P〈0.05）;多因素回归分析显示,术后肺动脉高压、一氧化氮吸入、血浆胶体渗透压监测是手术死亡的独立预测因子（OR=1.236,95%CI：1.080-1.415,P=0.002;OR=0.016,95%CI：0.001-0.345,P=0.008;OR=0.070,95%CI：0.006-0.829,P=0.035）,手术年龄与持续性肺动脉高压独立相关（OR=1.283,95%CI：1.073~1.536,P=0.006）。结论大动脉调转术治疗大龄重度肺动脉高压伴室间隔缺损、大动脉转位和Taussig-Bing畸形患者中期效果良好。     

Tetralogy of Fallot with anomalous origin of left pulmonary artery.

Between 1971 and 1990, 7 patients of tetralogy of Fallot with anomalous origin of left pulmonary artery underwent intracardiac repairs at Tohoku University Hospital. They were 2 males and 5 females with ages ranging from 4 to 26 years old. The right pulmonary artery connected to right ventricle in all cases, whereas no communications between right ventricle and the left pulmonary artery were found. The left pulmonary artery directly originated from the ascending aorta in 2 patients (group I) and connected to the ductus arteriosus in 5 patients (group II and III). In 2 patients (group II), the left pulmonary artery was separated from the pulmonary arterial trunk by the intraluminal membrane, receiving blood supply through the ductus. In the remaining 3 patients (group III), there were no continuations between both pulmonary arteries. At the correction, communication between the left pulmonary artery and the pulmonary arterial trunk could be reconstructed in groups I and II. However, it was not possible in group III, because the ductus arteriosus and the left pulmonary artery had already been occluded before the intracardiac repair. In group III, ventricular septal defect was closed using a one-way valved patch or a perforated patch to decrease supersystemic right ventricular pressure. Postoperative right ventricular aortic pressure ratio was between 0.5 and 0.8 in groups I and II, and between 0.8 and 1.0 in group III. Three patients (one in each group) died after the operation. Severe pulmonary vascular obstructive disease was found in the left lung of group I.(ABSTRACT TRUNCATED AT 250 WORDS)