主动脉弓补片成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良

目的总结缩窄段切除加主动脉弓补片成形术治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的临床经验。方法 2007年5月至2009年12月,上海交通大学医学院附属上海儿童医学中心对49例婴幼儿主动脉缩窄合并主动脉弓发育不良患者采用主动脉缩窄段切除加主动脉弓补片成形术治疗,其中男30例,女19例;年龄23 d至3岁1个月,6个月34例,6个月~1岁10例,1岁5例。31例患者在深低温体外循环下完成手术,采用选择性脑灌注技术;15例在深低温停循环下手术;3例在中低温体外循环下完成手术。31例用自体心包行主动脉弓成行补片,14例采用自体肺动脉壁组织,4例采用异种心包。合并的心内畸形同期纠治。结果围术期死亡1例,死于循环衰竭,手术死亡率2.04%(1/49)。5例术后发生低心排血量综合征,1例合并肾功能衰竭患者,均经相应处理治愈。术后复查超声心动图提示无残余梗阻。随访48例,随访时间4个月至3年。随访期间有1例患者主动脉弓压力阶差40 mm Hg,计算机断层扫描显示主动脉弓再狭窄,于术后8个月再次手术;2例主动脉弓压力阶差20 mm Hg,仍在继续随访;其余患者主动脉弓形态良好,与术后当时比较,随访期间主动脉弓降部血流速度无明显变化,计算机断层扫描显示:主动脉弓几何构型正常。术前存在左主支气管受压的患者在主动脉弓成形术后半年,左主支气管受压表现有明显改善或完全消失,无主动脉夹层动脉瘤发生。结论缩窄段切除术加主动脉弓补片成形术是治疗婴幼儿主动脉缩窄合并主动脉弓发育不良的理想手术方法。     

膜部室间隔缺损微创手术封堵初步经验总结

目的 总结通过微创手术经心室行膜部室间隔缺损（VSD）伞片封堵的临床经验,探讨该技术的手术方法和适应证。方法 对48例膜部VSD患儿采用胸下小切口经心室途径封堵治疗。患儿均为膜周限制性VSD,VSD基底部直径2～9 mm;伞腰直径4～10 mm;45例采用同心伞,3例采用偏心伞。在经食管超声心动图的引导下经右心室放入室间隔封堵伞。结果 45例患儿术后心功能稳定,随访1～6个月,伞片位置无移位,无心律失常和残余分流。其余3例患儿发生心律失常,其中1例发生室性早搏和短阵性室性心动过速,2例发生房室传导阻滞,经治疗均好转。结论 经胸小切口行伞片封堵膜部VSD为VSD的治疗提供了一种良好的选择,应严格把握手术适应证。     

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。     

胸壁、胸膜：改良Nuss手术纠治小儿不对称型漏斗胸

共施行漏斗胸Nuss手术纠治53例，其中17例为不对称型，男13例，女4例。最小年龄2岁5个月，最大14岁，平均8.9岁，手术在胸腔镜辅助下完成。根据患儿畸形情况，设计个性化钢饭形状，并对Nuss手术进行改良。结果：17俐患儿均顺利完成手术，术中平均失血少于15mL。1例患JL术后胸片显示少量气胸，1例患儿术后胸腔积血，1例患儿术后胸腔积液，无其他并发症；术后平均住院6d。     

双侧心房或右心房吻合治疗心下型完全性肺静脉异位引流的临床研究

目的探讨心下型完全性肺静脉异位引流中肺静脉共汇-垂直静脉与双侧心房的位置关系和肺静脉共汇-垂直静脉与双侧心房或右心房后壁吻合的可行性。方法回顾性分析心下型完全性肺静脉异位引流病例资料,利用CT影像重建技术分析肺静脉共汇-垂直静脉在其前方心房层面的投影与双侧心房的位置关系。结果对27例心下型完全性肺静脉异位引流患儿的心脏增强CT影像进行重建。将肺静脉共汇-垂直静脉的投影落在左心房区域称为左心房型(6例,22.22%),落在右心房区域称为右心房型(12例,44.44%),落在两侧心房中间称为中间型(9例,33.33%)。18例单纯性心下型完全性肺静脉异位引流手术病例无手术死亡,在8个月(4～45个月)的随访中未出现明显心律失常及临床症状,超声心动图随访提示1例病例出现右上肺静脉左心房入口处回流梗阻。8例行肺静脉共汇-垂直静脉与双侧心房或右心房后壁吻合的病例术后效果良好。结论心下型完全性肺静脉异位引流中肺静脉共汇-垂直静脉在心房层面的投影是以偏离左心房、偏向右心房为主的。术前通过CT重建判断两者的位置关系,并针对中间型和右心房型的患儿进行肺静脉共汇-垂直静脉和双侧心房或右心房后壁吻合是可行的新方法,对降低术后肺静脉吻合口梗阻的风险有积极作用。     

胎儿重度主动脉瓣狭窄宫内介入治疗二例

上海交通大学医学院附属新华医院为2例重度主动脉瓣狭窄胎儿成功实施了胎儿主动脉瓣球囊成形术(FAV)。2例胎儿分别于24周和28周胎龄被诊断为重度主动脉瓣狭窄,随访期间均有向左心发育不良综合征发展的趋势,分别于32周和30+4周胎龄接受FAV,取得技术成功。本文对FAV的适应证、操作要点及随访预后进行介绍分析,为该技术进一步发展积累经验。     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

改良Fontan手术加共同房室瓣置换术治疗心脾综合征

目的:总结对心脾综合征患儿同时进行Fontan手术和共同房室瓣(CAVV)置换术的方法和经验。方法:对3例伴有CAVV中到重度返流的心脾综合征患儿进行Fontan手术的同时置入29～31mm的ATS机械二尖瓣,观察治疗效果。结果:无手术死亡,1例并发完全性房室传导阻滞植入心外膜永久埋藏式起搏器,随访1～6年心功能均为Ⅰ级。结论:心脾综合征患儿的CAVV存在明显的结构缺陷,CAVV成形术效果差,Fontan手术加CAVV置换术是有效可行的手术方案;在行CAVV置换术时,应尽量保留CAVV瓣下组织结构,对Fontan术后心功能的稳定尤为有利;褥式缝合时应浅缝缝针尽量置于瓣环靠近瓣叶处,避免损伤房室传导系统;术后须及早抗凝治疗,维持INR在1.5～2.5,可避免抗凝并发症的发生。     

轻中度及中度房室瓣膜反流在Fontan术后的预后和相关危险因素分析

目的研究轻中度、中度房室瓣膜反流在Fontan术后的预后及危险因素。方法将2004~2018年房室瓣膜反流为轻中度、中度于本中心行Fontan术和瓣膜成形手术的34例患者作为成形组,在同期患者中以1∶1~2的比例匹配对照组患者65例。共入组99例,其中男64例、女35例,年龄(63.4±36.3)个月,体重(17.3±6.7)kg。反流程度评级下降≥1定义为反流明显改善。研究终点为死亡、Fontan拆除或房室瓣膜置换,研究的危险因素包括Fontan术式、房室瓣膜成形方法、心脏解剖等。结果随访时间1.5(0.3~4.0)年,死亡率15.2%。成形组34例,82.4%患者接受单一成形术式。部分性瓣环环缩最为常见(52.9%)。随着随访时间延长,全组患者瓣膜反流程度存在逐渐加重的趋势(r=0.352,P=0.000)。轻中度反流在Fontan术后自行改善,而中度反流没有。瓣膜成形可以改善中度反流术后的反流程度,不增加手术死亡率,8.8%患者反流明显下降。瓣膜成形手术对轻中度反流无效,会增加手术死亡率。结论房室瓣膜功能在Fontan术后呈现逐渐下降的趋势。瓣膜成形手术对中度反流有效,不增加手术死亡率,但改善程度有限,而对轻中度反流无效并增加手术死亡率。