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     

新生儿青紫型先天性心脏病156例的早期诊治分析

目的 了解青紫型先天性心脏病在新生儿期的临床表现、分布情况、治疗和转归。方法 1999年11月至2004年7月上海儿童医学中心对156例青紫型先天性心脏病(CHD)新生儿进行临床分析。结果 (1)青紫型CHD在新生儿期主要表现为中央性紫绀、心功能不全，个别可合并心律紊乱。(2)新生儿期青紫型CHD以完全性大动脉转位(TGA)居首位，其他依次为肺动脉闭锁(PA)、完全性肺静脉异位引流(TAPVC)和肺动脉狭窄(PS)。(3)内科治疗包括强心、利尿、扩血管药物和前列腺素E1(PGE1)的运用；156例中57例施行外科手术，其中以TGA最多。结论新生儿青紫型CHD心脏畸形复杂，病情多危重，在生后尽早明确诊断并给予适当的内外科治疗有助于提高其生存率和改善预后。 Abstract Objective To investigate the clinical manifestation,distribution,treatment and prognosis of congenital heart disease in neonatal period.Methods Analyze 156 cases of neonatal cyanotic congenital heart disease clinically.Results ① Neonatal cyanotic CHD was manifested with central cyanosis,heart failure,and arrhythmia occasionally.② D TGA stand first on the list of neonatal cyanotic CHD and the others ordinal was PA,TAPVC and PS.③ Internal medical therapy included using of cardiotonic,diuretic and prostaglandin E157 patients were given surgical operations and TGA lead the first.Conclusion For complex anomaly and critical condition in most cases,neonatal cyanotic CHD should be diagnosed as soon as possible and treated appropriately in order to improve the viability and prognosis of these neonates. Key words NeonateHeart disease;Congenital     

圆锥动脉干畸形室间隔缺损的三维超声定量研究

目的 探讨三维超声心动图(3DE)在圆锥动脉干畸形(CTD)室间隔缺损(VSD)定量研究中的准确性及其临床意义。 方法 2000年5月至2003年11月在上海儿童医学中心住院且二维超声心动图(2DE)、经胸3DE、心血管造影及手术等定量测量结果完整的CTD患儿共63例，将3DE测量结果与2DE、造影及手术测值对比。 结果 方差分析、SNK均数间两两比较及相关分析显示，3DE VSD长径测值为1.69cm(从右室面)和1.63cm(从左室面)，虽较手术测值(1.84cm)低估，但低估程度小于2DE测值(1.27cm)及造影测值(1.12cm)；3DE与手术测值的相关性(r=0.74)明显高于2DE(r=0.39)及造影测值(r=0.25)。3DE VSD短径测值与造影测值相接近，两者比较无统计学差异(P＞0.05)。3DE VSD短径测值与2DE及造影测值的相关性(r=0.45，r=0.54)高于3DE VSD长径测值与2DE及造影测值的相关性(r=0.37，r=0.44)。3DE VSD面积测值与手术测值呈良好相关(r=0.57)。 结论 3DE可作为CTD VSD定量分析的又一无创手段，能提供较2DE更准确的定量诊断。 Abstract Objective To evaluate the accuracy and clinical value of transthoracic three dimensional echocardiographic (3DE) quantitative analysis in ventricular septal defect (VSD) of conotruncal defects (CTD).Methods From May 2000 to November 2003 in Shanghai Children Medical Center,there were 63 inpatients of CTD with all quantitative findings measured by 3DE,two dimensional echocardiography (2DE),angiocardiography and surgical findings.Their measurements of VSD sizes and areas with 3DE were compared with those with 2DE,angiocardiography and surgical findings.Results From the analysis of variance,Student Newman Keuls (SNK) test and correlation analysis,the measurements of VSD long diameter (mean 1.69cm in en face view of ventricular septa from right ventricle and 1.63 cm in en face view of ventricular septa from left ventricle) with 3DE was less than the measurements of VSD diameter (mean 1.84 cm) with surgical findings,but they were better correlated with those with surgical findings (r=0.74) than the measurements of VSD diameter (mean 1.27 cm) with 2DE (r=0.39) and those (mean 1.12 cm) with angiocardiography (r=0.25).However,the measurements of VSD short diameter with 3DE was close to those with angiocardiography,and there was no significant difference (P>0.05) between them.The measurements of VSD short diameter with 3DE were better correlated with those with 2DE and angiocardiography than the measurements of VSD long diameter with 3DE.Meanwhile,the measurements of VSD area with 3DE were well correlated with the measurements of VSD diameter with surgical findings (r=0.57).Conclusion 3DE can provide more accurate quantitative diagnosis in CTD VSD than 2DE. Key words Conotruncal defects;Three dimensional echocardiography;Two dimensional echocardiography;Ventricular septal defect     

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      

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     

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     

儿童横纹肌肉瘤8例临床分析

目的 分析儿童横纹肌肉瘤的临床特点，提高其诊治水平。方法 对中国人民解放军总医院1998年1月至2002年10月收治的8例横纹肌肉瘤患儿临床资料进行分析。结果 8例患儿中，男5例，女3例；发病年龄7个月至11岁，<5岁者3例；依据美国横纹肌肉瘤研究组(IRS)的分期标准I期2例、Ⅱ期2例、Ⅲ期1例，Ⅳ期3例；原发于头颈部4例、四肢2例，泌尿、消化系统各1例。病理类型:胚胎型7例,腺泡型1例。临床表现随原发部位不同有很大差异，基本为肿瘤组织占位、侵蚀后引起。明确诊断依靠病理，治疗以手术、化疗和放疗相结合，放疗总量40~50Gy，化疗共有3种方案:VDCA、VAC和VadrC。对Ⅰ、Ⅱ期手术彻底切除肿瘤患儿行VAC、VadrC方案交替共2年，Ⅲ、Ⅳ期患儿前14周用VDCA方案，后VAC、VadrC方案交替共2年。全部患儿2年生存率为37.5%(3/8)。结论 儿童横纹肌肉瘤是多发于头颈部，病理多表现为胚胎型的极度恶性软组织肿瘤，早期诊断，合理应用手术、化疗与放疗结合的综合治疗，是提高该肿瘤患儿生存率的关键。 Abstract Objective To analyze the clinical characteristics of the childhood rhabdomyosarcoma.Methods Clinical data was analyzed in 8 children with rhabdomyosarcoma.Results From Jan,1998 to Oct,2002,eight children with rhabdomyosarcoma had been diagnosed in our department,5 boys and 3 girls,7 months to 11 years old.Primary site was head and neck (n=4),extremities(n=2),kidney (n=1),bile duct(n=1).2,2,1,3 patients were in stage Ⅰ,Ⅱ,Ⅲ,Ⅳ respectively.Pathological data revealed embryonal rhabdomyosarcoma(n=7) and alveolar rhabdomyosarcoma(n=1).The clinical manifestation was mass in primary site or the tumor erodes.All patients had been diagnosed pathologically.The treatment included multimodal therapy with combination of surgery,chemotherapy,and radiation,whose doses were 40 to 50 Gy.Chemotherapy was given to all patients with VDCA、VAC or VadrC.The patients with pre Stage II have received chemotherapy with protocol of VAC or VadrC for two years.The protocol of VDCA has been used to the patients with Stage Ⅲand Ⅳ for 14 weeks,then the VAC or VadrC has been used until 2 years later.Two year EFS was 37.5%.Conclusion The head and neck are the most common primary site for rhabdomyosarcoma in children,with mainly histological embryonal rhabdomyosarcoma.Early diagnosis and combination therapy including surgery,chemotherapy,and radiation are key for those with rhabdomyosarcoma to get long EFS. Key words Rhabdomyosarcoma;Children;Treatment     

小儿复杂上尿路感染的因素及致病菌分析

目的 了解小儿复杂上尿路感染的因素及致病菌的耐药现状。方法 1999年1月至2004年3月在复旦大学附属儿科医院住院治疗的复杂上尿路感染患儿178例，分析其尿培养阳性的因素、致病菌分布及其对抗生素的耐药性。结果 复杂上尿路感染患儿的年龄以≤1岁为主，占46.6%。致病因素中以膀胱输尿管反流为主，占61.8%,40例肾瘢痕患儿中有92.5%存在反流。复杂上尿路感染致病菌中以大肠埃希菌为主，占33%，但与单纯上尿路感染相比所占比例明显下降(P<0.001)；克雷白杆菌与绿脓假单孢菌感染所占比例明显高于单纯上尿路感染所占比例(分别P<0.01,P<0.05)。产超广谱β-内酰胺酶(ESBLs)菌株占革兰阴性杆菌的28%，以大肠埃希菌易产生ESBLs。革兰阴性杆菌对未加β-内酰胺酶抑制剂的青霉素类及第1、2、3代头孢菌素类抗生素耐药率均高，而ESBLs菌株交叉耐药情况更为严重。结论 复杂上尿路感染以婴儿期多见，膀胱输尿管反流是复杂上尿路感染及肾瘢痕的主要因素，大肠埃希菌仍是其主要致病菌，但克雷白杆菌、绿脓假单胞菌比例明显上升，致病菌耐药性高，产ESBLs菌株多重耐药严重。 Abstract Objective To investigate the factors and resistance of the pathogens of complicated upper urinary tract infection (UTI) in children.Methods Distribution and resistance of pathogens,complicated factors in 178 urinary culture positive UTI patients hospitalized from 1999 to 2004 were analyzed.Results Infants with complicated upper UTI accounted for 46.6%.Among the complicated factors,vesicoureteric reflux (VUR) accounted for 61.8%,and 37 of 40 children with venal scar had VUR.E.coli in the complicated upper UTI accounted for 33% of all the pathogens,which was significantly lower than in simple upper UTI (P<0.001)However,Klebsiella and Pseudomonas aeruginosa accounted for 15.8% and 8.1% respectively,which were significantly higher than in simple upper UTI (5.6% and 2.4% respectively,P<0.01 and 0.05 respectively).Nearly 28% of the Gram negative bacilli were the ESBLs producing strain,most of which were E.coli.Gram negative bacilli had a high resistance rate towards not only the penicillin uncombined with the beta lactamases inhibitors but also the first,second and third generations of cephalosporins.Furthermore,the multi cross resistance of the ESBLs strains was even more serious.Conclusion The complicated upper UTI is predominant in infants and VUR induces complicated upper UTI and renal scar.E.coli is still the major pathogen,while the proportion of Klebsiella,Pseudomonas aeruginosa are significantly increasing.The pathogens have high drug resistance and the ESBLs producing strains have serious multi resistance. Key wordsUpper urinary tract infectionPathogenResistance     

Analysis of the human zona pellucida during culture: Correlation with diagnosis and the preovulatory hormonal environment

Purpose: The objective of this study was to analyze sequentially the human zona pellucida changes in an in vitro fertilization program as it relates to several variables. Methods: The zona pellucida thickness was measured daily in zygotes and cleavage-stage embryos on a Nikon inverted microscope equipped with Hoffman modulation contrast optics, using an ocular micrometer. A total of 512 embryos from 96 patients was evaluated. Results: There was a highly significant direct correlation between zona thickness and preovulatory estradiol and basal day 3 FSH levels (P < 0.02 andP < 0.0006, respectively). This relationship showed a rapid reversal following 48 hr of culture; embryos from patients with the highest FSH levels had thinner zonae prior to transfer (P < 0.0007). The zonae from patients with unexplained infertility were thicker (19.4 ± 2.7 μm) than those from patients with endometriosis (17.7 ± 2.2 μm), tubal (17.5 ± 2.4 μm), or male-factor infertility (16.4 ± 2.7 μm) (P < 0.0001) on the first day of culture. Conclusions: We hypothesize that the thickness of the human zona pellucida is influenced by the preovulatory hormonal environment and diagnosis. These factors should be considered as part of the embryo quality evaluation prior to transfer or when assessing the possibility of using assisted hatching. More studies are needed to understand the factors regulating the thickness of the human zona pellucida.     

Survivin在儿童急性白血病中的表达和P53的相关性及其临床意义

目的探讨Survivin在儿童急性白血病(AL)中的表达及其与抑癌基因P53的相关性，同时观察Survivin与急性淋巴细胞白血病(ALL)临床分型的关系。方法应用免疫组织化学链霉亲和素-生物素-过氧化物酶复合物(SABC)方法，检测华中科技大学同济医学院附属同济医院儿科2004年1~7月收治的37例儿童AL骨髓细胞Survivin和P53的表达，对照组为10例非恶性血液病且骨髓正常患儿。结果在37例AL患儿中，Survivin蛋白表达阳性率为35.14%，P53表达阳性率为32.43%，显著高于正常骨髓组织(P均<0.05)，Survivin和P53表达呈强相关性(P<0.05)，Survivin在高危(HR)ALL患儿的表达为46.15%，在标危(SR)ALL患儿的表达为0，差异有显著性意义(P<0.05)。结论(1)Survivin在儿童AL中异常表达，且与ALL的临床分型有关，提示其在判定儿童ALL预后中有意义；(2)抑癌基因Ｐ53的失活与Survivin的表达可能在AL发生中起协同作用。 Abstract Objective To investigate the expression of Survivin in pediatric acute leukemia and its relationship with expression of P53 and clinical significance.Methods The expression of Survivin and P53 proteins were detected by immunohistochemical assay in 37 acute leukemia children.The control included 10 children with normal bone marrow.Results Survivin protein expression rate was 35.14% and P53 protein expression rate was 32.43% in 37 AL children.Both of them were significantly higher than that in control group (P<0.05).The expression of Survivin and P53 being strongly related (P<0.05).The expression rate of Survivin was 46.15% in HR ALL and 0% in SR ALL,the difference was statistically significant(P<0.05).Conclusion (1)Survivin protein expression rate is abnormal and is associated with the clinical typing of ALL.It may play an important role in the prognosis of ALL.(2)Expression of Survivin and de activation of anti oncogene P53 might play synergetic roles in the process of genesis of AL. Key words Survivin;P53;Children;Acute leukemia     

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.     

Perinatal outcomes of congenital heart disease after emergent neonatal cardiac procedures

Purpose: We compared outcome of neonates with prenatal and post-natal diagnosis of congenital heart disease presenting in our paediatric heart centre between March 2005 and May 2015 who underwent an emergent intervention within 48?h post-partum.

Materials and methods: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized foetal echocardiography, diagnosis was made post-natally. In 98/111 (88.2%), 30-day outcome was known.

Results: Regarding the entire cohort, 30-day survival did not differ significantly in prenatal and post-natal diagnosis group (71.2 vs. 72.9%; p?>?.1). Infants with prenatal diagnosis were more likely to be born by caesarean section (59.6% vs. 33.9%, p?=?.01). Those with post-natal diagnosis had a higher need for intubation (32.7% vs. 52.5%; p?p?=?.075).

Conclusion: For newborns who require emergent neonatal cardiac procedures, our results point towards a lower death rate after prenatal diagnosis of HLH/HLHC.     

MRI features and differential diagnoses of congenital vaginal atresia

Objective: To investigate the MRI manifestations of congenital vaginal atresia, analyze its imaging features, and improve the understanding of the disease.

Methods: MRI findings and clinical data of 12 patients with congenital vaginal atresia confirmed by hysteroscopy and laparoscopic surgery were retrospectively analyzed. Vaginal atresia was classified according to vaginal dysplasia in AFS female genital malformation classification system.

Results: In this study, 12 cases of congenital vaginal atresia were diagnosed by combined preoperative MRI with operative diagnosis. Among them, 10 patients all had type-I congenital vaginal atresia, and their uterus and cervix were normal (1 patient had ectopic renal malformation combined with left ovarian endometriosis cyst and 1 patient with uterine empyema). The other two cases were diagnosed congenital vaginal atresia type II (1 case merged with residual uterus, 1 case with cervical dysplasia). MRI mainly manifested as dilatation and hemorrhage in the uterine cavity, cervical canal and vaginal upper segment. T1WI showed high signal, T2WI showed slightly lower and slightly higher signal. The dilated vagina was above the perineal level.

Conclusion: MRI features of congenital vaginal atresia have certain characteristics. MRI cannot only accurately assess the type of vaginal dysplasia and its associated complications, but also make objective evaluation and diagnosis, so it can be used as the best effective preoperative image evaluation.     

Pregnancy outcomes in women with heart disease: Experience of a tertiary center in the Netherlands

ObjectivesClinical data of pregnant women with heart disease were obtained with the intention to provide input for local counseling and management guidelines.Study designRetrospective data from all pregnant women with congenital or acquired heart disease between 2000 and 2011 in the VU University Medical Centre Amsterdam.Main outcome measuresMaternal and neonatal outcomes were evaluated.ResultsData of 122 women with 160 pregnancies were obtained. The most common heart diseases were congenital heart disease (n = 65, 53.3%) and arrhythmia (n = 20, 16.4%). Based on the functional criteria of the New York Heart Association (NYHA), 114/122 patients (93.4%) were classified NYHA class I–II. Patients in NYHA class III–IV (n = 8/122, 6.6%), mainly had a history of myocardial infarction or pulmonary hypertension. There were 156 singleton and 4 twin pregnancies. 22 (13.5%) pregnancies were complicated by hypertensive disorders. Heart failure developed in 11 women (9.0%), 37.5% in NYHA class III–IV and 6.5% in NYHA class I–II. Mean gestational age and birth weight were 270 days and 3196 g in NYHA class I–II compared to 237 days and 1972 g for NHYA class III–IV. There were two maternal deaths (1.6%) and 5 fetal deaths (3.1%). There were 29 (12.8%) preterm births, 20 (12.8%) neonates small for gestational age and 34 (21.8%) admittances on the Neonatal Intensive Care Unit (NICU).ConclusionsPregnancy in women with pre-existing heart disease in all NYHA classes is associated with increased maternal morbidity and perinatal morbidity. Risk of structural fetal anomalies is especially high in women with congenital heart disease.     

Perceived Sexual Difficulties and Sexual Counseling in Men and Women Across Heart Diagnoses: A Nationwide Cross-Sectional Study

BackgroundIschemic heart disease and heart failure often lead to sexual difficulties in men, but little is known about the sexual difficulties in women and patients with other heart diagnoses or the level of information patients receive about the risk of sexual difficulties.AimTo investigate perceived sexual difficulties and associated factors in a mixed population of men and women newly diagnosed with heart disease and provide insight into sexual counseling and information given by health care professionals.MethodsThis article reports on a cross-sectional, questionnaire study sent to a randomly selected sample of men and women newly diagnosed with heart failure, ischemic heart disease, atrial fibrillation, or heart valve surgery. Eligible patients were identified by diagnosis using the Danish National Patient Register, which contains all diagnoses.OutcomesSexual difficulties were self-reported using single-item questions, and factors associated with sexual difficulties were collected from the survey and national registers.ResultsThe study population consisted of 1,549 men and 807 women (35–98 years old) with heart failure (n = 243), ischemic heart disease (n = 1,036), heart valve surgery (n = 375), and atrial fibrillation (n = 702). Sexual difficulties were reported by 55% of men and 29% of women. In a multiple regression analysis, difficulties in men were associated with being older (≥75 years old; odds ratio [OR] = 1.97, 95% CI = 1.13–3.43), having heart failure (OR = 2.07, 95% CI = 1.16–3.71), diabetes (OR = 1.80, 95% CI = 1.15–2.82), hypertension (OR = 1.43, 95% CI = 1.06–1.93), receiving β-blockers (OR = 1.37, 95% CI = 1.02–1.86), or having anxiety (OR = 2.25, 95% CI = 1.34–3.80) or depression (OR = 2.74, 95% CI = 1.38–5.43). In women, difficulties were significantly associated with anxiety (OR = 3.00, 95% CI = 1.51–5.95). A total of 48.6% of men and 58.8% of women did not feel informed about sexuality, and 18.1% of men and 10.3% of women were offered sexual counseling.Clinical ImplicationsHeart disease increases the risk of sexual difficulties and there is a need for improved information and counseling about sex and relationships for patients.Strengths and LimitationsThis large nationwide survey of men and women combined a survey with administrative data from national registries. However, this study used non-validated single-item questions to assess sexual difficulties without addressing sexual distress.ConclusionMore than half the men and one fourth the women across common heart diagnoses had sexual difficulties. No difference was found among diagnoses, except heart failure in men. Despite guidelines recommending sexual counseling, sexual difficulties were not met by sufficient information and counseling.Rundblad L, Zwisler AD, Johansen PP, et al. Perceived Sexual Difficulties and Sexual Counseling in Men and Women Across Heart Diagnoses: A Nationwide Cross-Sectional Study. J Sex Med 2017;14:785–796.     

The accuracy of antenatal fetal echocardiography screening in high- and low-risk patients

Objective: The purpose of this study was to evaluate the accuracy of four-chamber and left ventricular outflow tract views in low-risk patients and comprehensive fetal echocardiographic examinations in high-risk patients to diagnose structural heart disease or thoracic anomalies.Study design: A prospective outcome study of two cohorts was performed; 1136 low-risk patients and 886 high-risk patients were evaluated during a 2-year period. Low-risk patients had detailed fetal echocardiographic examinations. Accuracy of the ultrasonographic diagnosis was evaluated from neonatal discharge data.Results: Only 2 of 14 patients with congenital heart disease in the low-risk group were correctly identified (sensitivity 14.3%) whereas 10 of 16 patients with congenital heart disease or thoracic anomalies were correctly identified in the high-risk group (sensitivity 62.5%).Conclusion: The current study showed poor diagnostic accuracy of the standard four-chamber and left ventricular outflow tract views in low-risk patients for the diagnosis of structural cardiac anomalies. Patients with risk factors for congenital heart disease should be referred for comprehensive fetal echocardiographic examination.     

3D ultrasound and Doppler angiography for evaluation of fetal cardiovascular anomalies

ObjectiveTo highlight the value of 3D ultrasound in the prenatal assessment of fetal cardiovascular anomalies through offline diagnosis and/or second opinion (e.g. via internet link).MethodsA retrospective offline analysis of volume datasets of fetuses diagnosed with cardiovascular anomalies by 2D ultrasound was conducted.ResultsThirty-three fetuses with 38 cardiac malformations were evaluated. Mean gestational age at diagnosis was 26 weeks (range, 20–34 weeks). Isolated cardiovascular malformations were detected in 23 fetuses. Extracardiac abnormalities were identified in 8 fetuses, of which 2 had trisomy 21 and 1 had trisomy 18. One fetus exhibited 22q11 microdeletion. Ten pregnancy terminations were performed.ConclusionOffline analysis of cardiovascular anomalies conferred significant diagnostic advantages over 2D ultrasound. 3D ultrasound is invaluable for the prenatal diagnosis and management of congenital heart diseases. It may be used to facilitate scientific cooperation between high- and low-income countries.     

Value of fetal echocardiography in diagnosis of congenital heart disease in a Serbian university hospital

OBJECTIVE: Ultrasound examination for each and every pregnancy is being accepted as a standard practice worldwide, and prenatal ultrasound diagnosis of congenital heart disease (CHD) is improving due to both staff education and ultrasound equipment modernization. The objective of this study was to estimate the value and accuracy of fetal echocardiography for diagnosis of fetal CHD in a large tertiary referral center for obstetrics and gynecology. METHODS: This study was based on a prospective follow-up of 27,051 consecutive patients at the Institute of Ob/Gyn, Belgrade, during 1999-2003. Out of this population a total of 517 (1.9%) patients were selected for fetal echocardiography. The final diagnosis of congenital heart disease was confirmed or rejected on the basis of autopsy and histopathological findings, for the dead fetuses and newborns, or on the basis of neonatal echocardiography (followed by surgery/invasive diagnostic procedure), for the living neonates. RESULTS: From the total population analyzed, there was 71 gravida (0.26%) with congenital fetal heart disease. In that study group, the most frequent cardiac anomalies were complex congenital heart diseases. Analyzing the outcome, there were 67.6% cases with fetal CHD who delivered live neonates. Out of the 73 fetuses/neonates with CHD, abnormal karyotype was detected in only 9 cases. The parameters of the diagnostic value of fetal echocardiography were as follows: sensitivity 94.4%, specificity 99.8%, positive predictive value 98.5%, negative predictive value 99.1%, and overall diagnostic accuracy 99.0%. CONCLUSION: Fetal echocardiography has a very high diagnostic accuracy, commending referral of suspected patients to large tertiary referral centers.