同种带瓣主动脉和带瓣肺动脉补片在儿童复杂先心病的应用

１９８９年１１月至１９９２年７月期间，应用低温保存同种带瓣大动脉纠治儿童复杂性先心病１２例。病人年龄２～１６岁（７．２±３．７岁），体重８～３１．５ｋｇ（１８．９±６．６ｋｇ）。诊断为法乐氏四联症（ＴＯＦ）８例，其中伴肺动脉瓣闭锁３例；冠状动脉畸形１例；严重肺动脉发育不良２例；左肺动脉缺如２例。大动脉错位伴室间隔缺损和肺动脉狭窄２例；永存动脉干畸形（Ⅱ型）２例。８例应用同种带瓣管道作右室流出道与肺动脉连接，４例应用同种带瓣肺动脉片作跨瓣环右室流出道扩大术。本组死亡１例，最长随访时间３年。文中对同种带瓣管道的取材方法，保存技术及应用方法作了讨论。     

二尖瓣成形术治疗小儿先天性二尖瓣反流疗效分析

目的评价二尖瓣成形术在小儿先天性二尖瓣反流治疗中的应用效果。方法对接受二尖瓣成形术治疗的78例二尖瓣反流患儿的手术指征、手术方式、疗效及随访结果进行分析。结果 78例患儿平均随访时间12.6个月(0.5～73个月)。术后死亡3例,均为住院期间死亡。术后15例(19.2%)出现并发症。再次手术4例,其中3例行二尖瓣置换术,1例行二次成形术。1例因术后心力衰竭再次入院。随访期间二尖瓣反流评分与术前相比均有明显下降(P<0.05)。结论二尖瓣成形术治疗小儿先天性二尖瓣反流近期效果理想,能适应患儿的生长,可以有效地延迟或避免行二尖瓣置换术。     

新生儿、婴幼儿期主动脉弓中断一期矫治效果分析

目的 探讨新生儿、婴幼儿期主动脉弓中断一期矫治手术早、中期效果以及有关死亡风险因素.方法 回顾性分析1988年7月至2008年6月期间连续72例主动脉弓中断伴有合并畸形的一期矫治手术效果,其中63例手术中采用改良端侧直接吻合技术重建主动脉弓,9例在吻合的前壁用补片扩大成形;采用Logistic多元线形回归分析和评估早、中期结果以及有关影响手术后效果的危险因素.结果 住院总生存率83.4%,手术后0.5、2、5、10年生存率分别为98%、96%、96%、95%.与术后死亡有关危险因素是大年龄(P=0.018)、合并复杂畸形(P=0.001)、术前状况(P＝0.01)和肺动脉高压危象(P=0.014).结论 一期矫治主动脉弓中断以及合并畸形的早、中期预后好,重建主动脉弓采用直接端侧吻合技术以及适宜补片扩大效果佳,术前年龄和状况影响术后早期结果.     

外源性肺泡表面活性物质在婴儿心脏直视术后的应用

目的　评价外源性肺泡表面活性物质 ( PS)治疗婴儿体外循环后肺损伤的疗效。　方法　选择 7例术后反复撤机失败 ,依赖机械通气 ,胸部 X线片显示大片肺不张和 /或肺间质透亮度降低的婴儿 (年龄 0 .4 9± 0 .82岁 ,体重 4 .87± 2 .18kg) ,在至少 1次撤机失败后经气管内插管应用 PS,初始量按 10 0 mg/kg给药 ,12小时后重复 1次。分别在初始量后 4、6、12、2 4、4 8和 72小时测定氧合指数 ( OI)、动脉血氧饱和度 ( Sa O2 )和动脉血二氧化碳分压 ( Pa CO2 ) ,记录自主呼吸潮气量、胸部 X线片变化以及呼吸机应用时间。　结果　 PS使用 4小时后 OI、Sa O2 显著升高 ,2 4小时后增幅最大 ,分别为给药前的 34.7%和 6 .6 % ;Pa CO2 在给药 4小时后显著降低 ,6小时后最大降幅为 2 2 .8% ( P<0 .0 5 ,0 .0 1)。用药后患者最大自主呼吸潮气量、胸部 X线片明显改善 ,拔管成功率 85 .7%。　结论　外源性 PS替代治疗能显著改善婴儿心脏直视术后肺功能 ,并有效缩短机械通气时间     

心脏传导束染色的实验研究

本文报告了蒸汽碘染色心肌传导束的实验结果，选用８条杂种犬，分成两组，急性处死后取出心脏，分别用Ｌｕｇｏｌｓ溶液和碘蒸汽两种方法染色，另取８条杂种犬，在体外循环下切开右心房，通过三尖瓣暴露右室隔面，直视下进行碘蒸汽染色，记录心脏复跳后电图改变，最后急性处死取心肌作组织学观察，结果显示，离体犬心脏两种染色方法均能清晰显示右束枝，左束枝及其分枝，活体犬心脏经碘蒸汽染色后，未出现完全性传导阻滞和完全性右束     

小婴儿先天性主动脉弓中断合并心内畸形的Ⅰ期手术治疗

目的总结小婴儿主动脉弓中断合并心内畸形Ⅰ期手术治疗经验。方法2001年9月~2004年8月,手术治疗小婴儿主动脉弓中断合并心内畸形10例。男性7例,女性3例,手术年龄18d~7个月,平均年龄(3.37±2.45)个月,平均体重(5.33±1.72)kg。所有病例术前均有反复呼吸道感染史,病例分型A型7例,B型3例。均采用胸骨正中切口Ⅰ期手术纠治主动脉弓中断和心内畸形。结果全组病例均存活,随访3个月~3年,恢复良好。结论小婴儿主动脉弓中断合并心内畸形采用胸骨正中切口,操作简便,术野暴露清楚,创伤小,有利于术后恢复。由于该病早期发生肺动脉高压,宜尽早手术。     

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.     

同种带瓣管道重建右室流出道手术死因探讨

应用带瓣管道重建右室流出道治疗复杂先天性心脏病已有多年，80年代以来同种带瓣管道（VHC）应用逐渐增多。1989年11月～1998年12月10年间我们应用自行采集、制备和低温保存的同种异体大动脉治疗各类复杂先天性心脏病83例，存活71例，死亡12例，死亡数占14.5%。现对12例死亡病例进行初步分析，以期寻找失败原因，提高疗效。     

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.     

Konno术在小儿左心室流出道梗阻中的临床应用

目的 总结Konno手术治疗小儿左心室流出道梗阻(LVOTO)的近、中期效果.方法 2006年5月至2012年5月共收治16例LVOTO患儿,年龄1.9～13.6岁,平均(5.1±0.7)岁;体质量12 ～39 kg,平均(18.3±6.3) kg.其中13例(81.3％)有两个以上部位的LVOTO.超声评估LVOTO压差56 ～ 185mmHg(1mmHg =0.133 kPa),平均(96±31) mmHg.手术在中低温(28C ～ 30℃)体外循环下进行,体外循环70 ～182min,平均(98±21) min;主动脉阻断34 ～148min,平均(51±11) min.单纯Konno手术3例;Konno± Ross手术4例;Konno-Rastan(主动脉瓣置换)手术9例.结果 患儿术后无死亡.呼吸机辅助3～9天,平均(4.0±1.5)天.住ICU5～16天,平均(8.0±2.9)天.术后随访6个月～3年.心脏超声检查无明显残余梗阻,LVOTO流速1.10 ～2.42 m/s;左心室射血分数0.58 ～0.72,平均0.66±0.03.主动脉瓣反流轻到中度2例,中-重度1例,余均为轻度;1例出现肺动脉瓣中度反流,三尖瓣轻到中度反流.结论 Konno手术治疗小儿LVOTO安全、有效,利于患儿左心功能恢复,远期并发症情况尚需进一步随访.