Quality of Life in Congenital Heart Disease Patients according to Their Anatomical and Physiological Classification

Background:Living well is as important as living longer.The objective of this study is to assess quality of life(QoL)in congenital heart disease(CHD)according to current AHA/ACC anatomical and physiological classification.Methods:Cross-sectional study examining the World Health Organization QoL Bref questionnaire(WHOQoL-Bref)in consecutive outpatient CHD patients from a single unit.Results:191 CHD patients were studied.Median age was 28±13 years and 59%were male.44(23%),115(60%)and 33(17%)CHD patients showed mild,moderate and great anatomical defects respectively while 69(36%)patients were in physiological Stage A,27(14%)in Stage B,84(44%)in Stage C and 11(6%)in Stage D.No significant differences were seen in relation the anatomical classification and the different sections of the WHOQoL-Bref questionnaire.CHD patients in Stages C and D had significant lower physical domain scores than patients in the Stage A(p<0.05).However,no significant differences were seen in the psychological,social relationships and environmental domains.The binary logistic regression analysis showed that having a higher educational level was a protective factor[OR 0.32(95%CI,0.12-0.87),p=0.026]while being married or cohabit was a risk factor[OR 3.46(95%CI,1.13-10.63),p=0.030]for having a worse rated QoL.Meanwhile,having a worse functional class(NYHA≥2)[OR 3.44(95%CI,1.20-9.81),p=0.021]was associated with dissatisfaction with health.Conclusion:Patients with advanced physiological stages scored lower on the physiological domain.No statistical significance was seen,according to the anatomical and physiological classification,in the psychological,social relationship and environmental domains.

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few centers.This study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary center.Methods:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May 2022.Technical success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new regurgitation.Follow-up clinical records and echocardiography were obtained during the prenatal and postnatal periods.Results:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 years.The median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful procedures.One patient had pulmonary valve perforation without balloon dilation.No procedure-related deaths or significant complications occurred.However,one neonatal death occurred due to heart and renal failure.The median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up visit.Conclusion:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

Contemporary treatments and outcomes of preterm infants with critical congenital heart disease in a tertiary cardiovascular institute in China

Background The survival rate of preterm infants with critical congenital heart disease （P-CCHD） has been improved by medicine advances. The aims of this study were to investigate the contemporary treatments for short-term outcomes of P-CCHD and to evaluate risk factors associated with the outcomes. Methods Sixty-four P-CCHD patients admitted to Guangdong General Hospital between 2011 and 2015 were included in this study. De-mographic characteristics and patient records were reviewed. Logistic regression was used to analyze the risk fac-tors of P-CCHD outcome. Results Thirty-six patients underwent surgical treatments for cardiac anomalies. Moreover, 31.25% of the P-CCHD infants did not receive surgery because these parents refused further treat-ment. The in-hospital mortality rate was 8.3% for the patients who underwent surgeries. During a median follow-up of 1.2 years, the survivors were basically healthy. However, mental and physical growth retardation remained. Conclusions Compared to infants in developed Western countries, the treatments and short-term outcomes of P-CCHD infants were satisfactory. However, the long-term outcomes remain to be determined.

Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases

Background： Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions. Methods： Clinical and hemodynamic data from 40 pediatric patients （24 boys and 16 girls） with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis （PVS）, valved conduit stenosis, pulmonary artery stenosis （PAS）, and supravalvular pulmonary stenosis （SVPS）. Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed. Results： Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best （100%）, followed by the PVS group （90.9%） and the PAS group （85.7%）. There were a total of two transient complications （5.0%）. Conclusions： Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.

Impact of Coronary Artery Anatomy in Arterial Switch Operation:In-hospital and Post-discharge Outcomes

Objective The influence of the coronary artery anatomy on the prognosis of patients receiving an arterial switch operation(ASO)is currently controversial,and the risk factors for this operation may change in more complicated patients.This study aimed to investigate the influence of coronary artery anomalies on the in-hospital and post-discharge outcomes of ASO in patients with transposition of the great arteries(TGA)and Taussig-Bing anomaly(TBA).Methods We retrospectively reviewed 206 patients who underwent ASO from January 2007 to December 2019.The median age at operation was 33[interquartile range(IQR):20–71]days.Median follow-up time was 7.2 years(IQR:4.0–10.3 years).Results Coronary anomalies were present in 86 patients(41.7%),with 9(4.4%)of them having a single coronary artery.Additional coronary features included intramural courses in 5(2.4%)patients,ostial stenosis in 1(0.5%)patient,and accessory coronary artery orifices in 5(2.4%)patients.There were 32(15.5%)in-hospital deaths and 8(4.6%)post-discharge deaths,yielding an overall survival of 81.3%,80.7%and 79.9%at 1,5 and 10 years,respectively.Mortality due to ASO has been drastically decreased since 2013.Patients with a single coronary artery had higher rate of in-hospital mortality,but this finding was not statistically significant.The earlier surgical era(OR:2.756)and a longer cardiopulmonary bypass time(OR:2.336)were significantly associated with in-hospital mortality,while coronary patterns were not.An intramural coronary artery(HR:10.034)and a patient age of older than 1 year at the time of ASO(HR:9.706)were independent predictors of post-discharge mortality.Conclusion ASO remains the procedure of choice for TGA with coronary anomalies with acceptable in-hospital and post-discharge outcomes in terms of overall survival and freedom of reoperation.However,intramural coronary artery is an independent risk factor for post-discharge mortality.Timely surgery within the 1st year of life helps improve overall midterm survival of ASO.

Two approaches for newborns with critical congenital heart disease: a comparative study

Background Prenatal diagnosis and planned peripartum care is an unexplored concept in China.This study aimed to evaluate the effects of the“prenatal diagnosis and postnatal treatment integrated model”for newborns with critical congenital heart disease.Methods The medical records of neonates(≤28 days)admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020.The patients were divided into“prenatal diagnosis and postnatal treatment integrated group”(n=47)and“non-integrated group”(n=69).Results The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group(5.2±7.2 days vs.11.8±8.0 days,P<0.001;11.9±7.0 days vs.16.5±7.7 days,P=0.001,respectively).The weight at surgery also was lower in the integrated group than in the non-integrated group(3.3±0.4 kg vs.3.6±0.6 kg,P=0.010).Longer postoperative recovery time was needed in the integrated group,with a median mechanical ventilation time of 97 h(interquartile range 51–259 h)vs.69 h(29–168 h)(P=0.030)and with intensive care unit time of 13.0 days(8.0–21.0 days)vs.9.0 days(4.5–16.0 days)(P=0.048).No significant difference was observed in the all-cause mortality(2.1 vs.8.7%,P=0.238),but it was significantly lower in the integrated group for transposition of the great arteries(0 vs.18.8%,log rank P=0.032).Conclusions The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns,and surgical intervention could be performed with a lower risk of death,especially for transposition of the great arteries.

The application of aortopulmonary shunt in cyanotic congenital heart disease with severe pulmonary hypoplasia in adolescents and adults

Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.

Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of Pulmonary Atresia by Dual-Source Computed Tomography

Background:Pulmonary atresia(PA)is a group of heterogeneous complex congenital heart disease.Only one study modality might not get a correct diagnosis.This study aims to investigate the diagnostic power of dualsource computed tomography(DSCT)for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography(TTE).Materials and Methods:This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis.All associated malformations and clinical information,including treatments,were recorded and compared among the three groups.The diagnostic power of DSCT and TTE on all associated malformations were compared.The surgical index(McGoon ratio,pulmonary arterials index(PAI),and total neopulmonary arterial index)and radiation dose were calculated on the basis of DSCT.Results:Of the patients,32,30,and 17 were divided into the groups of PA with ventricular septal defect(VSD),PA with VSD and major aortopulmonary collateral arteries,and PA with other major malformations,respectively.Consequently,182,162,and 13 intracardiac,extracardiac,and other major malformations were diagnosed,respectively.Moreover,DSCT showed a better diagnostic performance in extracardiac deformities(154 vs.117,p<0.001),whereas TTE could diagnose intracardiac deformities better(159 vs.139,p=0.001).The McGoon ratio,PAI,and treatment methods were significantly different among the three groups(p=0.014,p=0.008,and p=0.018,respectively).Conclusion:More than one imaging modality should be used to make a correct diagnosis when clinically suspecting PA.DSCT is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.

家庭赋权方案对复杂先天性心脏病姑息术后患儿主要照顾者照顾能力的影响

目的:探讨家庭赋权方案对复杂先天性心脏病(CCHD)姑息术后患儿主要照顾者照顾能力的影响。方法:选取2022年6月1日~2024年4月1日首次接受CCHD姑息术后患儿主要照顾者60名,采取方便抽样法,将照顾者分为观察组和对照组各30名,对照组实施常规护理健康指导,观察组在常规健康指导基础上实施家庭赋权方案干预;比较两组主要照顾者照顾能力[采用照顾能力测量表(FCTI)]和照顾准备度。结果:出院时,两组FCTI评分和照顾准备度评分均高于入院时(P<0.05),且观察组高于对照组(P<0.01)。结论:对CCHD姑息术后的患儿主要照顾者实施家庭赋权方案,能有效提高照顾者的照顾准备度和照顾能力,帮助患儿家庭建立科学、有效的照顾方案,促进CCHD姑息术后患儿康复。

前门控技术在复杂先天性心脏病的应用价值

目的探讨128层CT前门控技术在复杂先天性心脏病的临床应用价值。方法收集我院进行小儿先天性心脏病CTA检查并进行手术治疗的56例患者,随机分为2组,A组为前门控扫描模式28例,B组为非门控扫描模式28例。比较两种扫描方式在复杂先天性心脏病中显示心内结构和病变的能力、图像质量、辐射剂量的差异。结果A组有效辐射剂量为0.91±0.16msv,B组为1.05±0.25msv,A组患儿所受的有效辐射剂量低于B组;A组图像质量主观评分高于B组,A组术前诊断准确率为96.0%,漏诊3例,B组诊断准确率为86.8%,漏诊12例,误诊1例,A组术前CT诊断准确率大于B组。结论128层MSCT前门控技术在小儿复杂先心病中既可以降低患儿所受的有效辐射剂量,又能清楚显示心内外结构和病变,提倡推广使用。

以三尖瓣下移畸形为主的复杂先心病合并脑梗死1例

三尖瓣下移畸形是一种罕见复杂的先心病,常累及三尖瓣及右心室,可合并其他心内畸形。当合并房间隔缺损时,可引起反常性脑栓塞。本文报道1例59岁女性患者,因脑梗死症状就诊而首次诊断为复杂先心病(三尖瓣下移畸形合并房间隔缺损、室间隔缺损并膜部瘤)。临床上引起脑梗死的原因很多,筛查病因尤为重要。本文通过回顾患者的诊疗经过和相关文献,旨在提高临床医师对三尖瓣下移畸形的认识,有助于医师制定防治策略,改善患者预后。

儿童复杂性先天性心脏病超声心动图与多层螺旋CT检查及应用的临床效果观察

目的:观察儿童复杂性先天性心脏病超声心动图与多层螺旋CT检查及应用的临床效果。方法:回顾性分析开封市中心医院2011年1月-2022年1月收治的50例患有复杂性先天性心脏病儿童的临床资料。所有患儿入院时均进行超声心动图与多层螺旋CT检查,以手术病理为金标准,分析两种检查方式对复杂性先天性心脏病的诊断效果。结果:50名患儿中,共发现178种心脏畸形,包括71种心内畸形及107种心外畸形。多层螺旋CT对心外畸形的检出率为98.13%,高于超声心动图的84.11%,差异有统计学意义(χ^(2)=11.321,P<0.05)。多层螺旋CT检查对心内畸形的检出率为95.77%,超声心动图检出率为92.96%,差异无统计学意义(χ^(2)=0.133,P>0.05)。结论:多层螺旋CT和超声心动图对复杂性先天性心脏病的阳性检出率较高,但多层螺旋CT在诊断心外畸形方面优于超声心动图。

新生儿危重先天性心脏病的超声心动图诊断及外科治疗经验

目的:总结新生儿期危重先天性心脏病(先心病)的术前超声心动图诊断及外科治疗情况。方法:回顾2012-01至2017-01来中国医学科学院阜外医院就诊的160例危重先心病新生儿资料,总结并分析这类患者的超声心动图诊断及外科治疗经验。危重先心病指:患有严重心内或大血管结构异常,容易导致难以纠正的低氧血症、酸中毒、呼吸道感染、心力衰竭等,只有通过外科或介入治疗才能生存。结果:患儿术前超声心动图诊断包括完全性大动脉转位(50%)、室间隔完整型肺动脉闭锁或重度肺动脉瓣狭窄(17.5%)、单纯主动脉离断或弓缩窄(15%)、完全型肺静脉异位回流(10.6%)、共同动脉干或右肺动脉起源于升主动脉(3.8%)、合并室间隔缺损的肺动脉闭锁或重度法乐四联症(1.9%)、巨大室间隔缺损并发严重左心衰竭(0.6%)、右心房占位(0.6%)。共140例患儿接受手术治疗,其中134例接受一期根治手术,4例接受姑息手术,2例接受分期根治手术。院内死亡率为7.9%。129例患儿接受术后随诊,平均随访时间(16.8±18.2)个月。仅2例患儿接受二次手术,其余患儿均康复良好。结论:有八类先心病需在新生儿期进行外科干预,既有紫绀型又有肺血多型。大部分危重先心病患儿经手术治疗可获得良好效果。

小儿重症及复杂先天性心脏病术后急性呼吸窘迫综合征的危险因素分析

目的通过对比小儿重症及复杂先天性心脏病（CCHD）术后发生急性呼吸窘迫综合征（ARDS）与未发生ARDS的两组患儿的临床资料,探讨重症及复杂先心病术后发生ARDS的危险因素。方法依据2011年ARDS的柏林定义,选取我院2009年1月~2014年5月心外科收治的75例未发生ARDS的重症及复杂先心病患儿作为I组,同期发生ARDS的80例患儿作为Ⅱ组,对比分析两组的临床资料,选取年龄、性别、体质量、术前氧分压及二氧化碳分压、术前及术后血白蛋白、术前及术后血肌酐、术前左室EF等39项指标进行单因素分析和多因素Logistic回归分析。结果Ⅱ组死亡17例,病死率21%。II组中轻度ARDS 27例,中度ARDS 25例,重度ARDS 28例。单因素分析发现,体质量、术前PCO2、术前左室射血分数、术后早期血清球蛋白、术中主动脉阻断时间、体外循环时间、手术时间、术中出血量、术中输血总量、术后8 h胸液量、术后8 h输血量、术后血白蛋白、术后血肌酐、术后初始乳酸值、术后24 h最大乳酸增高率、术后24 h B型尿钠肽、术后24 h降钙素原、术后24 h C反应蛋白、术后24 h血清前白蛋白、年龄、术前肺动脉压、术前肺部感染、术前凝血功能异常等23项指标在两组间差别有统计学意义（P〈0.05）,Logistic回归分析表明,术中主动脉阻断时间、体外循环时间、术后24 h最大乳酸增高率、术后24 h降钙素原及术中出血量等5项指标是CCHD术后发生ARDS的独立危险因素。结论 ARDS是重症及复杂先心病术后的一种严重并发症,大大增加患儿的病死率。根据其发生的主要危险因素：主动脉阻断时间、体外循环时间、术后24 h最大乳酸增高率、术后24 h降钙素原及术中出血量等可以预测ARDS的发生并早期采取必要的措施,以利于提高CCHD患儿术后ARDS救治的成功率。

应用脉搏血氧饱和度或/和临床评估(心脏杂音听诊)筛查新生儿危重型先天性心脏病的系统评价和Meta分析

目的评估各种先天性心脏病(CHD)筛查方案早期发现新生儿危重型CHD(CCHD)的准确性和假阳性率。方法本文文献检索和筛选是在新生儿CCHD筛查(NCCHDS)指南中实现的。以筛查阳性婴儿行心脏超声检查确诊为CCHD,或随访出现CHD症状和体征后以心脏超声或以手术或心脏导管介入术证实为CCHD为金标准。以QUADAS-2量表对纳入文献行偏倚风险及临床适用性评价,提取文献数据,汇总敏感度、特异度、假阳性率和AUC等诊断参数。结果21篇文献进入本文Meta分析,共有3种不同方案筛查新生儿CCHD:单纯脉搏血氧饱和度(POX)21篇、POX+临床评估(CE)3篇、POX+心脏杂音听诊(MUR)2篇。样本量435~167190例,研究来自美国7篇,欧洲9篇,亚洲4篇,非洲1篇;单纯POX、POX+CE和POX+MUR筛查敏感度分别为72.2%(95%CI:60.4%~81.6%)、91.0%(95%CI:86.3%~94.2%)和93.7%(95%CI:89.2%~96.4%),特异度分别为99.8%(95%CI:99.7%~99.9%)、98.5%(95%CI:96.7%~99.3%)和98.3%(95%CI:97.2%~99.0%),AUC分别为0.973、0.964和0.982。单纯POX筛查行亚组分析显示,以校正后P<0.01为标准,不同亚组敏感度差异均无统计学意义;重复测量较单次测量亚组假阳性率差异有统计学意义。POX筛查新生儿CCHD敏感度和特异度异质性与筛查者是否经过培训、不同等级医院筛查、筛查仪的核心技术不同和反复或一次性探头的种类有关。结论单纯POX、POX+CE和POX+MUR方案筛查新生儿CCHD的AUC差别不大;接受筛查中等敏感度时,可选择单纯POX筛查;接受筛查高敏感度时,可选择POX+CE和POX+MUR,更优化筛查指标可选择POX+MUR。

急诊外科治疗危重婴儿先天性心脏病的临床疗效分析

目的回顾性分析258例小于1岁的危重婴儿先天性心脏病的急诊外科治疗的临床疗效。方法从2006年1月至2009年2月期间,我院心血管外科住院患儿中,诊断明确后24h内接受外科手术治疗的258例危重先天性心脏病患儿,男性150例(58.1%),女性108(41.9%);年龄6d至11个月[(7.5±4.3)个月];体质量2.1～9.5(5.5±3.3)kg。术前合并有急性心功能衰竭、呼吸功能衰竭、肺部感染、重度贫血、营养不良、肝肾功能不全等严重并发症患儿占90.2%(233例)。全组中246例(95.3%)在体外循环下行心脏畸形矫治,12例(4.7%)行动脉导管结扎术。结果全组心血管畸形一期矫治率为92.3%,总治愈率达到96.9%。患儿的心内直视手术是在心脏停跳与心脏不停跳两种方法下进行,两者体外循环时间分别约(105.00±38.71)、(71.89±27.78)min。术后呼吸机支持时间为6～154(16.5±9.3)h;ICU住院时间为48～174(98.5±33.6)h;术后低心排综合征发生率为6.9%;心肺脑肝肾重要脏器严重并发症发生率为12.9%;死亡8例(3.1%),死亡原因:7例复杂畸形患儿中死于低心排综合征5例,心律紊乱1例,呼吸衰竭1例;另1例患儿死于心率紊乱。结论危重婴儿先心病在明确诊断后尽快进行外科手术治疗,并发有心肺肝肾等重要脏器功能不全者并不是手术绝对禁忌证;低月龄和畸形复杂是婴儿先天性心脏病手术治疗风险增加的重要因素。

实时三维超声心动图评估复杂型先天性心脏病心室收缩功能

目的观察实时三维超声心动图(RT3DE)评估复杂型先天性心脏病左、右室收缩功能的可靠性。方法复杂型先心病19例,男14例,女5例,年龄6个月～10岁,平均(2.65±2.46)岁。应用PhilipsSonos7500型超声仪(配X4矩阵探头)进行RT3DE检查,结合TomTec“4DCardioviewRT1.2”容积分析软件采样8平面法测定心室的收缩末期和舒张末期容量,计算每搏量。测值分别与磁共振和M型超声心动图描记(ME)的二、三尖瓣瓣环收缩期位移进行比较。结果RT3DE测量的左室每搏量[LVSV,(15.9±10.62)ml]和右室每搏量[RVSV,(12.42±4.69)ml]与磁共振测量的LVSV(16.56±10.64)ml和RVSV(13.96±7.96)ml均存在显著正相关,r值分别为0.98和0.75,P均小于0.05。RT3DE测定左室每搏量(12.67±10.10)ml和ME测定二尖瓣瓣环收缩期平均位移(8.17±1.92)mm之间的相关性显著,相关系数r=0.86(P<0.001);RT3DE测右室每搏量(11.83±12.02)ml和ME测定三尖瓣瓣环收缩期位移(11.04±3.06)mm两者亦存在显著正相关,相关系数r=0.88(P<0.001)。结论RT3DE可准确测定复杂型先心病左、右室的每搏量,进行可靠评估左、右室的收缩功能。

“3+1”复合超声切面培训在先天性心脏病产前筛查中的作用

目的:评价"3+1"复合超声切面(标准胎儿四腔心切面,左、右心室流出道切面+三血管切面)培训在先心病产前筛查中的作用。方法:自2006年7月始对北京地区6家妇幼保健院超声医生采取集中授课,观摩见习,指导实践等多种形式进行胎儿心脏超声"3+1"复合切面培训,并以2007年1月至2009年6月入组的17721例孕妇为研究对象,应用"3+1"复合超声切面行先天性心脏病(先心病)产前筛查,所有筛查过程均录像并保存电子图像,以生后超声或尸检结果验证产前超声结果并评价"3+1"切面培训成果。结果:17721例中,诊断胎儿先心病121例,其中复杂胎儿先心病100例,产前及生后超声心动图检查共确诊复杂先心病109例,产前漏诊10例,误诊1例,复杂先心病的产前诊断率为91%(99/109)。简单先心病病种以室间隔缺损(室缺)为主,存在假阳性和假阴性结果。小型膜周部室缺和肌部室缺漏诊较多。结论:"3+1"复合超声切面的培训和应用有效提高了复杂先心病产前诊断率,对降低复杂先心病出生率,进而降低新生儿及婴幼儿病死率意义重要。

复杂先心病DSCT影像表现与手术结果对照分析

目的：探讨双源CT对复杂先天性心脏病诊断价值。方法回顾性分析31例复杂先天性心脏病双源CT与超声心动图影像表现。结果 DSCT和ECHO对心脏畸形的诊断准确性分别为87．8％（29／33）和97％（32／33），两组比较差异无统计学意义（χ2＝0．866， P ＞0．05）。 DSCT和ECHO对心脏血管连接部及心脏周围血管畸形的诊断准确性分别为97．9％（48／49）和71％（35／49），两组比较差异具有统计学意义（χ2＝11．335， P ＜0．05）。 DSCT和ECHO对全部心血管畸形诊断准确性为93．9％（77／82）和81．7％（67／82），两者比较差异有统计学意义（χ2＝5．694， P ＜0．05）。结论DSCT对复杂先心病心脏血管连接部及心脏周围血管畸形诊断有明显优势。

杂交(hybrid)手术在复杂性先天性心脏病治疗中的初步应用

目的探讨结合介入器械和实时影像学的术中'杂交'(hybrid)手术对复杂性先天性心脏病的治疗价值.方法2005年3月～10月,我院行hybrid手术治疗7例复杂性先天性心脏病.球囊扩张组3例均为室间隔完整型肺动脉闭锁(PAIVS)的新生儿.缺损封堵组4例,其中1例右冠状动脉异常起源于肺动脉合并房间隔缺损,1例右侧肺静脉异位引流合并房间隔缺损(ASD),2例多发室间隔缺损(VSD).正中进胸,在超声引导下经右室流出道置入球囊扩张管扩张肺动脉瓣或经右心房植入封堵器.多发室间隔缺损于体外循环下经三尖瓣植入封堵器.合并的其他心脏病变同期常规外科矫正.术后心脏超声随访.结果7例均顺利出院,无一例手术死亡.3例PAIVS术后中位住院时间10 d,余4例均在术后7 d出院.随访1～6个月,7例生长发育良好,人工体肺分流管通畅,未发现中度以上的肺动脉瓣再狭窄,ASD和多发VSD术后均未发现明显残余分流及封堵器移位,均无影响瓣膜功能等并发症发生.结论hybrid手术可以不采用体外循环,减少手术创伤,对于提高复杂性先天性心脏病的疗效具有重要的意义.