Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction:A Rare Case Report

Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.

PULMONARY BLOOD DISTRIBUTION AFTER TOTAL CAVOPULMONARY CONNECTION OF DIFFERENT TYPES

Objective.To assess the feature of pulmonary blood flow distribution after total cavopulmonary connection(TCPC)of different types,and to provide the selection of the best type.Methods. Thirty-two consecutive survival patients after TCPC underwent radionuclide lung perfusion imaging. According to the radionuclide counts in the left and right lungs,analyses of the distribution of blood flow from superior venous cava(SVC) and inferior venous cava(IVC)and the whole pulmonary blood flow in both lungs were made. All patients were divided into 4 groups by the the anastomosis between IVC and pulmonary artery.Results. GroupⅠ:The flow ratio of the IVC to left lung was greater than that to the right lung,P≤0.01;the flow ratio of the SVC to right lung was greater than that to the left lung,P≤0.01;and the whole pulmonary blood flow went dominantly to the left lung,P≤0.05,which is not in line with physiological distribution. GroupⅡ:the flows from the SVC and IVC were mixed in the middle of the junction and ran evenly into the right and left lungs,the whole pulmonary blood flow went to both lungs,P≥0.05. Group Ⅲ:the flow ratio of the SVC to both lungs were the same,P≥0.05,and major part from IVC went to the right lung,P≤0.01;the pulmonary blood flow go dominantly to the right lung,P≤0.05,which is in accord with physiological distribution. Group Ⅳ:the flows from the right SVC went to right lung by 100%,P≤0.01,and that from the left SVC went to left lung by 100% too,P≤0.01;the flows from IVC went dominantly to the left lung,with little part to the right lung ,P≤0.05.Conclusions. Different types of TCPC can result in different pulmonary blood distributions. The best flow distribution between the left and right lungs can be obtained by an offset of the IVC anastomosis toward the RPA with widening anastomosis for the patients without persist left superior venous cava(PLSVC).

Total Cavopulmonary Connection:Lateral Tunnel Anastomosis or Extracardiac Conduit?——an Analysis of 114 Consecutive Patients

Objective To compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel （LT） operation with extracardiac conduit （EC） operation. Methods- From June 1996 to July 2007, 114 consecutive patients with a single ventricle underwent total cavopulmonary connection （TCPC） in Children＇s Heart Center, lAniversity Hospital Giessen and Marburg GmbH, Germany. A LT was performed in 19 （16.7%） patients, and an EC in 95 （83.3%） patients. The mean age of EC group was 50.8±31.6 （ranging from 22 to 212） months, and that of LT group was 61.5±41.2 （ranging from 30 to 168） months. Early and midterm outcomes of two groups were analyzed.Results One died in LT group （5.3%） and three in EC group （3.2%）. The overall mortality was 3.5%. There was no significant difference in mortality between EC-and-LT groups （P〉0.05）. The postoperative pulmonary arterial pressure, oxygen saturation, and effusion time of two groups had no significant difference （all P〉0.05）. No significant difference in the occurrences of complications （arrhythmias, enteropathy, and thrombosis） was found between two groups after operation （P〉0.05）. Conclusions There seems no difference between LT and EC in the clinical results in the early and middle postoperative stage. Glenn anastomosis followed by an EC seems to have some advantages.

Numerical Analyses of Idealized Total Cavopulmonary Connection Physiologies with Single and Bilateral Superior Vena Cava Assisted by an Axial Blood Pump

Our study evaluated the hemodynamic performance of an axial flow blood pump surgically implanted in idealized total cavopulmonary connection(TCPC)models.This blood pump was designed to augment pressure from the inferior vena cava(IVC)to the pulmonary circulation.Two Fontan procedures with single and bilateral superior vena cava(SVC)were compared to fit the mechanical supported TCPC physiologies.Computational fluid dynamics(CFD)analyses of two Pump-TCPC models were performed in the analyses.Pressure-flow characteristics,energy efficiency,fluid streamlines,hemolysis and thrombosis analyses were implemented.Numerical simulations indicate that the pump produces pressure generations of 1 mm to 24 mm Hg for rotational speeds ranging from 2000 RPM to 5000 RPM and flow rates of 2 LPM to 4 LPM.Two surgical models incorporated with the pump were found to be insignificant in pressure augmentation and energy boost.The risk assessment of blood trauma and thrombosis generation was evaluated representatively through blood damage index(BDI),particle resident time(PRT)and relative resistant time(RRT).The hemolysis and thrombosis analyses declare the advantage of the pump supported bilateral SVC surgical scheme in balancing flow distribution and reducing the risk of endothelial cell destruction and trauma generation.

Total Cavopulmonary Connection for Complex Cardiac Anomalies with the Functional Single Ventricle

From Aug. 1999 to Feb. 2001, 8 patients with complex congenital heart diseases, including 5 cases accompanied by hypoplastic left ventricle and 3 by hypoplastic right ventricle, were subjected to total cavopulmonary connection . Eight cases underwent the operation under cardiopulmonary bypass and 7 of whom under no cardiac clamp. Seven cases received cavopulmoanry anastomosis by flaring method and one case by end side anastomosis. All the patients underwent the intracardiac tunnels to drain inferior vena cava and plus 4 mm fenestration except one. The results showed that 6 patients had postoperative oxygen saturation more than 90 %, sinus rhythm, no anastomostic stoma obstruction, no flow reguigitation and CVP<16 cmH 2O. Two patients died postoperatively from high venous pressure of 18—20 cmH 2O, finally from cardiac failure and anoxima. It was concluded that TCPC was an effective treatment for complex congenital cardiac diseases, especially with ventricular maldevelopment. Intracardiac tunnel plus 4 mm fenestration and flaring cavopulmonary anastomosis could prevent the postoperative complications. Larger anastomotic stoma, venous pressure less than 16 cmH 2O and artery saturation more than 90 % might indicate excellent TCPC procedures in our experience.

Echocardiographic Diagnosis of Total Anomalous Pulmonary Venous Connection

To investigate the value of echocardiography in the diagnosis of total anomalous pulmonary venous connection ( TAPVC ), 16 patients in our hospital were diagnosed to have TAPVC by echocardiography from year 1994 to 2001. In 11 cases the results of echocardiography were compared to those of surgery. Each patient was examined by using a combination of precordial, suprasternal and subcostal windows to visualize all the pulmonary veins and their drainage sites, common pulmonary venous trunk, and other associated abnormalities. Of the 16 cases, the drainage sites were as follow: supracardiac in 10, via vertical vein in 9, directly to superior vena cava in 1; cardiac in 5, via coronary sinus in 2, directly to right atrium in 3. Diagnoses were correctly made in all the 11 cases as confirmed by surgery. Echocardiography can also assess pulmonary arterial pressure and detect other associated abnormalities. It is concluded that echocardiography is the preferred examination method in the diagnosis of TAPVC before surgery. With careful examination using multiple windows and sections, TAPVC can be accurately diagnosed by echocardiography.

Numerical Investigation of an Idealized Total Cavopulmonary Connection Physiology Assisted by the Axial Blood Pump With and Without Diffuser

In order to improve the surgical treatment of the congenital heart disease patient with single ventricle defect,two axial flow blood pumps,one with diffuser and the other without diffuser,were designed and virtually implanted into an idealized total cavopulmonary connection(TCPC)model to form two types of Pump-TCPC physiological structure.Computational fluid dynamics(CFD)simulationswere performed to analyze the variations of the hemodynamic characteristics,such as flow field,wall shear stress(WSS),oscillatory shear index(OSI),relative residence time(RRT),between the two Pump-TCPC models.Numerical results indicate that the Pump-TCPC with diffuser has better flow field stability,less damage on endothelial cell of vessel wall,and lower risk of vascular injury and thrombosis formation than that without diffuser.

Alagille syndrome associated with total anomalous pulmonary venous connection and severe xanthomas:A case report

BACKGROUND Alagille syndrome(ALGS)is an autosomal dominant genetic disorder caused by mutations in the JAG1 or NOTCH2 gene.It is characterized by decreased intrahepatic bile ducts associated with a variety of abnormalities in many other organ systems,such as the cardiovascular,skeletal,and urinary systems.CASE SUMMARY We report a rare case of ALGS.A 1-month-old male infant presented with sustained jaundice and had a rare congenital heart disease:Total anomalous pulmonary venous connection(TAPVC).Sustained jaundice,particularly with cardiac murmur,caught our attention.Laboratory tests revealed elevated levels of alanine aminotransferase,aspartate aminotransferase,gamma-glutamyl transpeptidase,total bilirubin,and total bile acids,indicating serious intrahepatic cholestasis.Imaging confirmed the presence of butterfly vertebra at the seventh thoracic vertebra.This suggested ALGS,which was confirmed by genetic testing with a c.3197dupC mutation in the JAG1 gene.Ursodiol was administered immediately after confirmation of the diagnosis,and cardiac surgery was performed when the patient was 1.5 month old.He recovered well after treatment and was discharged at the age of 3 mo.At the age of two years,the patient returned to our clinic because multiple cutaneous nodules with xanthomas appeared,and their size and number increased over time.CONCLUSION We report a unique case of ALGS associated with TAPVC and severe xanthomas.This study has enriched the clinical manifestations of ALGS and emphasized the association between JAG1 gene and TAPVC.

Coronary Sinus Atrial Septal Defect (Unroofed Coronary Sinus) with Total Anomalous Pulmonary Venous Connection—A Case Report

Introduction: To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC). Case Report: A 16-year-old girl was diagnosed with features of an atrial septal defect (ASD) by transthoracic echocardiography and the absence of PLSVC (persistent left superior vena cava) connection to coronary sinus was confirmed by saline contrast echocardiography (“bubble study”). Discussion: The cause of the dilated coronary sinus was due to total anomalous pulmonary venous connection. Conclusion: Coronary sinus atrial septal defect (ASD) is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava (PLSVC) and occurs in less than 1% of cases of ASDs. It is characterized by the absence of at least a portion of the common wall that separates the coronary sinus and left atrium. Isolated defects are associated with low rate of morbidity and mortality.

Paired, Total, and Connected Domination on the Queen’s Graph Revisited

The question associated with total domination on the queen’s graph has a long and rich history, first having been posed by Ahrens in 1910 [1]. The question is this: What is the minimum number of queens needed so that every square of an n × n board is attacked? Beginning in 2005 with Amirabadi, Burchett, and Hedetniemi [2] [3], work on this problem, and two other related problems, has seen progress. Bounds have been given for the values of all three domination parameters on the queen’s graph. In this paper, formations of queens are given that provide new bounds for the values of total, paired, and connected domination on the queen’s graph, denoted , , and respectively. For any n × n board size, the new bound of is arrived at, along with the separate bounds of , for with , and , for with .

The Edge Connectivities of Total P_(k)-graphs

The P_(k)-path graph P_(k)(G)corresponding to a graph G has for vertices the set of all paths of length k in G.Two vertices are joined by an edge if and only if the intersection of the corresponding paths forms a path of length k-1 in G,and their union forms either a cycle or a path of length k+1.Let Ek={(v,p),p E V(P_(k)(G)),v is an end vertex of p in G},we define total P_(k)-graphs T_(k)(G)as Yk(G)=(V(G)UV(P_(k)(G)),E(G)U E(PI(G))U Ek).In this note,we introduce total P,-graphs Th(G)and study their edge connectivity,as the generaliza-tion of total graphs.

产时手术矫治危重先天性心脏病1例

危重先天性心脏病(先心病)患儿生后即面临生命危险,一旦病情恶化,预后极差。针对这种术后危重状态可能导致的严重不良后果,在1例入住首都医科大学附属北京安贞医院的胎儿心脏结构异常孕妇的诊疗中我们首次选择新生儿娩出后即启动手术的多学科救治模式,来规避术前的高风险。母孕期胎儿诊断为完全型肺静脉异位引流,于本院经剖宫产娩出胎儿后,立即转入心脏手术室,明确诊断,完成必要术前检查后开始麻醉,完成手术矫治,手术过程、术后恢复顺利。该救治模式通过新生儿娩出后即刻的早期手术有望改善危重先心病患儿的预后,降低死亡率,提高远期健康水平。

全腔肺吻合术后患儿运动耐量状况及相关危险因素分析

目的:探查全腔肺吻合术(TCPC)后患儿运动耐量状况及引起运动耐量下降的危险因素。方法:对2021年3月至2023年6月在我院接受心肺运动试验(CPET)的81例TCPC术后患儿进行回顾性研究。分析相关CPET参数,并根据最大摄氧量(VO_(2)max)占预计值百分比情况将患儿分为运动耐量较差组(VO_(2)max占预计值百分比<60%,n=61)和运动耐量较好组(VO_(2)max占预计值百分比≥60%,n=20),分析影响心肺运动功能的危险因素。结果:81例患儿平均年龄(12.28±3.28)岁,VO_(2) max占预计值百分比为(55.43±14.80)%。运动耐量较好组的最大单位体重摄氧量[(30.26±3.16)ml/kg vs.(20.55±4.26)ml/kg]、变时性指数[(0.63±0.15) vs.(0.46±0.18)]、随功率增加的摄氧量[(11.89±2.43)ml/(min·W) vs.(9.88±2.25)ml/(min·W)]以及单位体重氧摄取效率斜率[(36.49±6.16)ml/(log L·kg)vs.(26.83±6.38)ml/(log L·kg)]较运动耐量较差组均更好(P均<0.01)。两组的最大呼吸末二氧化碳、二氧化碳通气当量斜率、运动振荡通气发生率等差异均无统计学意义(P均> 0.05)。多因素Logistic回归分析表明,年龄、体重指数和变时性指数是运动耐量较差的危险因素;最大单位体重摄氧量和无氧阈时随功率增加的摄氧量是发生运动振荡通气的危险因素。结论:TCPC术后患儿运动耐量普遍下降,表现为有氧运动能力受损,其危险因素为年龄、体重指数和变时性指数。最大单位体重摄氧量和无氧阈时随功率增加的摄氧量为术后运动振荡通气危险因素。

水资源“量-质-域-流-生”全要素保护体系架构与实施策略

水资源保护是关乎民生福祉和国家安全的重要基础性工作。在新时期生态文明和美丽中国建设背景下,水资源保护亟需向流域化、系统化、生态化方向发展。研究基于对水资源系统多维属性功能的解析,从流域整体视角剖析了人类活动对水资源系统的干扰特征,提出了水资源“量-质-域-流-生”全要素保护策略,明确了水资源全要素保护的概念、内涵及体系架构。进一步,从服务于我国水资源管理实践角度,提出了以“退水还河、退污还清、退地还盆、退堵还疏、退渔还生”为核心的水资源全要素保护策略,明确了东北地区、黄淮海地区、长江中下游地区、东南沿海地区、西南地区、西北地区的水资源保护核心要点。最后,从政策法规、制度建设、监管体系等提出了推进我国水资源全要素保护的对策与措施。研究成果是对传统水资源保护工作的有益发展,为新时期国家治水实践提供了有力抓手。

全偏联系数中不确定示性系数i取值的探究

一阶全偏联系数是揭示“同异反”三元联系数所描述的系统整体状态在内部动态演化的一个伴随函数.用量子力学理念揭示系统从“正常”到“失常”两个极端状态改进算法,获得新的表达式,用实例展示其成效.

电动汽车与电网互联技术在新能源并网中的应用

V2G (Vehicle-to-Grid,电动汽车与电网互联)技术的应用可以实现电网与车主的双赢。从电网角度,可以发挥削峰填谷、平抑新能源功率波动、延缓电网升级改造等作用;从用户角度,在满足用车的要求下可获取附加效益。随着电动汽车的推广和新能源并网规模的发展,V2G技术得到更多的关注。然而,V2G技术的发展仍然面临规模化互动、运营、调度等技术及经济性问题。因此,从V2G技术发展的现状出发,重点阐述V2G技术在新能源并网中的作用和应用模式;并对V2G技术在新能源并网中的充放电策略、电池寿命预测以及综合价值进行了探讨。V2G技术在新能源并网中的推广应用不仅可以提升新能源并网利用率,还可进一步提升电动汽车的减排效果。

基于多层感知机的楔环总接触力检测方法研究

楔环联接结构常用于薄壁圆筒产品联接,楔环之间的接触力可以用于表征楔环的装配紧凑程度,通过分析楔环夹紧力与脉冲力及其加速度响应的关系,建立了基于多层感知机的楔环总接触力预测模型,并根据该模型提出了总接触力检测方法。设计实验装置并对模型与测量方法进行了试验验证,试验结果表明,该方法的检测准确性优于±75 N,验证了检测方法的可行性。

超声下左心房后空间距离对胎儿完全性肺静脉异位引流的诊断价值

目的探讨超声检测左房后空间距离对胎儿完全性肺静脉异位引流(TAPVC)的诊断价值。方法选取2020年3月至2021年8月于医院行孕中期胎儿结构筛查的362名孕产妇为研究对象,均以彩色多普勒超声诊断仪于标准四腔心切面的收缩末期进行左房后空间距离测定。以产后胎儿超声心动图检查或引产后胎儿尸检结果为金标准,统计心脏超声检查结果、胎儿左心房后空间距离、胎儿TAPVC心脏超声检查特征,分析左心房后空间距离与孕周及新生儿出生Apgar评分的相关性。结果362名孕产妇中,7例通过超声诊断为心上型胎儿TAPVC,4例诊断为心内型胎儿TAPVC;TAPVC胎儿的左心房后空间距离大于正常胎儿(P<0.05);左心房后空间距离与孕周、新生儿出生Apgar评分呈负相关(r=-0.658、-0.747,P<0.05)。结论超声下测定左心房后空间距离可用于诊断胎儿TAPVC,且其结果与孕周及新生儿出生Apgar评分均呈负相关。

CT肺容积参数评估CTD-ILD肺功能、病情严重程度的价值分析

目的 分析CT肺容积参数评估结缔组织病相关间质性肺疾病(CTD-ILD)患者肺功能、病情严重程度的价值。方法 选取2020年4月—2022年12月收治的126例CTD-ILD,根据肺间质病变临床严重程度分级分为Ⅰ级组(43例)、Ⅱ级组(45例)、Ⅲ级组(38例),均接受肺功能检查,采用高分辨率CT检查获取CT肺容积参数[正常肺组织容积(NL)、间质纤维化肺容积(ILDV)、全肺总容积(WL)、正常肺组织容积百分比(NL%)、间质纤维化肺容积百分比(ILDV%)],比较各组肺功能指标[肺总量(TLC)、第1秒用力呼气容积(FEV1)、用力肺活量(FVC)、残气量(RV)、第1秒用力呼气容积占预计值百分比(FEV1%)]、GAP等级评分、图像纤维化评分及CT肺容积参数,分析CT肺容积参数与肺功能指标、GAP等级评分、图像纤维化评分的关系,以CT肺容积参数对CTD-ILDⅢ级进行诊断,并分析其与肺功能指标联合检测对CTD-ILDⅢ级的诊断效能。结果 FVC、FEV1、TLC、RV、FEV1%Ⅰ级组>Ⅱ级组>Ⅲ级组,GAP等级评分、国际纤维化评分Ⅰ级组<Ⅱ级组<Ⅲ级组(P<0.05);NL、WL、NL%Ⅰ级组>Ⅱ级组>Ⅲ级组,ILDV、ILDV%Ⅰ级组<Ⅱ级组<Ⅲ级组(P<0.05);NL、WL、NL%与FVC、FEV1、TLC、RV、FEV1%呈正相关,与GAP等级评分、图像纤维化评分呈负相关(P<0.05);ILDV、ILDV%与GAP等级评分、图像纤维化评分呈正相关,与FVC、FEV1、TLC、RV、FEV1%呈负相关(P<0.05);NL、WL、ILDV、NL%、ILDV%诊断CTD-ILDⅢ级的受试者工作特征曲线下面积(AUC)分别为0.765、0.715、0.754、0.789、0.768,NL、WL、ILDV、NL%、ILDV%联合诊断的AUC最大,为0.944。结论 CTD-ILD患者NL、WL、ILDV、NL%、ILDV%与肺功能联系密切,且联合检测对患者病情严重程度有较高的诊断效能。

不同坡向、坡度工业厂房屋面分布式光伏发电模拟分析

采用专业软件对西安某工业厂房屋面分布式光伏系统进行建模,分析在南北坡向、东西坡向厂房屋面坡度为5%~10%条件下光伏组件采光面的年辐照总量,并且对年并网电量、光伏系统效率(PR系数)进行分析,得出此地区的主要发电模拟数据的变化规律,用于指导工程设计。