体外循环下小儿肌部室间隔缺损的镶嵌治疗

目的 探讨镶嵌模式(hybrid procedure)治疗小儿肌部室间隔缺损(Mvsd)的手术方法及临床应用.方法 2006年1月至2010年6月,在体外循环心内直视手术下采用手术及封堵相结合的镶嵌技术矫治小儿Mvsd 45例,其中男20例,女25例;年龄52天～12岁;体重3～32 kg.7例为单个Mvsd,38例为多发性VSD.同时合并大血管错位(D-TGA)1例、法洛四联症(TOF)2例、肺动脉狭窄(PS)3例、动脉导管未闭(PDA)6例、房间隔缺损(ASD)6例、主动脉缩窄1例.均在心脏停跳后直视下将导引钢丝经三尖瓣孔自心脏右室面穿过VSD至左室面,直视下置入导引器,然后送入封堵器,完成Mvsd封堵.多发性VSD 38例,予自体心包片修补膜周部等较大的VSD,心内其他畸形同期完成矫治.结果 42例置入单枚封堵器(直径4～10 mm)、3例置入双枚封堵器(直径4～7 mm).手术经过顺利,术前左室射血分数(EF)均在正常范围,术后1天小于8月龄组EF均值低于正常,大于8月龄组EF正常,两者差异有统计学意义.术后常规每天给予5 mg/kg肠溶阿司匹林3～6个月.术后随访超声检查示封堵器位置无偏移,无残余分流,无二尖瓣、主动脉瓣反流、Ⅲ度传导阻滞及新发心律失常等.术后因重症感染放弃治疗1例,无远期死亡病例.结论 体外循环下镶嵌技术治疗小儿 Mvsd明显降低了围手术期并发症及病死率,简化了手术过程,降低了手术风险,是一种安全、有效的方法.

Abstract：

Objective To summarize the technique and clinical experience of hybrid procedure under cardiopulmonary bypass (CPB) in children with muscular ventricular septal defect (mVSD). Methods From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedure with CPB. mVSDs were closed with devices under direct vision in 45 cases. Of them, there were 20 males and 25 females. They ranged from 52 days to 12 years [mean (2.05 ±2.48) year] in age and from 3 to 30 kg [(11.93 ±7.70)kg] in body weight. Preoperatively, most of children were highly susceptible to respiratory tract infections. The hybrid approach was used in all patients with CPB under the guidance of transesophageal echocardiography (TEE). The diameter of mVSDs ranged from 2 to 7 mm under TEE. Of 45 cases, 40 patients had increased rates of pulmonary blood flow. 29 patients had left axis deviation and 12 cases had sinus arrhythmia on electrocardiography (ECG). 19 had other congenital heart lesions, including transposition of great arteries in 1 case, tetralogy of Fallot in 2, pulmonary artery stenosis in 3, patent ductus arteriosus in 6, atrial septal defects in 6) and aorta coactation in 1. The quantity of VSDs were from 1 to 7 (single, in 7; two, in 24 case; three, in 8 case; four, in 5 case and seven, in lease. 37 patients were combined with pulmonary hypertension in our cohort. 38 patients with another large VSD and 19 with other congenital heart lesions were required surgical repair at sometime. Results The hybrid procedures were undertaken in all 45 cases of this cohort. All cases were successful and no deaths occurred during operation. A total of 48 devices were implanted in 45 patients, including single devices in 42 cases (device size ranged from 4 to 10 mm) and two devices in 3 cases (device size ranged from 4 -7 mm). The average time on CPB was (58.28 ±20.70) min , while aortic crossclamp time was(34. 94 ± 14.75) min. In addition, the time on mechanical ventilation postoperatively ranged from 2 hours to 6 days. Compared to the older children, 20 infante aged less than 8 monhad a significant difference in cardiac function in the early postoperative period. One infant was given up treatment because of serious infection. Anather cases recovered with the use of supportive treatment, such as using vasoactive agents, digoxin, inhaling nitric oxide, diuresis, and so on. The enteric-coated aspirin was given at dose of 5 mg ? kg -1. day -1 for a period of 3 to 6 months as usual postoperatively. All patients attended follow-up at 1 week, 1 month, 3 months, 6 months, 1 year and 2 years post-procedure. No major complications were encountered during this period. All cases were no instance of migration of any of the devices, residual shunt, aortic regurgitation, atrioventricular valve dysfunction, Ⅲo atrial-ventricular conduction block, new arrhythmia, and so on. There are no death in long-term follow-up. Conclusion Hybrid procedure is safe and effective for the closure of congenital heart defects in children.     

经胸膜外微创镶嵌术治疗小儿房间隔缺损16例报告

目的探讨经胸膜外微创镶嵌术治疗小儿房间隔缺损的治疗经验和早期疗效。方法全麻气管插管后置入食道超声探头,行右前胸第4肋间胸骨旁1.5～2cm微创小切口经胸膜外镶嵌房间隔缺损手术16例。术后不置胸腔引流管,术后第一天X线胸片检查。结果16例患儿均一次镶嵌成功。术后无胸腔积液、肺部并发症、残余分流及瓣膜功能障碍。术后住院时间2～3天。术后第二天下床活动,开始口服肠溶阿斯匹林5mg/(kg·d),分两次,抗凝6个月。随访3～6个月,恢复顺利,无残余分流和栓塞。结论经胸膜外微创行房间隔缺损镶嵌术是一种安全、有效的微创外科治疗方法。     

超声心动图诊断胸外心脏合并十字交叉心1例

病例 男,9岁,因反复胸闷气急7余年、加重半月就诊,既往无手术、外伤史.查体:心脏位于胸腔外,伴有唇裂及无脐综合征.经心脏超声检查,结果如下:心房正位,心室右襻,右左室呈上下排列,右心室位于左上方,通过三尖瓣与右心房相连;左心室位于右下方,通过二尖瓣与左心房相连(图1),主动脉、肺动脉呈右左位准平行走形,前者径线正常,发自位于左上方的右心室,后者径线增宽,发自位于右下方的左心室,室间隔呈水平位,于肺动脉瓣下探及18 mm的回声中断,缺损距离主动脉瓣22 mm,房间隔连续,右位主动脉弓未见狭窄.     

经食管超声心动图在体外循环下小儿肌部室间隔缺损镶嵌治疗中的应用

目的 探讨经食管超声心动图(TEE)在体外循环(CPB)下小儿肌部室间隔缺损(mVSD)镶嵌治疗中的应用价值.方法 经TEE指导、监测共完成49例CPB下mVSD的镶嵌治疗,其中9例为单纯mVSD,40例为mVSD合并心内其他畸形.心脏停跳前TEE详细观察mVSD的部位、大小、数量、范围及与周边的解剖关系,提示术者选择合适的封堵器,心脏复跳后TEE评价其疗效.结果 49例行镶嵌治疗的患儿中,44例置入单枚封堵器,5例置入双枚封堵器.术后TEE均显示封堵器位置无偏移,2例出现细小残余分流,无加重二尖瓣、主动脉瓣反流等并发症.术后1例患儿因重症感染放弃治疗,其余患儿随访6个月～2年,均见封堵器位置正常;除1例仍存在微量残余分流,其余患儿未出现残余分流及瓣膜反流等并发症.结论 经TEE指导、监测,体外循环下镶嵌治疗小儿肌部室间隔缺损可以缩短体外循环时间,降低外科手术创伤的并发症及风险性,提高手术成功率.     

超声心动图在Ⅳ型主⁃肺动脉间隔缺损外科微创封堵手术中的临床价值

目的：探讨经胸超声心动图（transthoracic echocardiography,TTE）及经食管超声心动图（transesophageal echocardiog?raphy,TEE）在非体外循环下经胸微创封堵Ⅳ型主?肺动脉间隔缺损（aortopulmonary septal defect,APSD）的应用价值。方法：回顾性分析9例利用TEE引导经胸微创封堵Ⅳ型APSD的患儿,选用膜部室间隔缺损（ventriculap septal defect,VSD）封堵器或房间隔缺损（atrial septal defect,ASD）封堵器。术前所有患儿均行TTE详细筛查,主要测量缺损大小、缺损上缘距右肺动脉起始部的距离、下缘距窦管交界部的距离、大动脉内径。胸骨正中切口,于大动脉表面缝置荷包,术中TEE引导下置入导丝鞘管、进行封堵器释放、评价即刻封堵效果。术后进行TEE随访。结果：9例术前TTE显示缺损上下缘距各相关结构均有一定距离、均无其他需要手术干预的心内畸形。TEE指导下9例微创封堵手术全部成功,术中即刻TEE观察封堵器与APSD上下缘组织结构的关系,9例半月瓣启闭均无影响,升主动脉及右肺动脉均无梗阻征象,2例封堵器边缘存在细小残余分流（分流束<1.5 mm）。所有患儿术后随访6个月~2年,均见封堵器位置正常,未出现严重并发症。上述2例残余分流分别于术后第1个月和第3个月复查消失。结论：在TEE指导和监测下行外科微创封堵治疗Ⅳ型APSD操作有效,安全性高,创伤小,近期疗效满意。     

超声检查在女孩特发性中枢性性早熟治疗中的应用

特发性中枢性性早熟(ICSP)是一种生长发育异常,表现为青春期特征提前出现,同时生长加速,由于骨骼生长加速,骨骺提前闭合,患者开始身材虽较同龄儿高,但成年后往往比正常人矮小,多见于女孩.近年来由于发病率较前提高,越来越引起家长关注[1].本文应用二维超声动态监测45例中枢性性早熟治疗前后子宫、卵巢的形态学变化,现报道如下.     

主动脉左室隧道的超声诊断价值及预后分析

目的 探讨主动脉左室隧道（ALVT）的超声诊断价值,并分析其图像特征,追踪患儿手术结果及预后情况.方法 对6例经彩色多普勒超声心动图（CDE）诊断为ALVT的患者进行回顾性分析,并将全部患着的声像图表现与心血管造影、手术结果、术后结果对比分析,并总结其诊断特点.结果 6例经超声心动图诊断为ALVT的患者与心血管造影、手术结果基本符合,ALVT图像特征明显,二维超声心动图胸骨旁左室长轴切面上清晰显示主动脉瓣周至左室流出道的隧道样结构,彩色多普勒血流显像（CDFI）显示收缩期血流信号从左室流出道经隧道进入主动脉,舒张期血流信号由主动脉经隧道进入左室,术后跟踪复查均闭合良好.结论 超声心动图诊断ALVT有效、快捷、无创,是诊断ALVT的首选方法,为临床诊断提供了准确可靠的参考依据,并注意与右冠状窦瘤破裂、冠状动脉瘘及主动脉瓣关闭不全等相鉴别.     

偏心型封堵器在毗邻主动脉瓣的室间隔缺损微创镶嵌术中的应用

目的 探讨偏心型封堵器在经胸微创镶嵌手术治疗毗邻主动脉瓣的室间隔缺损(VSD)中的临床应用价值.方法 用偏心型封堵器治疗VSD边缘距主动脉瓣均＜2mm的22例VSD患儿.经食道心脏超声(TEE)观察室间隔缺损大小、部位及与周边组织的解剖关系,选择合适的封堵器.结果 22例VSD微创镶嵌术全部成功.术后TEE显示封堵器与VSD边缘吻合紧密,其中3例封堵器边缘存在细小左向右分流(分流束＜1.5 mm),均于术后1个月内愈合.术后1个月、3个月、6个月随访,22例患儿均无封堵器移位.结论 微创镶嵌术使用偏心型封堵器治疗毗邻主动脉瓣的VSD损疗效确切.     

高频超声诊断小儿牵牛花综合征的临床价值

目的:探讨高频超声诊断小儿牵牛花综合征的临床价值,提高对牵牛花综合征的认识?方法:回顾性分析5例经高频超声检查诊断为牵牛花综合征患儿的超声表现,分析声像图特点,并与CT及MRI检查结果进行综合分析?结果:5例牵牛花综合征患儿均为单眼发病,超声均表现为球后视乳头沿视神经有杯状暗区与玻璃体相连,边缘及底部回声增强?其中合并小眼球1例,斜视2例,视网膜脱离3例,永存玻璃体动脉1例,白内障1例?结论:高频超声对小儿牵牛花综合征诊断准确率高,操作方便,声像图特征性强,并且不受屈光间质的限制,可作为该病的首选辅助检查?     

高频超声在发育性髋关节异常早期诊断及随访中的临床价值

目的:探讨Graf法髋关节高频超声检查技术在6月龄内婴幼儿发育性髋关节异常(developmental dysplasia of the hip,DDH)诊断中的临床应用价值?方法:对1 357例6月龄内临床疑似DDH的患儿行Graf法髋关节超声检查,通过观察双侧髋关节形态?发育及头臼匹配关系,测量髋关节α和β角度,并进行Graf分型诊断?结果按月龄分组(每1个月为一组)统计α和β角度值并进行分析?DDH阳性患儿经临床干预治疗后超声随访复查,4月龄以上且已出现骨化中心的患儿同时行X线检查?结果:1 357例受检婴幼儿,共计2 714个髋关节,超声检查发现正常髋关节2 584个(Ⅰ型,占95.21%),发育性髋关节异常者130个(Ⅱa~Ⅳ型,占4.79%)?其中45个发育欠成熟的髋关节(Ⅱa型)接受了超声复查的随访,41个恢复正常,4个发展为Ⅱb型;对后者与首次检查为异常(Ⅱb~Ⅳ型)的髋关节(共计89个),使用Pavlik吊带治疗,78个先后达到稳定复位;8个脱位髋经吊带治疗失败后,后期行X线确诊,手术开放复位加石膏固定;3个髋关节失访?结论:Graf法髋关节高频超声检查技术是一种安全?可靠的诊断方法,在婴幼儿DDH的早期确诊和治疗随访中具有积极的指导意义?