超声评估儿童右心室流出道重建围手术期右心收缩功能变化及临床价值探讨

目的对行右心室流出道重建术的圆锥动脉干畸形患儿围手术期右心总体及节段收缩功能进行超声评估,并探讨几种超声测量指标变化的临床价值及意义。方法应用实时三维超声(RT3DE)、二维超声(2DE)及M型超声(M-Echo)对40例行右心室流出道重建术的圆锥动脉干畸形患儿术前、术后2～3d进行右心收缩功能评估,并比较手术前、后RT3DE所测右心舒张末容积(3D-RVEDV)、收缩末容积(3D-RVESV)及射血分数(3D-RVEF)、右心室心尖四腔切面面积变化率(RV-4FAC)、右心室两腔切面面积变化率(RV-2FAC)、三尖瓣瓣环收缩期位移(TAPSE)及舒张末期右心室游离壁心肌厚度(RVWT)等指标,分析各指标变化的临床价值和意义。结果与术前相比,术后2～3d3D-RVEDV、3D-RVEF、RV-2FAC及TAPSE均显著降低(P<0.001),而3D-RVESV、RV-4FAC无明显变化,术后RVWT较术前显著增加。术前RV-2FAC与RV-4FAC测值无显著差异,术后RV-2FAC较RV-4FAC降低。结论 3D-RVEF、RV-2FAC及TAPSE是反映右心室流出道重建术围手术期右心收缩功能的敏感指标。右心室流出道重建术后右心室总体、右心室流出道、游离壁长轴方向运动功能均受累,其中右心室流出道收缩功能异常对右心总体收缩功能影响较大。     

采用国产同心伞片经胸小切口封堵膜部室间隔缺损的研究

目的总结我们通过微创经心室采用国产同心伞片行膜部室间隔缺损封堵的临床经验,探讨这项技术的手术方法和适应证。方法回顾性分析2011年12月至2012年7月上海交通大学医学院附属上海儿童医学中心45例膜部室间隔缺损患者[男25例,女20例;年龄(34.1±24.2)个月;体重(12.4±9.2)kg]采用胸下小切口经心室途径封堵治疗,患者均为膜周限制性室间隔缺损,室间隔缺损基底部直径2～9mm,缺损上缘距主动脉右冠瓣≥2mm,所选伞腰直径4～10mm。在经食管超声心动图的引导下非体外循环下经右心室放入室间隔封堵伞。结果术后发生室性早搏1例,经治疗好转并未复发。其余患者术后心功能稳定,随访1～6个月,患儿伞片位置无移位,无心律失常和残余分流。结论经胸小切口采用同心伞片封堵膜部室间隔缺损安全有效,但对于初学者应该严格把握手术适应证。     

新生儿梗阻型完全性肺静脉异位引流的治疗

目的 评估新生儿完全性肺静脉异位引流( TAPVC)不同类型矫治手术方法和预后.方法 1999年至2011年,共收治68例新生儿梗阻型TAPVC急诊手术治疗,平均年龄16天,其中心上型21例,心内型8例,心下型36例和混合型3例.心上型和心下型TAPVC是将肺静脉共汇与左心房后壁作侧侧吻合,心内型TAPVC在心房内将扩大的冠状窦去顶将异位的肺静脉隔入左心房.结果 术后早期死亡2例,占2.9％.随访6个月至3年,经超声心动图随访,肺静脉吻合口均无明显狭窄,血液流速1.10 ～ 1.42 m/s.结论 早期的梗阻型TAPVC的纠治中左心房后壁与肺静脉共汇的侧侧吻合远期效果良好,肺静脉的梗阻情况需要远期进一步随访.     

部分液体通气参与体外循环肺损伤保护的基础研究

目的通过体外循环(CPB)肺损伤动物模型,探讨以全氟化碳(PFC)为媒介的部分液体通气(PLV)对深低温CPB肺损伤的保护作用及机制。方法建立CPB动物实验模型。20只幼猪随机分为对照组(CPB组)和实验组(CPB+PLV组):对照组只行单纯CPB;实验组在CPB停后即刻经气管插管向幼猪肺内灌入FC-77。实验组与对照组以同样的呼吸参数行机械通气。分别于CPB前、CPB停后即刻、CPB停后1及2 h抽取动脉血,行动脉血气分析及血清中炎性因子[肿瘤坏死因子(TNF)-α、白细胞介素(IL)-8和IL-6]含量检测。于实验结束即刻取左下肺小块组织,经过实验室处理,观察肺组织组织黏附分子(ICAM-1)的表达和组织形态学变化。结果实验组CPB停后2 h,TNF-α、IL-8、IL-6均显著低于对照组(P值均<0.05)。实验组CPB停后1、2 h,动脉血氧分压(paO2)显著高于对照组(P值均<0.05),动脉血二氧化碳分压(paCO2)显著低于对照组(P值均<0.005)。光学显微镜下实验组肺损伤程度明显轻于对照组。CPB停后对照组肺组织中ICAM-1的表达呈强阳性,实验组呈弱阳性。结论PLV对CPB肺损伤有较好...     

完全性肺静脉异位引流病理谱及个体化手术治疗

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.     

先天性纠正型大动脉错位不同手术方法结果的比较

目的 比较先天性纠正型大动脉错位(ccTGA)的各种手术治疗结果方法,为临床治疗ccTGA提出更合理的手术方式.方法 1999年6月至2009年12月外科手术治疗95例ccTGA,包括传统纠治手术25例,功能性单心室纠治手术26例,双调转术(Double Switch)手术26例,姑息手术15例,另有3例接受其他手术.结果 术后死亡7例,其中传统手术组死亡2例,Double Switch手术组死亡4例,其他手术组死亡1例,单心室手术组全部存活.结论 纠正型大动脉错位的手术方法的选择取决于解剖条件和生理参数.在各类手术结果的比较中,功能性单心室纠治手术的死亡率低、并发症少、再手术率低,可适当放宽功能性单心室纠治手术指征,可能得到更好的治疗效果.但长期的手术疗效还有待于进一步随访.

Abstract：

Objective To study the clinical outcomes of various corrective surgeries for congenitally corrected transposition of the great arteries (ccTGA) in children. Methods Between June 1999 and December 2009, 95 patients with ccTGA who underwent corrective surgeries at this center were enrolled in this study. Among the 95 patients, 25 patients were performed conventional surgical repairs, 26 had functionally univentricular surgical repair, 26 had double switch operation, 15 had palliative surgeries, and 3 underwent other surgical treatments. Results Seven deaths after surgery were recorded, including 2 patients who had conventional surgical repairs, 4 with double switch operation and 1 with other surgical treatments. No death of the patients who underwent functionally univentricular surgical repair was noted. Conclusions The surgical strategy to correct ccTGA should be made according to patient's individual anatomic anomalies and pathophysiology. Short term outcome study revealed that functionally univentricular surgical repair is safe and effective.     

49例儿童先天性主动脉瓣狭窄的外科治疗

目的 回顾性总结先天性主动脉瓣狭窄患儿手术治疗的效果及经验.方法 2006年2月至2011年11月,共收治49例先天性主动脉瓣狭窄患儿,男29例,女20例;年龄1个月～ 14岁;体质量3.2 ～47.0 kg.轻度狭窄2例,中度狭窄21例,重度狭窄26例.术前跨瓣压差45～123 mm Hg(1 mm Hg =0.133 kPa),平均(74.9±20.4)mm Hg.单纯主动脉瓣狭窄14例,伴有中—重度反流4例.伴其他心内畸形33例,包括室间隔缺损、房间隔缺损、动脉导管未闭、主动脉缩窄、左心室流出道狭窄、二尖瓣瓣上环等.行主动脉瓣交界切开术31例,主动脉瓣成形术9例,主动脉瓣置换术2例,Ross手术1例,Ross-Konno手术2例,Konno+主动脉瓣置换术4例.术后随访2～55个月,平均20个月.评估患儿心功能、主动脉瓣跨瓣压差及瓣膜反流程度.结果 死亡1例,生存患儿术后心功能良好,左心室射血分数0.69±0.10,短轴缩短分数0.38±0.09.术后跨瓣压差20 ～ 73 mm Hg,平均(38.6±15.8)mm Hg,较术前降低(36.2±18.3)mm Hg(P ＜0.001).40例患儿行主动脉瓣交界切开和成形,术后主动脉瓣反流程度为无或轻微8例,轻度25例,中度7例.1例主动脉瓣交界切开术后因切开处复粘连再次行交界切开术.结论 先天性主动脉瓣狭窄的患儿如无明显反流,可行主动脉瓣交界切开术或同时行主动脉瓣成形术,如果反流中度以上,则需根据患儿年龄等情况选择合适的手术方法.     

小儿先天性心脏病肺动脉高压术后八年随访

为随访小儿先天性心脏病（先心病）肺高压术后远期情况，了解患儿的生命选本病１９８年中０．３－７．５岁，体得５－９．２ｋｇ，以术前肺循环阻力／体循环阻力（ＲＰ／ＲＳ）０．５为界分Ａ、Ｂ胡访生长发育情况，心电图、胸片和彩超检查。结果： 几年患儿生长发育、活动量明显增加。６例Ｐ２仍有亢进（９．６％），３便仍闻及有杂时（４．８％）。心电图均示窦律，其他异常与室缺及修补途径均无关。彩超示无余分流，９例轻度ＴＲ     

小儿复杂型先心病一期和分期全腔肺吻合术

1998年 3月至 2 0 0 2年 3月 ,我们共收治 15例复杂型心内畸形病儿 ,分别采用一期和分期全腔肺吻合术 (totalcavopulmonaryconnection ,TCPC)治疗 ,现报道如下。资料和方法　本组中 11例行一期手术 ,年龄 ( 5 7± 0 8)岁 ;单心室 6例 ,三尖瓣闭锁 5例。 4例行分期手术 ,年龄( 7 1± 0 5 )岁 ,单心室、三尖瓣闭锁各 2例 ,TCPC术前分别先做单侧或双侧双向腔肺分流术 (Glenn)术 ,行Glenn术时年龄( 1 8± 0 6 )岁 ,两次手术平均间隔 ( 5 7± 0 3)年。在进行双向腔肺吻合术前 ,分期手术组比一期手术组具有较多的高危因素 ,如年龄小、肺…     

功能性单心室合并肺动脉高压的分期手术结果

目的分析功能性单心室合并肺动脉高压(FSV-PH)的分期手术结果,探讨初期手术时机及相关风险因素与治疗结果的关系。方法回顾性分析2008年4月至2015年12月我院收治功能性单心室合并肺动脉高压129例患者的临床资料,其中男81例、女48例,右心室双出口71例,三尖瓣闭锁17例,大动脉错位7例,单心室33例,完全性房室间隔缺损1例。初期进行肺动脉环缩术(PAB),之后根据肺血管状况,适时分期行Glenn手术和Fontan手术。结果 129例患者共行159例次PAB,术后死亡6例(4.7%),失访9例;87例(67.4%)患者行二期Glenn手术,死亡2例;43例(33.3%)患者行三期Fontan手术,术后死亡3例。32例合并主动脉缩窄、主动脉弓中断、心脾综合征、完全性肺静脉异位引流或房室瓣反流,分别有15例(46.9%)行Glenn手术和6例(18.8%)行Fontan手术。14例患者出现左室流出道狭窄。结论早期PAB有利于FSV-PH进行后期的Glenn和Fontan手术,合并复杂畸形是降低Glenn和Fontan完成率的危险因素。