经食管超声心动图在小儿先天性心脏病围术期的应用价值

目的 评价经食管超声心动图(TEE)对指导小儿先天性心脏病(先心病)外科手术和介入治疗的应用价值及其安全性。方法 2000年11月至2005年1月，在上海市复旦大学附属儿科医院心血管中心接受外科开胸手术或经导管介入治疗的先心病患儿中有317例进行了TEE检查，年龄2个月至17岁6个月(平均4.7岁)。采用HP/SONOS2500超声诊断仪，频率5.5~7.5MHz双平面经食管探头，全麻状态下进行。结果 与术前经胸超声心动图(TTE)比较，术前TEE检查对诊断作出修正或补充者51例(16.1%)，其中因此而修正了手术治疗方案25例(7.9%)。术后TEE检查发现有并发症或残余问题57例(18.0%)，其中8例(2.5%)因此立即再次手术。所有病例均未因TEE检查而引起并发症。结论 TEE可安全地应用于小儿先心病围术期检查，对术前诊断做出修正或补充，术后可及时发现并发症或残余问题。 Abstract Objective To evaluate the utility of transesophageal echocardiography (TEE) for surgical and interventional repairs in children with congenital heart disease (CHD) and its safety as well.Methods 317 patients with CHD underwent TEE examinations at the age of 2 months~17 years and 6 months(mean 4.7 years).HP/SONOS2500 ultrasonic instrument with transesophageal biplane probe of 5.5~7.5MHz was used to perform TEE under general anesthesia.Results Compared with preoperative transthoracic echocardiography(TTE),TEE had new findings or made revision of the diagnoses in 51 cases(16.1%) before operations,which led to the changes of surgical or interventional precedure in 25 cases(7.9%).TEE had found residual problems or evolving situations in 57 cases(18.0%) after operations,which led to a second immediate surgical intervention in 8 cases(2.5%).There were no detectable complications due to TEE in all cases.Conclusion TEE is valuable in providing meaningful preoperative emendations or additions and finding residual problems after operations. The technique is safe in children. Key words Transesophageal echocardiography (TEE);Congenital heart disease (CHD);Perioperative period;Children     

多层螺旋CT和经胸二维超声心动图检查

目的 探讨多层螺旋CT(MSCT)及联合经胸二维超声心动图(TTE)检查在先天性心脏病诊断中的意义。方法收集广东省心血管病研究所自2002年9月至2003年12月间86例儿童先天性心脏病病例，全部患儿接受了MSCT和TTE检查，12例接受心导管检查，其中69例进行了外科手术，将术前MSCT、TTE和心导管检查结果分别与手术诊断进行比较。结果 69例患儿共计有129处畸形，TTE正确诊断116处(89.9%)，MSCT正确诊断112处畸形(86.8%)，两者联合正确诊断127处畸形(98.4%)，12例心导管检查未能提供更有价值的资料。结论 MSCT对先天性心脏病诊断具有较高的价值，尤其是联合TTE可取代部分心导管检查为外科手术提供正确和充分的术前诊断。对于年龄小或重症不耐受心导管检查的患儿具有更大的意义。 Abstract Objective To evaluate the effectiveness of multislice computed tomography (MSCT) in diagnosis of congenital heart diseases in children.Methods 86 patients were involved.69 had been performed operations.The preoperative results of transthoracic two dimensional echocardiography (TTE),MSCT and angiocardiography were compared with those of surgery.Results Of the totally 129 malformations,TTE had correctly revealed 116 (90%),MSCT 112(86.8%),and both combined had correct diagnosis for 127(98.4%).Angiocardiography in 12 patients had not provided further information. Conclusion MSCT is a valuable additional diagnosis tool in congenital heart diseases,especially when combined with TTE.They might take place some of the angiocardiography before surgery. Key words Multislice computed tomography;Transthoracic two dimensional echocardiography;Congenital heart disease     

婴幼儿铅中毒脑病临床特点及预后分析

目的 探讨铅中毒脑病的临床特点及预后。方法 回顾分析衡阳市中心医院儿科2002~2003年26例铅中毒脑病患儿的临床资料及随访结果。结果 26例铅中毒脑病患儿临床均表现为频繁的抽搐伴意识障碍，中度以上贫血。伴呕吐23例、腹泻21例、发热11例、肝大5例。26例患儿治疗前血铅平均为8.486μmol/L，尿铅平均为3.598μmol/L，Hb均低于90g/L，最低55g/L。血钙均低于1.86mmol/L， 最低1.03mmol/L。脑脊液蛋白均增高，22例心肌酶谱增高，提示心肌损害明显。头颅CT/MRI仅1例脑室系统轻度扩张，全部病例经驱铅治疗后无一例死亡，1年后随访,继发性癫疒间4例，脑瘫4例。结论铅中毒脑病是铅中毒的严重类型，预后不良。对不明原因的无热惊厥者应注意及时行血铅检查，以便早期诊断，防止漏诊。 Abstract Objective Study the clinical characteristics and prognosis of lead poisoning encephalopathy.Methods We reviewed and analyzed the clinical data and the results of the 26 cases of lead poisoning encephalopathy.Results All the 26 cases had frequen convulsion,accompanied by disturbance of consciousness and above middle pitch of anemia.Among the 26 cases vomit ting was found in 23 cases,diarrhea in 21 cases,fever in 11 cases,live swelling in 5 cases.Supplemental examination:before treatment,the average of blood lead level was 8.486μmol/L，urine lead level was 3.598μmol/L,Hb was lower than 90g/ L,the lowest being 5g/L.The blood calcium was all lower than 1.86 mmol/ L,the lowest being 1.03 mmol/L.Protein of cerebrospinal fluid in all cases was over normal.Myocardial enzyme of 22 cases was over normal,suggesting myocardials injured obviously.Only 1 case was found ventricle system expanding lightly in CT/MRI.All cases had no death after treatment of driving lead.The 12 month follow up result suggested that second epilepsy was found in 4 cases and cerebral palsy in 4 cases.Conclusion The lead poisoning encephalopathy is the most serious type of the lead poisoning,and prognosis is not good. The patient of convulsion with unknown reason,and no fever,should be checked the blood lead for early diagnosis in order to prevent from leaking diagnosis. Key wordsLead poisoning;Encephalopathy;Clinical characteristics;Prognosis      

儿童急性白血病细胞周期蛋白D3、E的表达

目的 探讨细胞周期蛋白D3、E的表达与儿童急性白血病(AL)的关系。方法 采用免疫组织化学法，对广西医科大学第一附属医院儿科2002年1月至2004年5月收治的50例初发或复发儿童AL，其中急性淋巴细胞白血病(ALL)30例(高危ALL 12例，标危ALL 18例)，急性非淋巴细胞白血病(ANLL)20例，和26例完全缓解AL(AL CR)患儿，以及23例同期住院的非恶性疾病患儿(对照组)，检测其骨髓细胞中细胞周期蛋白D3、E的表达情况。结果 AL组中细胞周期蛋白D3、E阳性率分别为54%和46%，高于AL-CR组和对照组(P<0.01)；在ALL和ANLL间差异无显著性(P>0.05)。高危ALL细胞周期蛋白D3阳性率明显高于标危ALL(P<0.01)。化疗前细胞周期蛋白D3、E表达阳性率明显高于化疗缓解后(P<0.01)。细胞周期蛋白D3、E在AL组中的表达呈正相关(r=0.298，P<0.01)。结论 细胞周期蛋白D3、E与儿童AL发病有关，它们的表达存在相关性，可作为疗效观察的指标；细胞周期蛋白D3可能与患儿不良预后有关。 Abstract Objective To investigate the relationship between the expression of cyclin D3，cyclin E and childhood acute leukemia(AL).Methods The expression of cyclin D3 and cyclin E was detected in bone marrow cells from 50 cases of newly diagnosed or relapsing AL，in whom there were 30 cases of acute lymphoblastic leukemia(ALL，including 12 cases of high risk ALL and 18 cases of standard risk ALL ) and 20 cases of acute non lymphocytic leukemia(ANLL)，26 cases of complete remission AL(AL CR) patients and 23 cases of control group(no malignant disease) using immunohistochemical assay.Results 54% AL cases were positive for cyclin D3，and 46% for cyclin E，both being remarkably higher than that of AL CR group and control group(P<0.01).There was no difference between ALL and ANLL in the expression of cyclin D3 and cyclin E(P>0.05).The positive expression of cyclin D3 was higher in high risk ALL than that in standard risk ALL(P<0.01).The expressions of cyclin D3 and cyclin E before chemotherapy were significantly higher than that after remission using chemotherapy(P<0.01).There was significantly positive correlation between the expression of cyclin D3 and cyclin E in AL (r=0.5298，P<0.01).Conclusion In childhood AL，the expression of cyclin D3 and cyclin E is quite high，which is related to the development of AL and might be index of treatment effect.There is positive relation between cyclin D3 and cyclin E.Cyclin D3 might be related to poor prognosis. Key words Acute leukemia;Cyclin;Immunohistochemistry     

新生儿肺部疾病支气管肺泡灌洗液肺表面活性蛋白A的检测及意义

目的 探讨新生儿常见肺部疾病支气管肺泡灌洗液肺表面活性蛋白A(BAL SP-A)水平及其与临床的关系。方法 收集2000年1月至2003年2月在广州市儿童医院新生儿重症监护室住院的需行机械通气治疗的新生儿重症肺炎、胎粪吸入综合征(MAS)、急性呼吸窘迫综合征(ARDS)以及新生儿呼吸窘迫综合征(RDS)患儿共57例。测定其BAL SP-A水平，监测血气、PaO2/FiO2水平。结果重症肺炎组与MAS组患儿BAL SP-A水平无明显差异，但MAS组患儿PaO2、PaCO2及PaO2/FiO2水平较重症肺炎组明显降低(P值<0.01,<0.05,<0.05)；ARDS及RDS组患儿BAL SP-A水平均较上述两组低(P值均<0.001)，而RDS组患儿BAL SP-A水平较ARDS组低(P<0.001)，但ARDS组患儿PaO2水平较RDS组患儿低(P<0.05)。PS治疗组患儿的病死率较非PS治疗组明显降低(P=0.049)，其PaO2/FiO2与BAL SP-A水平密切相关(r=0.741,P=0.000)。结论 与重症肺炎患儿比较胎粪吸入综合征患儿BAL SP-A水平无明显降低；ARDS及RDS患儿BAL SP-A水平明显降低；BAL SP-A水平能反映新生儿肺损伤的严重程度，对于新生儿肺部疾病预后的判断有一定意义。 Abstract ObjectiveTo investigate the bronchoalveolar lavage (BAL) SP-A concentrations from newborn infants with lung disease,and to study the relationship between BAL SP-A and clinical outcome.Methods 57 cases of newborn infants with lung disease were admitted in our NICU between Jan.2000 and Feb.2003.BAL SP-A concentrations,PO2 value,PCO2 value,and PaO2/FiO2 ratio were measured.ResultsBAL SP-A concentrations did not differ between severe pneumonia group and MAS group,but the value of PaO2、PaCO2 and PaO2/FiO2 ratio in MAS group were significantly lower than that in severe pneumonia group (p respectively<0.001,<0.05,<0.05).BAL SP-A concentrations in RDS and ARDS groups were significantly lower than that in aforesaid groups ( all P<0.05).BAL SP-A concentrations in RDS group were significantly lower than that in ARDS group,but PaO2 value in ARDS group was lower significantly than that in RDS group( P<0.05).The mortality of infants treated with PS was significantly lower than that of infants treated without PS (P=0.049).PaO2/FiO2 ratio for the cohort was related to their BAL SP A concentrations ( r=0.741,P=0.000).Conclusion Surfactant protein A content in MAS is not different from that of severe pneumonia.BAL SP-A concentrations of neonates with ARDS or RDS decrease significantly.BAL SP-A concentrations can evaluate the severity of lung injury and the prognosis of neonatal lung disease. Key wordsInfant,newbornLung disease;Bronchoalveolar lavae;Surfactant protein A     

新生儿血培养中凝固酶阴性葡萄球菌细胞间粘附基因A和D的检测及临床意义

目的 探讨细胞间粘附基因(ica)A和icaD在新生儿凝固酶阴性葡萄球菌(CNS)败血症中的诊断意义。方法 收集北京儿童医院新生儿病房2001年11月至2003年3月间血培养为CNS的患儿为研究入选病例。通过应用聚合酶链反应(PCR)方法检测icaA及icaD的存在。结果 在80例入选患儿中，血培养均分离出CNS。根据新生儿败血症的诊断标准，临床诊断败血症27例(33.8%)，其中早产儿7例，足月小样儿2例。在菌种的分布上，表皮葡萄球菌18例(66.7%)，溶血葡萄球菌7例(25.9%)，人葡萄球菌2例(7.4%)。对90株CNS菌株应用PCR方法检测icaA及icaD，共有8株阳性，均为表皮葡萄球菌，并且icaA和icaD同时阳性。在经PCR扩增后所产生的图谱中，icaA在814bp，icaD在282bp。在ica阳性的8例病例中，临床诊断为败血症7例，一致率为87.5%，在临床排除败血症的53例患儿中，仅有1例ica阳性(1.8%)。ica的阳性预测值为259%，阴性预测值为981%。结论 ica基因PCR检测是临床诊断CNS败血症或导管相关感染的一种潜在的实验室方法。 Abstract Objective The purpose of this study was to assess whether the person making the clinical decision may benefit from the detection of icaA and icaD genes encoding putative virulent factors.Methods From Nov.2001 to Mar.2003,we detected the icaA and icaD genes in 80 neonates with a collection of clinical isolates from blood cultures by using a simple,rapid,and reliable PCR method.Results An overall total of 80 neonates with CNS strains from blood cultures were identified.There were 27 cases(33.8%)diagnosed neonatal sepsis clinically according to the standard,of which 7 were premature,2 were small for gestational age of full term infants.The distribution of species among the clinical CNS strains,18(66.7%)cases were Staphylococcus epidermidis,which was the leading cause,and then S.hemolyticus (n=7),S.hominis (n=2).There was a statistical difference in them.Of the 90 strains,eight were positive to icaA and icaD genes,with PCR products being obtained for the icaA and icaD genes in all of these strains,a 814 bp band for the icaA gene and a 282 bp band for the icaD gene.All of them were Staphylococcus epidermidis.Among 8 cases with positive ica genes,7 were diagnosed as sepsis clinically.The coincidence rate was 87.5%.Positive predictive value of the ica genes were 25.9%，and negative predictive value were 98.1%.Conclusion Ica genes may potentiate the clinical criteria used for the diagnosis of neonatal sepsis,and may discriminate between contamination and infections. Key words Coagulase negative staphylococcus;PCR;Newborn     

新生儿重症感染降钙素原检测的诊断价值

目的 探讨降钙素原(PCT)在新生儿重症感染时的诊断价值。方法 将自2002年3~12月于天津市儿童医院新生儿科住院的229例新生儿分别归入无感染对照组(116例)，全身感染组(39例)，局部感染组(51例)和病毒感染组(23例)。检测入院时血清PCT和C-反应蛋白(CRP),白细胞计数及分类。用SPSS 10.0 For Windows进行数据分析。结果 在全身感染时，血清PCT和CRP质量浓度升高均有显著性，但PCT显著性更高(P<0.001)。PCT≥2μg/L作为全身感染的诊断依据，其敏感度(0.804)和特异度(0.824)均优于CRP。结论 与CRP和白细胞计数相比,PCT是一个较好的新生儿全身细菌感染的诊断指标。 Abstract Objective The aim of this research is to evaluate the diagnostic value of PCT in neonates with severe bacterial infectious disease.Methods 229 neonates admitted to Neonatology Department,Tianjin Children’s Hospital since March to December 2002 were divided into 4 groups:non infection control(116),systemic bacterial infection (39),localized bacterial infection (51) and viral infection (23).Serum PCT and CRP were measured on admission;leucocyte count and differentiation was measured within 24 hours after admission.A serial measurement of PCT and CRP were taken during the course when necessary.Analyze data using SPSS 10.0 for Windows.Results Although both PCT and CRP significantly elevated in neonates with systemic bacterial infection,PCT revealed more sensitivity in systemic bacterial infection (P<0.001).The sensitivity and specificity of PCT to diagnose neonatal systemic bacterial infection using a cut off level 2 (μg/L) was 0.804 and 0.824,respectively.Conclusion PCT is a better diagnostic marker of neonatal systemic bacterial infection than CRP and leucocyte count. Key words Procalcitonin;Infection;C-reactive protein;Infant,neonate     

大剂量维生素C对川崎病急性期肱动脉血流介导扩张反应影响的临床观察

目的 观察大剂量维生素C(VitC)对川崎病(KD)急性期肱动脉血流介导的扩张反应(FMD)的影响，探讨VitC对KD急性期血管内皮功能障碍是否有改善作用。方法 采用高分辨率多普勒超声仪测定2001年1月至2002年12月在华中科技大学同济医学院附属同济医院儿科住院的36例KD急性期患儿(KD组)及15名健康儿童(正常对照组)肱动脉FMD，并比较KD组静脉滴注大剂量VitC(200mg/kg，最大量为4g)和安慰剂(10%葡萄糖)前后肱动脉FMD的变化。结果 KD组肱动脉FMD明显低于正常对照组(P<0.001)；KD组静滴大剂量VitC后肱动脉FMD明显增加(P<0.01)，静滴安慰剂后肱动脉FMD无明显增加(P>0.05)。结论 大剂量VitC能明显改善KD急性期肱动脉FMD，其对KD急性期血管内皮功能障碍可能有显著改善作用。 Abstract Objective To observe the effect of high dose vitamin C on flow mediated vasodilation(FMD) of brachial artery in acute phase of Kawasaki disease(KD) and investigate whether high dose vitamin C could improve vascular endothelial dysfunction in KD.Methods Using high resolution vascular ultrasound,we measured FMD of the brachial artery of 36 patients who were admitted to our hospital from Jan.2001 to Dec.2002. in acute phase of KD and 15 matched healthy subjects as controls.Studies were performed before and after intravenous infusion of high dose vitamin C (200mg/kg，max≤4g) or placebo in KD group.Results The FMD of the brachial artery in KD group was significantly lower than that in the control group (3.55±3.00% vs 10.34±4.29%，P<0.001).Intravenous infusion of vitamin C significantly increase the FMD of the brachial artery in 18 KD patients (6.97±2.93% vs 3.51±2.01%，P<0.01),while after administration of placebo in the other 18 patients,there was no significantly increased in the FMD(4.12±2.21% vs 3.75±3.00%，P>0.05).Conclusion Intravenous infusion of high dose vitamin C can increase the FMD of the brachial artery,and it can improve vascular endothelial dysfunction in KD. Key words Kawasaki disease;Vitamin C;Flow mediated vasodilation;Endothelial dysfunction     

大剂量氨甲蝶呤治疗小儿急性淋巴细胞白血病水化和碱化方法的探讨

目的 探讨进一步降低大剂量氨甲蝶呤(HD-MTX)治疗小儿急性淋巴细胞白血病(ALL)毒副反应的方法。方法 对2000年1月至2001年12月在上海儿童医学中心血液肿瘤科住院治疗的47例ALL患儿共进行的134例次HD-MTX治疗，按照两种不同的水化、碱化方法，分为全国标准治疗组和观察组。观察患儿血清中的MTX浓度、治疗效果及毒副反应的发生率。结果 (1)按WHO相关分度标准，各种毒副反应的发生率、Ⅲ度或Ⅲ度以上的毒副作用者的发生率两组比较差异有显著性意义。(2)观察组有2例次出现尿素氮的升高，标准治疗组未出现。(3)在应用HD-MTX开始后44h、68h时血清MTX浓度异常者两组相比较差异有显著性意义。结论 观察组HD-MTX的毒副反应发生率及严重程度均明显低于全国标准治疗组，且减少治疗费用,而不影响HD-MTX治疗效果。 Abstract Objective To study the ways to further reduce the toxic side effects of the HD-MTX in the treatment of childhood acute lymphoblastic leukemia.Methods The patients were divided into two groups:the testing and the treatment group.Two different ways of hydration and alkalization were used in the course of HD-MTX to evaluate the treatment results.Monitor the dynamic changes of the serum MTX level and occurrence of the toxic side effects.Results The results showed:(1)comparing the above toxic side effects occurred in both groups,significant difference could be found;(2)blood urea nitrogen(BUN) increased in 2 cases in the testing group but no one happened in the treatment group;(3)the occurrence of the serum MTX level abnormalities after 44 and 68 hours of HD-MTX treatment appeared in both groups with significant difference.Conclusion It shows the protocol for hydration and alkalization in the testing group has greater advantage than that in the treatment group in reducing the toxic side effects.This may decrease the cost of treatment,but may not influence the treatment effect of HD-MTX. Key words Methotrexate;Leukemia;Lymphoblastic;AcuteSide effect     

法洛四联症根治术后急性肺损伤的治疗

目的 分析法洛四联症(TOF)根治术后急性肺损伤(ALI)的原因，总结治疗体会。 方法 2003年7月至2004年6月，上海第二医科大学附属新华医院上海儿童医学中心心胸外科共行TOF根治术161例，发生ALI 5例。记录5例ALI患儿术前经皮氧饱和度(SpO2)、红细胞压积比(HCT)、McGoon比值、肺动脉指数(PAI)和手术、体外循环(CPB)资料，以及ALI发生时间、ALI发生后各项治疗措施、各治疗阶段肺功能指标、计算其肺损伤分数。 结果 5例ALI患儿，年龄7~24(13.8±3.1)个月，体重7.0~9.5(8.2±0.5)kg，均在静吸复合麻醉CPB下进行，术毕给予改良超滤，平均CPB时间(67.2±5.9)min，主动脉阻断时间(43.6±2.4)min，ALI发生率为3.1%，发生时间为术后8~60h，死亡2例。5例均施行腹膜透析术(PD)，2例给予一氧化氮吸入，1例给予肺表面活性物质替代治疗。各治疗阶段肺功能指标逐步改善，至治疗后期，肺功能指标在统计学上有显著性差异(P<0.05)。 结论 TOF术后ALI治疗困难，合理机械通气、尽早维持体液平衡、改善肺通气和氧合功能有利于及时阻断低氧酸中毒导致的恶性循环。 Abstract Objective To analyse the reasons of acute lung injury (ALI) after the surgical correction of tetralogy of Fallot (TOF) and summarize the postoperative treatment skill.Methods There were 161 cases of patients with TOF who underwent corrective repair operation in the Department of Cardiovascular Surgery of Xinhua Hospital/ Shanghai Children’s Medical Center from Jul.2003 to Jun.2004,in which there were 5 patients who developed ALI after the surgical correction of TOF.The mean age was 13.8±3.06 months (7~24 months) and the mean body weight was (8.20±0.46)kg (7~9.5kg).Before operation,their mean SpO2 was (72.8±2.2)% (67%~80%),mean HCT was (49.0±5.5)%,mean McGoon ratio was (1.27±0.05) and mean PAI was (136.0±16.8)mm2/m2.The total cardiopulmonary bypass (CPB) time and aortic clamp time were (67.2±3.9) min and (43.6±2.4) min,respectively.The modified ultrafiltration was used throughout the CPB.The treatment and the blood analysis were recorded when the ALI developed.The parameters of pulmonary function and lung injury score were calculated.Results ALI developed (30.0±9.7) hrs after the operation and the incidence of ALI after the surgical correction of TOF was 3.1%.Two cases died within 48 hrs.Peritoneal dialysis (PD) was performed on all the cases.Meanwhile,2 cases were given inhaled nitric oxide (NO) and 1 case was given pulmonary surfactant (PS).The parameters of pulmonary function were improved step by step and the differences were statistically significant at the latest stage of treatment (P<0.05).Conclusion The treatment of ALI after surgical correction of TOF includes reasonable mechanical ventilation and maintaining the balance of fluid.Inhaling NO and using PS will be benefitial to improve the pulmonary function and to stop the vicious circle which results in the hypoxemia and refractory acidosis. Key wordsTetralogy of Fallot;Acute lung injury;Cardiopulmonary bypass     

Retrospective analysis of outcome of pregnancy in women with congenital heart disease: Single-centre experience from North India

Objective: To study maternal and perinatal outcome in congenital heart disease (CHD) and to compare outcome between cyanotic and acyanotic CHD.
Method: A retrospective analysis of 196 cases of CHD was undertaken, and maternal and perinatal outcome of pregnancy was compared in cyanotic and acyanotic cases and between surgically corrected and uncorrected cases.
Results: Maternal and perinatal outcome was better in the acyanotic group. Maternal complications included higher incidence of cardiac complications in cyanotic group, (33.3% vs 3.4% in acyanotic group, P  = 0.001), abruption (12.5% vs nil) and pregnancy-induced hypertension (16.6% vs 5.2%). Rate of prematurity (25% vs 11.6%), intrauterine growth retardation (50% vs 15.1%, P  = 0.003) and abortion (4.1% vs 2.1%) was higher in cyanotic group. Mean gestational age at delivery was better in corrected group, 37.13 vs 34.93 weeks in uncorrected group. There was no case of infective endocarditis. There were four cases of maternal mortality in cyanotic group, two of which were in women with Eisenmenger syndrome. In acyanotic heart disease one case died undelivered and one died on first postoperative day.
Conclusion: Maternal and perinatal outcome is better in acyanotic CHD compared to cyanotic CHD. Surgical correction of cardiac lesions prior to conception improves outcome.     

Place de l’échocardiographie fœtale au premier trimestre pour les situations à risque accru de cardiopathie congénitale

ObjectiveTo describe the preliminary results of fetal cardiac scanning in the first trimester of pregnancy for fetuses at higher risk for congenital heart defect (CHD).Patients and MethodsEchocardiographic examination was performed at 12 to 14 + 6 in 67 patients at higher risk for fetal CHD. The indications for referral were: increased nuchal translucency more or equal to 99^e centile (56 cases), risk of recurrence for CHD (eight cases), embryonic toxic exposure (eight cases), maternal disease (four cases).ResultsComplete examination of the fetal heart was possible in 54/55 cases. Fetal cardiac examination was normal in 58 cases (87 %). Six cases of severe CHD were diagnosed: hypoplastic left heart syndrome, pulmonary atresia with intact septum, complex univentricular CHD leading to termination of pregnancy, conotroncal Fallot like abnormality, and transposition of great arteries. Post-mortem examination was performed in three cases and confirmed the cardiac anomalies.Discussion and conclusionOur preliminary results confirmed that fetal heart scanning is feasible at the end of the first trimester of pregnancy. Severe CHD are amenable to prenatal diagnosis in the first trimester in the population at higher risk for CHD.     

Obstetric and perinatal outcomes in pregnancies with isolated foetal congenital heart abnormalities

Objective: To determine obstetric, intrapartum, and perinatal outcomes for pregnancies with isolated foetal congenital heart defects (CHDs).

Methods: This was a retrospective cohort study of women that delivered an infant with an isolated major CHD between January 2010 and April 2017 at a major Australian perinatal centre. The study cohort was compared with a cohort of women with infants without CHD. Cardiac abnormalities were broadly subdivided into the following five categories using the International Classification of Diseases Tenth Revision (ICD-10) as a guide – transposition of the great arteries (TGA), septal defects, right heart lesions (RHL), left heart lesions (LHL), and “other”. Demographic characteristics and obstetric, intrapartum, and perinatal outcomes were compared between the two cohorts.

Results: The final study cohort comprised of 342 infants with isolated CHD and 68,911 controls. Of the infants with CHD, 20.4% (70/342) had transposition of the great vessels, 23% (79/342) had septal lesions, 14.6% (50/342) had right sided lesions, 23.3% (80/342) left sided, and 18.4% (63/342) categorised as “other”. Women with foetal CHD had a higher BMI and had higher rates of cardiac disease, diabetes mellitus, and hypertension, be smokers and consume alcohol compared to controls. The CHD cohort had lower odds of spontaneous vaginal delivery (SVD) (OR 0.73, 95%CI 0.58–0.90) and higher odds of caesarean for nonreassuring foetal status (aOR 1.65, 95%CI 1.07–2.55), birth weight <5th (aOR 3.44, 95%CI 2.38–4.98) and <10th (aOR 2.49, 95%CI 1.82–3.40) centiles, neonatal intensive care unit (NICU) admission (aOR 109.14, 95%CI 74.44–160.02), severe respiratory distress (aOR 2.90, 95%CI 2.33–3.76), 5?minutes Apgar score <7 (aOR 2.48, 95%CI 1.46–4.20), severe acidosis (aOR 1.80, 95%CI 1.14–2.85), stillbirth (aOR 4.09, 95%CI 1.62–10.33), neonatal death (aOR 24.30, 95%CI 13.24–44.61), and overall perinatal death (aOR 13.42, 95%CI 8.08–22.30). Infants with TGA had the lowest overall risk of complications whilst infants with RHL, LHL, and “others” had the highest risk of adverse outcomes, particularly death.

Conclusion: Infants with CHD have overall worse obstetric and perinatal outcomes compared with controls. Infants with TGA have the best perinatal outcomes of all the CHD subcategories.     

Cardiovascular profile score as a predictor of acute intrapartum non-reassuring fetal status in infants with congenital heart defects

Objectives: To investigate the predictive factors of urgent cesarean delivery (CD) due to acute intrapartum non-reassuring fetal status (NRFS) in infants with congenital heart defects (CHDs).

Study design: This was a retrospective review of 199 singletons prenatally diagnosed with a CHD and for whom vaginal delivery was attempted in our institution between 2007 and 2014. A cardiovascular profile (CVP) score was used to assess fetal heart failure.

Results: The number of urgent CDs due to NRFS was 37 (18.6%). Fetuses with a CVP score ≤7 were significantly more likely to require urgent CD due to NRFS than those with a CVP score ≥8 (p?p?=?0.017). Multivariate analysis showed that a CVP score ≤7, a birth weight <2500?g, and primipara status were significant predictors of urgent CD due to NRFS.

Conclusions: Fetal heart failure, low birth weight, and primipara status were revealed to be independent predictors of urgent CD due to acute intrapartum NRFS in CHD infants. The CVP score may be a useful echocardiographic marker in perinatal management planning.     

Does prenatal diagnosis of critical congenital heart diseases influence the prereferral mortality in a center without surgical intervention?

Background: Prenatal diagnosis ameliorates some preoperative and postoperative outcomes in critical congenital heart disease (CHD). Despite large variability among anatomical defect types, nearly half of them are diagnosed antenatally. We aimed to investigate the effect of the antenatal diagnosis on prereferral mortality of infants with critical CHD in a center without cardiovascular surgery clinic.

Methods: Medical records of the neonates who were diagnosed with critical CHD between the years 2010 and 2016 in Zekai Tahir Burak Women’s health Education and Research Hospital were retrospectively reviewed for the study. Patients were divided in two groups as prenatal and postnatal regarding the time of diagnosis. Groups were compared in terms of demographical, clinical characteristics, and prereferral mortality rates.

Results: Seventy seven neonates were included in the study. Of those, 39 (50%) infants had prenatal diagnosis. Most common types of CHD were tetralogy of Fallot (TOF) with pulmonary atresia, hypoplastic left heart syndrome (HLHS), and transposition of the great arteries (TGA), respectively. Demographical and clinical characteristics were similar between the groups. Minor congenital anomalies were more common in the patients with prenatal diagnosis (41 versus 18%, p?=?.021). Blood gas parameters were similar except pCO₂ levels, which were slightly lower in the postnatal diagnosis group (p?=?.048). There was no difference with regard to prereferral mortality between prenatal and postnatal diagnosed infants.

Conclusions: Prenatal diagnosis may not be always associated with improved survival in critical CHD in a center without immediate surgical intervention opportunity.     

Pregnancy outcomes and relative risk factors among Chinese women with congenital heart disease

ObjectiveTo summarize pregnancy outcomes among a cohort of Chinese women with congenital heart disease (CHD) and to identify predictive risk factors for maternal, fetal, and neonatal complications.MethodsA retrospective analysis of 529 women with CHD who gave birth at the Shanghai Obstetrical Cardiology Intensive Care Center, Shanghai, China, between January 1, 1993, and September 30, 2010. Maternal, fetal, and neonatal complications were evaluated for these women.ResultsMaternal cardiac complications were reported in 33 (6.2%) of the women, whereas fetal and neonatal complications were reported in 145 (27.4%). The factors found to be significantly predictive for maternal cardiac complications were cardiac events before pregnancy (odds ratio [OR] 36.811), New York Heart Association functional class greater than II (OR 27.702), oxygen saturation below 90% (OR 7.506), and left ventricular obstruction (aorta stenosis) (OR 2.408). Factors significantly predictive for fetal and neonatal complications were New York Heart Association functional class greater than II (OR 20.12), oxygen saturation below 90% (OR 3.78), and pulmonary arterial hypertension (systolic pressure ≥ 50 mm Hg) (OR 3.42).ConclusionIdentification of maternal, fetal, and neonatal complications among women with CHD may guide medical intervention and therefore reduce pregnancy-associated risk for these patients.     

新生儿先天性心脏病145例分析

目的　了解新生儿先天性心脏病（简称先心病）的临床特点和预后。　方法　对１９９１年～１９９７年新生儿病房所收治的１４５例先心病进行分析，并随访２４例非青紫型先心病患者３个月～７年。　结果　非青紫型先心病９３例（６４．１％ ），青紫型５２例（３５．９％ ），最常见者依次为室间隔缺损（４４．１）％ 、动脉导管未闭１３．８％ 、大动脉转位（９．７％ ）。１３．１％ 合并有心外畸形，以染色体畸形居首位３１．６％ 。比较青紫型与非青紫型的临床表现（青紫型％ 、非青紫型％ ）：生后２４小时内出现青紫（４２．０％ 、０％ ）、气促（７０．０％ 、５９．０％ ）、心率快（４０．０％ 、２３．０％ ）、心力衰竭（４８．０％ 、１４．０％ ）、心脏杂音（７６．０％ 、８９．０％ ）、低氧血症（８２．６％ 、９．８％ ）、代酸（２１．７％ 、８．５％ ）、住院时的病死率（３６．５％ 、０％ ）、２例青紫型先心病在新生儿期作了手术，放弃治疗（５１．９％ 、１１．８％ ）。随访非青紫型者２４例，２例死亡、５例手术、１２例自愈、５例未愈无合并症。　结论　先心病是婴幼儿死亡的主要原因。现国外已用胎儿超声心动图作产前诊断并对重症终止妊娠，国内应在产科中广泛开展此项检查     

Effect of maternal congenital heart defects on labor and delivery outcome: A population-based study

Objective. The primary objective of this study was to characterize the delivery outcome of parturients with congenital heart defects (CHD), from maternal and from neonatal perspectives.

Study design. A retrospective population-based study was conducted, covering a 13-year period (1989–2002) with an aggregate of 151 487 deliveries of all women with and without CHD. Maternal demographics, obstetrical and medical history, delivery outcome, and neonatal outcome were drawn from a computerized perinatal database.

Results. Sixty-seven women with CHD had 156 deliveries. The severity of CHD, based on the New York Heart Association (NYHA) classification, was I or II in 99.1% of the deliveries. CHD patients had significantly higher rates of labor induction and neonatal malformations. Maternal CHD was discovered as an independent risk factor associated with neonatal malformations (OR 2.10, 95% CI 1.18–3.72). No significant differences were noted between women with CHD and the controls regarding maternal morbidities and Apgar scores.

Conclusions. The labor outcome of CHD patients with NYHA classification I and II resembles that of non-CHD women in a tertiary center setting. Neonates of CHD mothers have higher rates of congenital malformations even among asymptomatic or mildly symptomatic mothers. A careful sonographic follow-up is warranted among all pregnancies of CHD patients.     

Nuchal translucency and cardiac abnormalities in euploid singleton pregnancies

Abstract

Objective: To investigate different cut-off levels of nuchal translucency (NT) to predict abnormal cardiac findings (ACF) in second trimester ultrasound examination and confirmed postnatal congenital heart defects (CHD) in euploid pregnancies.

Methods: A retrospective analysis was performed on singleton pregnancies examined in our ultrasound units from 2006 to 2011. Fetuses with an abnormal karyotype were excluded. Different cut-off levels of NT thickness were analyzed to evaluate its performance to detect the ACF on second trimester ultrasound (2nd US) examination and also the CHD detected in neonatal follow-up evaluation of ACF cases.

Results: Of the 12?840 cases, a total number of 8541 euploid pregnancies were included in the study. Thirty-three had ACFs detected by 2nd US (3.86/1000). The mean NT thickness was found to be higher in fetuses with ACFs (p?<?0.0001). Of 33 ACFs, 17 (52%, 1.99/1000) had major CHDs in neonatal follow-up. The area under the ROC curves for NT thickness to predict ACFs and CHDs were 0.67 and 0.65, respectively.

Conclusions: Higher NT thickness is associated with higher risk of ACF. NT is a weak predictor of ACF and major CHD; however, fetuses with an unexplained increase in NT measurement should be referred for further cardiac investigations.