Primary reconstruction of the common truncus arteriosus in infants, using modified pulmonary homograft]

Truncus arteriosus communis (TAC) is a complex heart disease, it presents 1% of the congenital heart defects. A 7 weeks old infant with cardiac failure was operated on with TAC. The bicuspidized (14 mm diameter) pulmonary homograft valved conduit was used to reconstruct the right ventricular outflow tract (RVOT). The sternum closure was delayed. The important points of the indications for the surgical management of TAC: 1. Management or prevention of the pulmonary hypertensive crisis. 2. Patient's age. 3. Reconstruction of the RVOT (choices: homograft, dacron valve conduit, autologous pericardial valve conduit, porcine aortic root, direct anastomosis of the pulmonary trunc to the right ventricle). The surgical management is based on the optimal combination of these three important points. This is the first successfully performed primary surgical repair of an infant with TAC using modified pulmonary homograft in Hungary.     

Ischemic hepatitis in an 81 year old patient with congenitally corrected transposition of the great arteries

The congenitally corrected transposition of the great arteries is a rare form of congenital heart disease, with survival beyond the 6th decade of life being rare. Even more unusual is its presentation alone, without any other form of congenital heart disease. Ischemic hepatitis is a rare entity characterized by an elevation of transaminasas and a centrilobular necrosis due to a reduction in hepatic blood flow, generally of reversible evolution. The authors present a case of ischemic hepatitis in an 81-year-old patient with congenitally corrected transposition of the great arteries. The fatal evolution of the episode and the longevity of the patient are both notable. We comment on the clinical and diagnostic aspects of both entities.     

液氮深低温保存同种带瓣血管的临床应用

目的报告应用液氮深低温保存的同种带瓣血管（valved homograft conduit,VHC）纠治复杂心脏畸形的经验。方法患者男27例,女19例,年龄1.9～31（9.5±5.1）岁,体质量10～64（29.5±13.2）kg。病种包括右室双出口15例,矫正性大动脉转位9例,共同动脉干7例,肺动脉闭锁5例,法乐氏四联症4例,完全性大动脉转位3例,先天性主动脉瓣关闭不全2例,法乐氏四联症术后肺动脉瓣重度关闭不全1例。合并畸形包括肺动脉狭窄、房间隔缺损、动脉导管未闭、多发室间隔缺损等。手术均在全麻低温体外循环下进行。结果无手术死亡。早期死亡2例,分别为术后低心排综合症和呼吸道出血。存活44例,随访（32/44）3～96（30±19.5）月,死亡2例,1例右室双出口患者术后26个月死于感染性心内膜炎,另1例为矫正性血管转位患者术后8个月死亡,死因不明。其余30例患者心功能明显改善,超声心动图检查显示吻合口无狭窄,VHC管腔通畅,瓣膜无明显返流。结论液氮深低温保存的VHC是理想的人工材料,用于纠治复杂心脏畸形可获得较好的治疗效果。     

先天性心脏病患儿围手术期能量代谢规律研究进展

先天性心脏病住院患儿营养风险比例较高,导致围手术期营养风险提高的因素包括营养不良、肺动脉高压及血流动力学不稳定等.规范化围手术期营养支持可以有效降低患儿营养风险,并已成为先天性心脏病临床治疗的重要方面,而明确围手术期能量代谢规律是其首要问题.本文通过分析先天性心脏病患儿围手术期能量代谢规律的研究成果,为先天性心脏病围手术期临床营养支持工作提供依据.     

Heart transplantation after corrective surgery of a congenital heart disease. Surgical technical problems

Heart transplantation was performed in a 17-year old boy with severe left ventricular failure. Previously a congenital heart disease, tetralogy of Fallot was reconstructed in one and half years old of age. Authors present the different surgical problems complicated this procedure. In Hungary this is the first heart transplantation after a corrective surgical procedure of a complex congenital heart disease.     

产前B超检查对先天性心脏病筛查的应用分析

目的探讨产前B超检查对先天性心脏病筛查的应用效果。方法本文纳入我院2017年1月—2018年6月进行先天性心脏病筛查的500例孕妇,所有孕妇均接受产前B超检查,对孕妇宫内胎儿的胎心结构、形态、方式等进行观察,统计胎儿胎心异常的孕妇,检查确诊后终止妊娠。对胎心检查正常的孕妇进行妊娠结局随访,分娩新生儿接受心脏彩超检查,判断有无先天性心脏病发生,评定结果以新生儿出生后彩超检查为金标准,计算出产前B超检查对先天性心脏病筛查的准确性。结果500例孕妇经产前B超检查,有6例检出胎心异常,比例为1.2%。以心脏彩超检查金标准为依据,有8例新生儿被确认先天性心脏病。产前B超检查对先天性心脏筛查病诊断的准确性为98.8%,敏感度为91.2%,特异性为99.9%,差异无统计学意义(P>0.05)。结论产前B超检查对先天性心脏病筛查的应用效果显著,可有效检出先天性心脏病胎儿,准确性较高,临床应用价值较高,可大大减少缺陷胎儿的出生,值得在超声科中推广应用。     

Effect of fathers'' age and birth order on occurrence of congenital heart disease.

STUDY OBJECTIVE--The aim was to examine if there is an effect of fathers' age and of birth order on the occurrence of congenital heart disease. DESIGN--This was a hospital based case-referent study including use of birth defects surveillance data. SUBJECTS--Subjects were 497 cases of congenital heart disease aged between 3 months and 5 years, born in Beijing and Hebei Province, China; 6222 children without congenital heart disease serve as reference baseline. MEASUREMENTS AND MAIN RESULTS--With stratified analysis and logistic regression analyses, congenital heart disease was found to be associated with fathers' age less than 25 years (odds ratio 2.63), independent of mothers' age and of birth order. There was also evidence to show a higher birth order effect on the occurrence of congenital heart disease independent of parental ages. CONCLUSION--Higher birth order and fathers aged less than 25 years were both independently associated with some categories of congenital heart disease and with congenital heart disease overall.     

紫绀型与非紫绀型先天性心脏病患儿围手术期输血量比较

目的探讨紫绀型与非紫绀型先天性心脏病患儿的围手术期输血量,以供临床参考。方法选择2009年1月-2011年8月在深圳市孙逸仙心血管医院先天性心脏病体外循环下进行心脏手术的患儿449例,根据其疾病类型分为紫绀型先天性心脏病和非紫绀型先天性心脏病,比较两种心脏病类型患儿围手术期的输血量,并将结果进行统计学分析。结果紫绀型患儿围手术期输注的红细胞和血浆均明显多于非紫绀型患儿,组间差异有统计学意义（P〈0．05）。结论紫绀型与非紫绀型先天性心脏病患儿围手术期输血量不同,医生要根据患儿的实际情况给予判断。     

孕早期不同类型心理社会应激对先天性心脏病的影响

目的:探讨孕早期不同类型心理社会应激与先天性心脏病的关系,为先天性心脏病的预防提供依据。方法:使用自编调查问卷,对86例先天性心脏病患儿和按照1∶2原则匹配性别、年龄、出生地选择的172例正常对照儿童的母亲分别进行面对面问卷调查。使用SPSS 10.0统计学软件进行统计分析。结果:孕早期总负性心理社会应激性生活事件数量在文化程度低的孕妇中高于文化程度较高的孕妇,差异具有统计学意义(P<0.05)。先天性心脏病主要为房间隔缺损(41.9%)和室间隔缺损(33.7%)。孕早期急性负性心理社会应激性生活事件数量先天性心脏病组高于对照组,差异具有统计学意义(P<0.05)。结论:孕早期急性负性心理社会应激可能对子代先天性心脏病的发病影响更大,文化程度较低的孕妇群体更加应该引起关注。     

Incidence and invasive treatment of congenital heart diseases in Hajdu-Bihar county

The incidence of congenital heart diseases was evaluated in the period 1994-1998. The number of those who went through therapeutic intervention and those infants dying of congenital heart disease were also assessed. Data were collected retrospectively. During the study period 26,932 live-births occurred in Hajdú-Bihar county and 421 congenital heart disease were diagnosed, 81% of whom were diagnosed under the age of one year. The most frequent diseases were secundum type atrial septal defect, ventricular septal defect and patent ductus arteriosus. 121 therapeutic procedures were performed (109 operations and 12 interventional heart catheterizations). 41 interventions occurred under the age of one and 13 before the age of 28 days. The mean age of children older than 1 year was 6 years at the time of the operation or interventional catheterization. The overall postoperative mortality within 30 days was 8.3%. During the study period 28 infants with significant heart disease died, 20 of whom also had an associated disease (most frequently prematurity). For congenital heart disease 4.5 operations or interventional heart catheterizations were required/1000 live births. The higher rate of diagnosed congenital heart disease is due to the development in diagnostic techniques, especially to Doppler-echocardiography. Owing to the operational waiting list the children's age at the time of operation is higher than optimal. The mortality among infants with heart disease is influenced by many factors, that is why stepping forward is a complex task in this field.     

产前超声筛查胎儿先天性心脏病的诊断价值及高危因素分析

目的 探讨产前超声筛查胎儿先天性心脏病的临床价值并分析其高危因素。方法 应用回顾性分析2019年1月—2020年7月在本院接受产前胎儿先天性心脏病超声筛查的2 869例孕妇的临床资料,计算超声对胎儿先天性心脏病的诊断灵敏度、特异度及准确度。将发生胎儿先天性心脏病者作为先天性心脏病组,未发生者作为正常组。采用Logistic回归分析胎儿发生先天性心脏病的高危因素。结果 2 869例孕妇中126例发生胎儿先天性心脏病,检出率为4.39%,超声检查结果灵敏度为87.30%(110/126)、特异度为88.52%(2 428/2 743)、准确度为88.46%(2 538/2 869)。与正常组相比,先天性心脏病组胎儿早期颈项透明层(nuchal translucency,NT)值高或患有颈部淋巴水囊瘤(61.90%)、母亲患有结缔组织病或糖尿病(64.29%)、先天性心脏病家族史(77.78%)、羊水异常(62.70%)占比均高于正常组的35.29%、46.85%、60.30%、44.29%(χ2=36.850、14.688、15.480、16.479,P值均＜0.001)。经Logistic回归分析,胎儿早期NT值高或患有颈部淋巴水囊瘤、母亲患有结缔组织病或糖尿病、先天性心脏病家族史与羊水异常均为导致胎儿先天性心脏病的高危因素(P＜0.001)。结论 产前超声筛查对于胎儿先天性心脏病的诊断价值高,胎儿早期NT值高或患有颈部淋巴水囊瘤、母亲患有结缔组织病或糖尿病、先天性心脏病家族史与羊水异常均为导致胎儿先天性心脏病的高危因素。     

Aortic root replacement with pulmonary allograft (Ross procedure) in children. Early results

INTRODUCTION: For infants and children with congenital aortic valve disease root replacement with pulmonary allograft (Ross procedure) is the preferred method of choice. PATIENTS/RESULTS: The authors have successfully applied this operation in 12 children (age range from 2.5 to 17 years--mean 9 years, body weight from 12 to 58 kg--mean 46 kg), one of whom has also required a Konno extension for long segment left ventricular outflow tract obstruction. The operation was complicated by early postoperative endocarditis in one case, and the child required redo homograft root replacement on the ninth postoperative day. All patients, including this one survived, and are doing well at present. CONCLUSIONS: In the Hungarian literature this is the first report on the Ross and Konno procedure in children. On the basis of our excellent early results, Ross procedure is the method of choice in aortic valve disease in children.     

先天性心脏病患儿脑利肽水平的临床研究

目的探讨先天性心脏病患儿血浆脑利钠肽（BNP）水平与心功能的关系及其临床意义。方法回顾性分析70例先心病患儿的临床资料,根据先天性心脏病的类型分为两组：A组为左向右分流型先心病,B组为发绀型先心病。常见左向右分流型先心病又按心功能分级及肺动脉高压情况分为2类。用化学发光免疫分析法测定血浆BNP水平。同时选取30例正常儿童作为对照组。结果⑴除法洛四联症外,其他类型的先心病患儿血浆BNP水平均高于正常对照组;⑵常见左向右分流型先心病患儿心功能Ⅲ～Ⅳ级合并肺动脉高压者与心功能Ⅰ～Ⅱ级无肺动脉高压者,血浆BNP水平存在明显差异（P〈0.01）。结论血浆BNP水平不仅与心功能存在明显正相关,而且对判断肺循环血流量具有重要意义。     

National registry and DNA-bank of patients with congenital heart disease: the CONCOR-project

Although survival of patients with congenital heart disease has dramatically improved since surgical repair has become available, cure is seldom achieved. Exact data on long-term outcome are not available, however, because a national registry is lacking. Furthermore, little is known about the role of genetic defects in the development of congenital heart disease. The CONCOR-project (CONgenital CORvitia) has been set up to facilitate the investigation of the long-term outcome and molecular basis of specific congenital heart defects and their treatment. It will also facilitate the development of an efficient organisational structure for the improvement of healthcare for patients with congenital heart disease.     

儿童先天性心脏病诊断中彩色多普勒超声的应用及其临床价值

目的探讨儿童先天性心脏病(CHD)诊断中彩色多普勒超声的应用及其临床价值。方法选取2015年1月-2018年12月在丽水市妇幼保健院行CHD筛查的儿童13805例。所有患儿均经彩色多普勒超声检查,将所有患儿最终临床诊断和(或)手术结果作为对比金标准。观察所有患儿的最终诊断结果;观察与最终临床诊断结果相比彩色多普勒超声的诊断符合率;观察彩色多普勒超声诊断CHD患儿的临床效果。结果其中,有3138例(22.7%)被最终确诊为CHD。3138例CHD患儿当中,占比最高的畸形为:房间隔缺损2517例(80.2%)和室间隔缺损485例(15.5%)。3138例CHD患儿中,彩色多普勒超声共检查出3046例,总符合率为97.1%。彩色多普勒超声诊断CHD患儿的准确率为99.3%,特异度为100.0%,灵敏度为97.1%。结论给予CHD患儿进行临床诊断的过程当中,使用彩色多普勒超声可以将患儿体内的房间隔、室间隔的生理结构进行更为清晰的显示,为临床诊断提供更多的参考信息,从而提高临床对CHD的诊断效率。     

维生素A缺乏或过量与先天性心脏病的关联

近年研究表明,维生素A在胚胎时期参与心脏系统的形态构建,维生素A缺乏或过量均可造成不同程度或不同类型的先天性心脏病。其中胚胎原肠胚期是维生素A作用于心脏发育的特异性时段,它通过激活一系列基因调控途径,进而影响到随后的心脏发育事件。本文对妊娠期维生素A缺乏和过量与先天性心脏病的关系及其作用机制进行简要综述。     

CONCOR,an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: Rationale,design, and first results

Introduction:Survival of patients with congenital heart disease has dramatically improved after surgical repair became available 40 years ago. Instead of a mortality of 85% during childhood following the natural course, over 85% of these infants are now expected to reach adulthood. However, data on long-term outcome is scarce due to the lack of large, national registries. Moreover, little is known about the genetic basis of congenital heart defects. In 2000, the Interuniversity Cardiology Institute of the Netherlands and the Netherlands Heart Foundation have taken the initiative to develop a national registry and DNA-bank of patients with congenital heart disease in the Netherlands named CONCOR.Objectives: The aims of the CONCOR-project are to facilitate investigation of the prevalence and long-term outcome of specific congenital heart defects and their treatment, to develop an efficient organisational structure for the improvement of healthcare for patients with congenital heart disease, and to allow investigation of the molecular basis of congenital heart defects. Methods: After informed consent, research nurses enter data of participating patients into the CONCOR database using a web application. Data is transferred over the Internet via a secure connection. About 20 ml blood is withdrawn from the patient, and the DNA is isolated and stored. From each participating patient family history on congenital heart disease is obtained. Results: Within two and a half years more than 4200 patients have agreed to participate. More than 99% of the patients that were asked have given their consent to participate in CONCOR. From 60% of these patients DNA has already been obtained. Mean age of the patients included is 34 years; more than 85% of the patients are younger than 45 years. Late complications occur frequently and the incidence increases with advancing age. 18% of the patients are known with supraventricular or ventricular arrhythmias. 2% of the included patients suffered a cerebrovascular accident, 139 (3%) had endocarditis. 6% of the patients has pulmonary hypertension or Eisenmenger syndrome. More than 15% of the patients reported an affected family member with congenital heart disease in the first, second, or third degree. 6% has an affected first-degree relative, and 4% a second-degree relative. Already 10 research projects have started using the CONCOR data and DNA. Conclusion: The population of patients with congenital heart disease is young and rapidly growing. Late complications occur frequently and the incidence increases with advances age. The CONCOR registry and DNA-bank facilitates research on prevalence and long-term outcome and allows investigation of the molecular basis of congenital heart disease. An erratum to this article is available at .     

先天性心脏病患儿手术前后血BNP的变化及临床意义

目的：分析比较先天性心脏病患儿手术前后血BNP的变化及临床意义。方法：对本院从2013年1月-2014年8月收治的60例先天性心脏病患儿进行研究,30例简单先天性心脏病患儿作为对照组,30例危重症先天性心脏病患儿作为试验组,对比分析两组患儿在手术前后血BNP变化,分析BNP在危重症先心病患儿术后心功能评定中的意义。结果：试验组先天性心脏病患儿手术前血BNP水平为（19.0±9.9）ng/L,术后5 min为（23±14）ng/L,术后24 h为（32±11）ng/L,较对照组差异有统计学意义（P〈0.05）。结论：在临床中通过研究先天性心脏病患儿手术前后血BNP变化,可以得出血清BNP水平与相应心功能水平之间的内在联系,找到一种准确、快速了解心功能水平的方法,有助于保障患者的健康,值得在实际中推广。     

宁波市海曙区婴幼儿先天性心脏病监测与随访

【目的】 监测婴幼儿先天性心脏病(先心病)的发病情况和预后,为幼儿先心病的保健管理提供资料。 【方法】 2008-2010年在宁波市海曙区出生的足月活产儿8 259例中,经心脏彩超确诊为先天性心脏病的婴幼儿221例进行定期随访跟踪。 【结果】 婴幼儿先心发病率居前4位的分别为室间隔缺损(占24.89%),卵圆孔未闭(占22.62%),房间隔缺损合并动脉导管未闭(占13.57%),动脉导管未闭合并卵圆孔未闭(占13.57%);221例先心病患儿均在1岁前确诊。 【结论】 先天性心脏病是我国重要的公共卫生问题之一,开展对先天性心脏病的监测和随访,提高先天性心脏病婴幼儿的生活质量。     

孕中期复杂性先心病的产前超声筛查

目的探讨孕中期超声对复杂性先天性心脏病的筛查作用,提高复杂性先心病的检出率。方法 2010年10月至2013年10月期间,我院7 295例孕妇于孕21⁴0周,分别进行对照组(B超检查)和观察组(通过美国GE Voluson 730彩色多普勒超声诊断仪,通过心脏超声筛查的5个标准切面,发现异常心脏节段,再通过彩色多普勒血流信号,观察房室、大动脉血流情况,分析先天性心脏病类型)检查。产前超声诊断结果与本院尸检结果,进行对比分析。结果与对照组相比,观察组复杂性先天性心脏病的临床诊断率明显升高(0.27%vs.0.12%),差异有统计学意义(P<0.05)。7 295例胎儿检出20例复杂性先天性心脏病,其中6例合并心外畸形,14例本院引产,其尸检结果与产前超声诊断结果相一致。结论复杂性先天性心脏病的畸形情况多变,超声分段检查结合彩色多普勒超声的产前超声筛查能够有效提高复杂性先心病的检出率。