OBJECTIVE:To assess the accuracy of cardiac output (CO) measured by transpulmonary thermodilution technique (TPTD)and explore the validity of intrathoracic blood volume index (ITBVI) for assessment of circulatory volume status.METHODS:Ten immature pigs with a mean weight of (20.6±1.9)kg were studied during the conditions including normovolemia, hypervolemia, and hypovolemia. Simultaneous CO was measured in each condition using pulmonary artery thermodilution (PATD) method and TPTD. More specifically, CO (COPA) was determined with PATD, while CO (COTP) and ITBVI were determined with TPTD. All measurements were repeated 3 times. Central venous pressure (CVP) and heart rate were measured at the same time. The potential correlations of CVP and ITBVI with cardiac index (CI) and stroke volume index (SVI) in each blood volume status were analyzed.RESULTS:A total of 90 simultaneous measurements of COPA and COTP in 3 different blood volume conditions were made. The correlation coefficient between the two measurements was 0.977 (P<0.001) and the mean difference was (0.25±0.26)L/min (95%CI:0.20-0.30 L/min, P<0.001). The coefficient of variation of COTP was 3.7%, while COPA was 5.4%. Compared with those in normovolemia, CVP and ITBVI in hypervolemia significantly increased (P=0.002, 0.019), ITBVI in hypovolemia decreased significantly (P<0.001), and CVP in hypovolemia decreased insignificantly (P=0.05). Correlation analysis revealed a significant correlation between ITBVI with CI and SVI in normovolemia (r=0.741, P=0.014; r=0.885, P=0.001). In contrast, correlations between CVP with CI and SVI were poor.CONCLUSIONS:TPTD can accurately and precisely measure CO in different blood volume conditions. ITBVI measured by TPTD has better validity for the assessment of circulatory volume status than CVP.

Objective To analyze heart rate variability during asymptomatic periods in children with vasovagal syncope(VVS). Me-thods Forty-five children diagnosed as VVS in clinic were recruited as a study group,38 with positive and 7 with negative response to head-up tilt test(HUTT).Twenty healthy children were matched as healthy controls.Twenty-four hours holter monitoring was performed and time and frequency domain indices were studied. Results 1.Compared with the healthy controls,the values of time-domain SDNN(121.82±33.0 vs 152.95±31.66,P0.01) and SDANN(114.42±33.41 vs 134.7±27.43,P0.05) were lower,and the ratio of low frequency / high frequency(LF/ HF)(1.67±0.54 vs 1.25±0.2,P0.01) were higher in study group.2.There was no significant difference in the time-domain and frequency-domain indices(P0.05) in HUTT positive and negative children.3.Compared with cardioinhibitory response(CI) children,SDNN(100.61±23.02 vs 150.5±29.46,P0.01),SDANN(96.78±28.77 vs 141.25±29.06,P0.05),rMSSD(29.22±19.56 vs 53.75±23.08,P0.05) and PNN50(11.29±9.31 vs 23.96±12.54,P0.05) were significantly lower,LF(39.61±8.45 vs 19.68±4.17,P0.01) and LF/HF(2.12±0.55 vs 1.09±0.11,P0.01) were significantly higher in vasodepressor response(VD) children.And compared with mixed responses(MX)children,rMSSD(29.22±19.56 vs 52.57±18.54,P0.05) and PNN50(11.29±9.31 vs 22.96±11.13,P0.05) were lower significantly,LF(39.61±8.45 vs 29.72±9.87,P0.05) and LF/HF(2.12±0.55 vs 1.38±0.30,P0.01) were higher significantly in VD children,but there was no difference between CI and MX children(P0.05).4.Compared with the healthy controls,rMSSD and PNN50 obviously declined in VD group while LF significantly increased(P0.05). Conclusions There is a different alteration in autonomic activity in VVS children during asymptomatic periods.The changes in baseline of RMSSD,PNN50 and LF parameters may serve as a predictor to identify VD clinically.

小儿急性重症病毒性心肌炎,又称暴发性心肌炎.起病急骤,病情进展迅猛,早期常以心外表现为主,可突然发生充血性心力衰竭、心源性休克或致死性的心律失常.由于其临床表现缺乏特异性,早期确诊有一定难度,诊治如不及时,病死率极高.本研究通过分析1994年1月～2004年6月我院收治的13例小儿暴发性心肌炎的临床资料,探讨对其早期诊断及抢救治疗的要点。

Fontan手术作为治疗复杂先天性心脏病的一种生理性矫正手术,其效果很大程度上取决于病例的选择.大量研究表明该手术受多方面因素的影响,包括年龄、肺循环状况(肺血管大小、阻力、肺动脉压)、心室功能及某些特殊解剖异常等.术前对有关危险因素进行综合评价有助于提高手术的成功率.

目的研究与法洛四联症(TOF)一期根治手术早期死亡相关的危险因素.方法回顾1995年4月至2004年3月在复旦大学附属儿科医院心血管中心行一期根治术的TOF病例152例,术后早期死亡17例,对其术前、术中27个可能的危险因素指标与术后早期死亡的关系进行单因素和多因素分析.结果单因素分析结果显示与术后早期死亡有关的指标包括:手术时体重、发生青紫年龄、动脉血氧饱和度、升主动脉与肺动脉干直径比AO/MPA、McGoon比值、Nakata指数、肺体循环血流量之比Qp/Qs、右向左分流量占体循环血流量比例(Qs-eQp)/Qs、体肺侧支循环和跨瓣补片.多因素分析结果显示AO/MPA和McGoon比值与手术早期死亡有关.结论肺动脉的发育情况是TOF一期根治术后能否生存的关键.手术病例的选择需要综合考虑多种危险因素.

1 原发性高血压的诊断要点 成人高血压被定义为在未服高血压药和安静情况下持续血压≥18.6/12.0kPa.而儿童高血压的界定需考虑年龄、性别和身高对血压的影响.1990年美国第二届儿童血压控制专题工作组会议对儿童血压评定为:正常血压为收缩压和舒张压小于同年龄、性别及身高儿童血压的第90百分位;临界高血压为90～95百分位;高于95百分位为高血压.

目的 了解人类妊娠早期胎心血流动力学变化的规律,初步筛查出与染色体畸形可能相关的血流讯号.方法采用经阴道二维+多普勒超声技术对172例妊娠早期正常孕妇的胚胎/胎儿心脏进行研究,得出正常参数;随之对1022例行人工流产的孕妇胚胎/胎儿心脏进行探查,总结异常变化并进行绒毛染色体分析.结果(1)妊娠早期胎心血流动力学的变化:妊娠7周以前,原始心搏引起的多普勒讯号表现为单一峰值;妊娠7周后,可观察到流入流出道方向相反的血流,通过流入道的血流流速波形为单峰;妊娠第9周起,通过流入道的血流流速波形开始呈现双峰,e峰出现;胎心率早期随胎龄增加而增快,第10周时最快,之后则有所减慢.(2)观察到27例胎儿心脏血流动力学异常,其中9例核型分析结果异常(3例心动过缓,4例半月瓣返流,2例房室瓣返流).结论妊娠早期胎儿心脏可用高频率经阴道超声进行观察,其血流动力学变化存在时相性特征.早期超声探及心动过缓、出现瓣膜返流讯号的胚胎/胎儿与染色体畸形有一定相关性。

Purpose Chronic diabetic complications like diabetic nephropathy(DN) have been the major death cause of diabetes mellitus,early detection and intervention of DN in microalbuminuria stage or early may reverse the kidney lesion,microalbumin measurement has been the wide accepted standard for DN early screening for several decades,but studies have shown it not fully correlated with kidney pathological changes,so,exploring new measurement for the early detection of DN has been one of the focus of DN researches.The aim of present study is to exploring the value of urinary ceruloplasmin screening in the early detection of diabetic nephropathy in children and adolescents with type 1 diabetes mellitus(T1DM) in contrast to the urinary microalbumin examination. Methods Forty cases of T1DM children and adolescents,twenty-one cases of nephrotic syndrome children and sixty cases normal children were recruited.Twenty four hours urine was collected and the urinary ceruloplasmin and albumin were detected by ELISA and radioimmnunoassay respectively. Results The urinary ceruloplasmin creatinine ratio(UCp/Cr) (median 1.08 ng/min) and urinary albumin excretion rate(UAER) (median 6.84 μg/min) in T1DM and nephrotic syndrome groups(UCp/Cr median 1.8 ng/mmol,UAER median 6.29 μg/min) were significantly increased comparing with normal control(UAER median 1.94 μg/min;UCp/Cr median 0.08 ng/mmol;P0.05).47.5% of T1DM patients were found with abnormal excretion of urinary ceruloplasmin and 10% with microalbuminuria.UCp/Cr was significantly increased in UAER≥20 μg/min group(UCp/Cr median 2.65 ng/mmol) than UAER20 μg/min group(UCp/Cr median 0.34 ng/mmol,P=0.02).UAER in diabetic patients with duration less than 2 year(UAER median 4.9 μg/min) was 1.44 times than that of duration more than 10 years(UAER median 7.08 μg/min),meanwhile,UCp/Cr in DM patients with duration less than 2 year(UCp/Cr 0.28 ng/mmol) was 2.54 times than that of duration more than 10 years(UCp/Cr 0.71 ng/mmol).The change of UCp/Cr in T1DM children and adolescents was significantly correlated with HbA 1c( r=0.45,P=0.01) and UAER( r=0.47,P=0.002). Conclusions Like early DN kidney lesion,lose of negative charge selectivity was the typical change of nephrotic syndrome(NS),UCp in NS control was significantly increased than normal control and positively correlated with UAE,so,the leakage of urinary ceruloplasmin in TIDM adolescents may due to the same change.Because the more significant increase of UCp/Cr in longer DM duration patients than UAE and more abnormal ratio of UCp/Cr than UAE in type 1 diabetes patients,urinary ceruloplasmin screening may be a potential more sensitive and noninvasive mark of early diabetes kidney lesions.

心律失常是儿科的常见病,对儿童的生长发育有较大影响,心律失常儿童的心功能尤为重要,本文前瞻性运用极量活动平板试验(TMT)及多巴酚丁胺负荷超声心动图检查(DSE)评价室早患儿的左心储备功能.

Objective To assess the exercise risk in children with atrial ectopies (AE) with Treadmill Test (TMT) and Dynamic cardiogram (DCG).Methods TMT and DCG are performed in 56 children in AE which have been confirmed in ECG.Results 55(98\^2%) of 56 children had AE in DCG which is higher than that in TMT(85\^7%),41\^7% of these children have positive results in TMT and some showed multiform premature beats or couplet and depressed ST segment during exercise.Except for 5 children whose test were terminated due to severe arrhythmias,all others reach maximal exercise tolerance without striking adverse reactions.Peak time,no frequency and severity of AE, is correlated to TMT positive rate.Positive rate is higher in patients with night time AE than that in patients whose AE are more frequent in daytime.Disappointed results may not occur during exercise in severe AE and Running can exacerbate simple premature beats.Conclusion Children with AE have exercise risk and TMT should be performed in these children.

目的探讨彩色多普勒超声心动图检测胎儿先天性心脏病(先心病)的可行性. 方法 296例胎儿分为两组:142例有高危因素的胎儿,154例正常妊娠胎儿.应用多普勒测定胎儿心脏各切面,并在生后作超声心动随访. 结果在142例有高危因素的胎儿中,发现胎儿先心病5例,其中右心发育不良综合征1例、法洛四联症2例、单纯性室间隔缺损(室缺)2例.漏诊2例,为单纯性室缺1例及房间隔缺损1例.154例正常妊娠胎儿中未发现先心病.生后超声心动随访或引产尸检结果与产前诊断相符. 结论彩色多普勒超声心动图在诊断胎儿先心病方面是比较准确和安全的,对高危孕妇需加强胎儿先心病的检测.

目的:探讨经胸三维超声心动图测量复杂型先心病患者的左室容量及收缩功能的可行性.方法:复杂型先心病患儿19例,应用HP5500型彩色多普勒超声心动图仪,通过手动控制探头采集三维数据,采用圆盘总和法(Disk summation)测量了左室舒张末期容积(LVEDV)、收缩末期容积(LVESV)、每搏量(SV)和射血分数(LVEF).同时在二维引导下以M型超声心动图(Teichholtz)测量了上述指标.并应用心导管检查(Fick)测量了患儿左室的每搏量.结果:经胸M型超声心动图及心导管检查所测SV呈中度相关,r为0.88,而经胸三维超声心动图测量SV与心导管检查所测结果呈高度相关,r为0.90.经胸M型超声心动图与三维超声心动图所测LVEDV、LVESV、SV及LVEF显著相关,r分别为0.86、0.87、0.88和0.89,两者间有显著性差异.结论:经胸三维超声心动图测定复杂型先心病患者的左室容量和收缩功能是准确可行的.

目的运用经阴道超声对妊娠早期胎儿心脏进行血流动力学检测,了解胎心血流的时相变化.方法采用经阴道多普勒超声技术对120例胎龄为7～12周正常孕妇的胎儿心脏进行研究.结果妊娠早期胎心血流存在时相变化:①妊娠第9周起,胎心流入道波形由单峰转变为双峰,e峰出现.②e/a值随胎龄增大而增大,提示心脏顺应性的改善.e峰的逐渐增大是e/a值增大的原因,a峰在妊娠8周后增加不显著.③胎心率早期随胎龄增加而增快,第10周时最快,之后则有所减慢.④等容收缩期(ICT)和等容舒张期(IRT)随胎龄增大而减小,ICT的减小较显著.⑤流出道血流速度随胎龄增加而增大.结论妊娠早期胎儿心脏可用经阴道超声进行无创和连续的观测,其血流动力学变化存在时相性特征.本研究为连续监测正常孕期胎心的发育及血流动力学变化提供了重要依据.

Objective: To evaluate magnetic resonance imaging (MRI) in diagnosing congenital heart disease in children.Method: Thirty eight cases were scanned with ECG gated spin echo technique (SE), cine MRI and magnetic resonance angiography (MRA) using superconductive magnetic system.Three dimensional imaging was reconstructed in four of the patients. Of all, 30 cases underwent angiography and 23 received surgical procedure.Result: MRI findings showed 12 cases with COA, 12 with transposition of the great vessels, 5 tetralogy of fallot, 4 double outlets of the right ventride, 2 pulmary atresia, 2 persistent truncus arteriosus and 1 interrupted aortic arch. Compared with the results of angiography and surgery, MRI could correctly display the defects of the great arteries.MRI also showed excellent correlation with angiography in measuring the smallest diameter (r= 0.95 ,P 0.001 ) in 12 cases of COA.Conclusion: MRI is valuable in diagnosing congenital heart disease with abnormalities of the great arteries.

运动试验在儿科应用不多。国内1982年曾有正常儿童运动平板试验结果[1]报道，但以后少有研究；近年在儿科临床逐步开始应用运动试验来诊断评价心肺疾病，但缺乏新的正常参考标准。我院1999年10月～2000年1月对282例健康儿童进行极量活动平板试验。

Objective This study was to evaluate magnetic resonance imaging(MRI) in diagnosing congenital transposition of the great arteries (TGA).Methods 14 patients (male 8,female 6) aged 2 months to 12 years (mean age 5.4±3.6 years) were scanned with ECG-gated spin echo technique (SE),cine-MRI and magnetic resonance angiography (MRA) at superconductive magnetic system.All patients were examined with 2-dimensional color Doppler echocardiography and 10 of them had received angiography.2 patients had surgical treatment.Results MRI could correctly depict the anatomic structure of TGA,including complete TGA (13 cases) and corrected TGA(1 case).The associated defects included ventricular septal defects (12 cases),intact ventricular septal (2 cases),double outlet of right ventricle (4 cases),cardiac malposition (5cases) and pulmonary stenosis (6cases).The results of MRI were comformed by Angio and /or surgery in 10 patients.Conclusion MRI is accurate in diagnosing congenital transposition of the great arteries.

目的:探讨磁共振成像(MRI)对先天性心脏位置异常的诊断价值.方法:对本组患儿10例,应用超导磁共振仪,选用心电图门控自旋回波技术(SE)、电影磁共振(cine-MRI)及磁共振造影(MRA)进行诊断,所有病例均经 X光胸片和二维彩色多普勒超声心动图检查,其中3例同时进行心血管造影(Angio)检查,一例经手术治疗.结果:MRI准确显示本组患者的心脏和/或内脏位置异常,诊断结果包括右旋心4例、镜像右位心3例,中位心2例及孤立性左位心1例.结论:MRI能准确诊断先天性心脏位置异常.

目的探讨法洛四联症(TOF)患儿心肌细胞钙运转与心功能的关系.方法钙荧光指示剂Fura-2法测定心肌细胞游离钙浓度,HP-1000型超声心动图机测定心功能.结果 28例TOF患儿红细胞游离钙和总钙浓度较健康组明显高,而钙泵活性明显低下;其中8例舒张功能正常者红细胞钙泵活性与健康组无差别,而20例舒张功能异常者不仅钙泵和钠泵活性较舒张功能正常者明显下降,且红细胞游离钙和总钙浓度亦较舒张功能正常者升高.结论 TOF患儿心肌细胞内钙运转与其心肌舒张功能异常密切相关.

目的对复杂型先天性心脏病(先心病)磁共振成像(MRI)与心血管造影(Angio)进行对比分析研究,评价MRI在小儿复杂型先心病的诊断价值.方法对复杂型先心病患儿42例,应用超导磁共振仪,选用心电图门控自旋回波法技术(SE),电影磁共振(cine-MRI),磁共振造影(MRA),获取T1加权像(T1WI),并进行二维彩色多谱勒超声心动图检查,其中30例做了Angio检查,25例经手术治疗.结果 MRI能准确诊断本组病例,包括大动脉转位(TGA)、主动脉缩窄(COA)、法洛四联症(TOF)、右室双出口(DORV)、永存动脉单干(TA)、肺静脉易位引流(TAPVD)及单心室(SV) 的主要解剖畸形及合并畸形.MRI亦能准确显示肺动脉及其分枝.结论 MRI在小儿复杂型先心病诊断上有重要的临床应用价值.

Objective To find out the feasibility of rat embryos with vitamin A deficiency (VAD) as an animal model of congenital heart disease. Methods Both normal and VAD rat embryos were taken out in different embryonic days (ED). After embedding in paraffin, serial sections and HE staining were done to observe the development of the embryonic heart. Results 1 .The prepared diet of VAD group contained 7μg vitamin A(VA)/ 100g diet. After feeding with VAD diet, the serum VA concentrations of rats in VAD group were obviously lower than in control group [(0. 168 ± 0.059) μmol/L Vs (2.18 ± 0.23) μmol/L, t=32.88, P0.001]. 2.The percentage of death: rats of control group all surivived; VAD rats maintained in barrier environment survived up tO 4.6 times longer than in open environment (10% Vs 45.83%,'x2=16.64, P0.001). 3. The percentage of pregnant and the litter number of every maternal rat in VAD were lower than the control[58.33% Vs 81.5%,x2=4.37, P0.05][6.97 ± 2.79) Vs (13 ± 1.05), t=7.16, P＜0.001]. 4.The histologic study showed that in VAD group 36.67 percent of embryos appeared obviously delay in the heart development during ED11 to ED15, while during ED16 to ED19 41 .43 percent of embryos showed heart malformation and 18.57 percent of embryos showed anomalies of the vascular system. Conclusion it seems that VAD embryos can be regarded as an animal model of congenital heart disease, but it needs to improve the feeding sanitation to reduce abnormal deaths.