永存第五对主动脉弓狭窄二例

永存第五对主动脉弓（persistent fifth aortic arch,PFAA）狭窄是罕见的主动脉弓畸形，我院自1988年7月至2004年8月收治2例PFAA狭窄同时合并主动脉弓离断（interrupted aortic arch,IAA）的患儿，手术治疗效果满意。     

经食管超声心动图在室间隔肌部缺损镶嵌治疗中的价值

目的 评价经食管超声心动图（TEE）在室间隔肌部缺损镶嵌治疗中的价值。方法 7例室间隔肌部缺损患者，经超声心动图、心血管造影检查明确诊断。外科开胸暴露心脏，TEE监测下，在跳动的右心室表面选择穿刺点，使动脉止血鞘通过缺损进入左心室，然后释放肌部缺损堵塞装置。若TEE证实封堵器位置良好，无明显残余分流，释放封堵器。结果 6例堵塞成功，术后即刻TEE检查3例见少量残余分流，出院时复查经胸超声心动图2例存在少量残余分流。1例患者由于经胸超声心动图诊断室间隔肌部缺损位置不准确，未行镶嵌治疗，最终体外循环下直视修补。结论 TEE可以准确地显示室间隔缺损的大小、数目、部位及其边缘的解剖形态，有利于术前病例的选择、术中穿刺点的定位及闭合器的安置、术后即刻疗效的判定，是一种安全、有效的监测手段，值得在镶嵌治疗中推广应用。     

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.     

含血预充液超滤洗涤法对婴幼儿围体外循环期肺功能影响的观察

目的：探讨婴幼儿体外循环（CPB）手术中对含血预充液进行超滤洗涤预处理后是否能使预充液达到生理状态,以及该方法对患儿围CPB期电解质、酸碱平衡和肺功能的影响。方法选取2013年7月-2014年7月在常州市儿童医院施行先天性心脏病矫治手术的患儿30例,随机分为2组;实验组15例：男8例,女7例,年龄（13.25±7.01）个月,体重（9.88±1.71）kg,术前利用CPB管道中血液超滤器对含血预充液进行超滤洗涤20 min。对照组15例：男9例,女6例,年龄（12.75±9.91）个月,体重（9.69±2.55）kg,预充液在CPB管道中循环20 min。测定含血预充液超滤洗涤前、后和患儿围CPB期血气分析和电解质等指标,并观察肺功能变化。结果2组患儿均无死亡,顺利出院。实验组呼吸机辅助呼吸时间显著短于对照组（P＜0.05）,实验组含血预充液经超滤洗涤后的各项指标均趋近生理值,实验组PaCO2、K＋、Lac、Glu、TNF-α浓度均降低,与对照组相比差异有统计学意义（P＜0.05）,而pH、PaO2、BE均升高,与对照组相比差异有统计学意义（P＜0.05）。术后48 h 两组A-aDO2和RI显著上升,然后开始呈现逐渐下降恢复趋势。实验组趋向更早开始恢复肺功能,术后各时点实验组A-aDO2和RI均明显低于对照组（P＜0.05）。结论对含血预充液进行超滤洗涤处理可使CPB预充液更趋近生理状态,减少炎性介质,改善婴幼儿术后肺功能,这对低体重婴幼儿进行先心手术时显得很有必要。     

超声心动图评价糖尿病妊娠胎儿心脏功能变化的研究

目的:应用超声心动图技术观察糖尿病妊娠胎儿心脏功能的变化。方法:测量糖尿病妊娠组与正常对照组胎儿的心脏结构与功能的各项指标,并分析其与不同孕周间的关系。结果:孕34周后两组胎儿左室短轴缩短率(LVFS)、右室短轴缩短率(RVFS)、二尖瓣E、A峰值速度比值(E/AMV)、三尖瓣E、A峰值速度比值(E/ATV)、主动脉瓣血流峰值速度(VPAO)、肺动脉瓣血流峰值速度(VPPA)差异有统计学意义(P<0.05)。30周后两组胎儿Tei指数、室间隔舒张末期厚度(IVSd)、左室壁厚度(LVWT)、右室壁厚度(RVWT)差异有统计学意义(P<0.05)。结论:糖尿病妊娠胎儿心功能及结构变化发生于30周后,Tei指数是评价糖尿病妊娠胎儿心脏功能变化的敏感指标。     

心脏手术围术期肝素诱导性血小板减少症的研究进展

肝素诱导性血小板减少症(heparin-induced thrombocytopenia,HIT)是肝素治疗期间抗体介导的严重并发症,通常在肝素治疗后5～ 14天发生.其临床特点为血小板减少和发生动(静)脉血栓风险增加.38％～76％ HIT患者形成血栓,其中10％需要进行截肢手术,20％ ～ 30％ HIT患者在1个月内死亡[1-2].HIT抗体是暂时性存在的,常会在3个月内消失.若患者体内产生永久性抗体则再次接触肝素时其后果将更为严重.据推算,在美国每年仅用于治疗心脏手术患者HIT并发症的相关费用就高达3亿美元[3].现围绕心脏手术围术期HIT的研究进展综述如下.     

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。     

全腔静脉-肺动脉直接吻合术中期效果的临床研究

目的总结全腔静脉-肺动脉直接吻合(DCPC)Fontan术纠治功能性单心室的中期效果。方法 2005年1月至2011年5月我院共对25例复杂型心内畸形患儿施行DCPC Fontan术,男15例,女10例,平均年龄6.8岁(1.3～12.3岁);平均体重25.0kg(11.0～46.0kg)。病种为单心室7例,完全性大动脉错位(D-TGA)合并肺动脉狭窄伴室间隔缺损6例,纠正型大动脉转位(ccTGA)合并肺动脉狭窄伴室间隔缺损6例,完全性房室共通道的右心室双出口(DORV)4例,心脾综合征2例。术前分别行右侧双向腔肺动脉吻合术和双侧双向腔肺动脉吻合术各1例。结果其中10例患儿常规建立体外循环并阻断主动脉,体外转流时间76～179min,平均(134.7±43.8)min,主动脉阻断23～99min,平均(40.4±16.9)min;另15例患儿在平行循环下行DCPC术,体外转流时间82～139min,平均(112.1±26.3)min,不阻断主动脉。10例患儿合并其他心内畸形需纠治,15例患儿行"开窗术"。术后并发症为室上性心动过速1例,肾功能受损导致无尿需行腹膜透析1例,置胸腔引流管时间>7d5例,窦性心动过缓伴窦性停搏1例,心包积液1例。门诊随访2个月至6年,无一例死亡。结论 DCPC术中期随访效果满意,对术前具备肺动脉发育可,主、肺动脉位置合适及上、下腔静脉有足够长度等条件的患儿具有一定的适用性。