Arrhythmias in Common Arterial Trunk(CAT):Uncommon Atrial Tachycardia in CAT with Anomalous Pulmonary Venous Connection and Re-entry Atrial Tachycardia in CAT with HIV Seropositive Mother

We show a brief report of two common arterial trunk cases(CAT)with different arrhythmias and discuss anatomy,clinical and diagnostic management.The burden of volume and pressure overload of this cardiac malformation may predispose to different types of arrhythmia before and after surgical repair.Because of labile hemodynamic state in this group of patients,prompt diagnosis of any arrhythmia is mandatory as the devastating factor on prognosis.The first patient with a diagnosis of CAT Type II Collett and Edwards(CE)had a particular history with HIV seropositive mother assuming antiretroviral therapy during pregnancy,who presented hyperbilirubinemia and liver dysfunction at birth,and re-entry atrial tachycardia after repair.The second patient had CAT Type I CE with a partial anomalous venous connection of left superior pulmonary vein and uncommon type of atrial tachycardia with dual AV nodal physiology.

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.

Partial Anomalous Pulmonary Venous Connection and the Nature of Associated Sinus Venosus Defect

Background:Partial anomalous pulmonary venous connection(PAPVC)is frequently associated with atrial septal defect(ASD),especially sinus venosus defect(SVD).Although Waggstaffe described the pathology of SVDs in 1868,the exact anatomic features and the nature of SVD remains controversial.SVDs with no posterior atrial rim were observed in recent years.However,no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD.The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD.Methods:From January 2011 to December 2019,256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled.Comprehensive review of preoperative transthoracic echocardiography,computed-tomography images and surgical findings were performed by experienced pediatric cardiologists.The subtypes of PAPVC,locations and types of ASD,and presence of posterior atrial rim of associated ASD were investigated.Results:PAPVC was right-sided in 244 children,left-sided in 6 children,and bilateral in 6 children.In PAPVC cases,ASD without posterior atrial rim existed in 226 SVD cases.ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium(RA)or to RA-superior vena cava junction.In cases with isolated ASD,there were 3 SVD,and the other 875 cases were secundum ASD.Conclusions:ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava(SVC)junction.For SVD,the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim,rather than adjacent to the SVC or to the inferior vena cava.

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.

Obstructed infracardiac total anomalous pulmonary venous connection:The challenge of palliative stenting for the stenotic vertical vein

Introduction Obstructed total anomalous pulmonary venous connection(TAPVC)is one of the commonest seen emergencies in pediatric cardiology centers.Case presentation Our case was diagnosed to have this anomaly,showing early respiratory distress resulting from severe pulmonary congestion.Palliative stenting of the obstruction was done,which helped in stabilizing the debilitated hemodynamics of the baby before surgery,thus a good surgical outcome and prognosis are expected.Conclusion This intervention may be listed as a vital measurement in the preoperative cardiac stabilization plan for infants with obstructed TAPVC.

Surgical outcomes of functional single ventricle with total anomalous pulmonary venous connection

Background The surgical outcomes have been greatly improved over the past four decades for functional single ventricle(FSV). However,the repair for coexistence of FSV and total anomalous pulmonary venous connection(TAPVC),especially obstructed TAPVC,has remained a clinical challenge. Little is known about the results of surgical treatment for patients with FSV-TAPVC in China. Methods Forty consecutive patients with FSV-TAPVC undergoing initial surgical palliation(median age:16.8 months;body weight:8.25 kg)were retrospectively enrolled from 2006 to 2016 in a specialized cardiovascular institute. TAPVC was repaired in 30 patients. The mean follow-up period was 49 months. Results All of the 7 patients with preoperative pulmonary vein obstruction(PVO)underwent repair of TAPVC at the first operation and survived. When a systemic to pulmonary(S-P)shunt or pulmonary artery banding(PAB)was necessary,patients with concomitant TAPVC repair had lower mortality(30.0%)and higher total cavopulmonary connection completion rate(20.0%)than those without TAPVC repair[75.0% and 0%,respectively],although with no statistical significance(Fisher test,P=0.505 and P=0.245). The overall survival rates for the cohort at 1,3 and 5 years after the initial surgical intervention were 80.0%,77.1% and 77.1%,respectively. Multivariate COX regression analysis detected that SP shunt(adjusted odds ratio:6.51;95%CI:1.26-33.71,P=0.030)is the only risk factor for survival. The reintervention rate for postoperative PVO was higher in patients with preoperative PVO than those without(57.1%vs. 4.3%,Log Rank:P=0.006). Conclusion The mid-term results of surgical repair of FSV-TAPVC are still challenging. When PVO exists,surgical repair for TAPVC is suggested as soon as possible. Repair for TAPVC should also be considered when S-P shunt or PAB is necessary. Further study with larger population are warranted to support our finding.

Long-Term Outcomes of Systemic-to-Pulmonary Artery Shunt in Patients with Functional Single Ventricle and Heterotaxy Syndrome

Objectives: We sought to determine the long-term outcomes and mortality-associated factors after systemic-topulmonaryartery shunt (SPS) in patients with heterotaxy syndrome. Methods: We retrospectively analyzed allpatients with a functional single ventricle and heterotaxy syndrome who underwent SPS at our center from January2001 to April 2022. Results: This study involved 84 patients. Restrictive pulmonary blood flow requiringearly modulation was presented in 34 (40%) patients. Compared with patients without restrictive pulmonaryblood flow (N = 50 [60%]), the postnatal survival of these 34 patients was significantly lower at 10 years (log rank:p = 0.04), but the statistical significance disappeared at 20 years (log rank: p = 0.18). Among 31 patients whounderwent SPS, 11 (35%) had extracardiac total anomalous pulmonary venous connection (TAPVC). The survivalrate after SPS was 80% at 10 years. Cox regression analysis showed that extracardiac TAPVC (hazard ratio6.44, 95% confidence interval 1.23–33.7, p = 0.03) and pulmonary venous obstruction (PVO) at TAPVC repair(hazard ratio 11.2, 95% confidence interval 2.13–58.5, p = 0.004) were significantly associated with death. In25 patients who underwent bidirectional cavopulmonary shunt (BCPS), surgical interventions on the pulmonaryartery (PA) were performed after SPS in 7 of 9 patients with PA coarctation, 3 of 4 with non-confluent PAs, and4 of 12 with normal PAs. At SPS, primary central PA plasty was performed in three patients with PA coarctationand 2 with non-confluent PAs. There was no significant difference in the PA index before BCPS between patientswith and without primary central PA plasty (p = 0.49). Among 20 patients who underwent total cavopulmonaryconnection (TCPC), adverse events occurred in 7 (35%) patients, including death in 1 (5%), intervention for pulmonaryarteriovenous malformation (PAVM) in 3 (15%), and surgical intervention for PVO in 3 (15%). TheB-type natriuretic peptide concentration was significantly higher in patients with than without adverse events(p = 0.02). The adverse event-free survival rate after TCPC was 69% at 10 years. Conclusion: ExtracardiacTAPVC and PVO at TAPVC repair were significantly associated with death after SPS in patients who had heterotaxysyndrome with a single ventricle. Surgical interventions on the PA were frequently required after SPS inpatients with PA coarctation or non-confluent PAs. Although satisfactory survival was achievable after TCPC,late-onset PAVM and PVO remain concerns.

超声心动图在评估完全型肺静脉异位引流合并限制型与非限制型房间隔交通中的应用价值分析

目的 总结超声心动图在评估完全型肺静脉异位引流(TAPVC)合并限制型与非限制型房间隔交通中的应用价值。方法 对2020年1月至2022年6月在首都医科大学附属北京安贞医院经外科手术确诊的49例TAPVC患者的超声心动图及临床资料进行回顾性分析。设定超声心动图测量房间隔交通≤5 mm为限制型房间隔交通,将患者分为限制组(23例)和非限制组(26例)。分别于术前及术后1、3个月行超声心动图检查,测量左心室内径(LVD)、右心室内径(RVD)、三尖瓣反流峰值压差(PTR)。分析并比较2组患者术前、术后心室内径和PTR的变化。结果 49例患者术前超声心动图诊断均与手术诊断一致,符合率100%。术前,限制组LVD、LVD/RVD比值明显低于非限制组,而PTR、RVD则明显高于非限制组,差异均有统计学意义(均P<0.01)。术后1、3个月2组PTR、RVD和LVD/RVD比值比较差异均无统计学意义(均P>0.05),限制组LVD小于非限制组[(19.3±4.2)mm比(22.3±4.6)mm、(22.6±3.8)mm比(25.9±3.7)mm],差异均有统计学意义(均P<0.05)。结论 超声心动图是诊断TAPVC的首选及准确的检查方法,早期诊断有利于尽早手术治疗,并能有效评价心脏容量改变及肺动脉高压下降情况,具有较高的临床应用价值。

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。

完全性肺静脉异位连接的围生期一体化管理

目的 评价经产前超声心动图诊断的完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)胎儿围生期一体化管理的临床有效性。方法 回顾性分析2017年1月-2021年12月在青岛市妇女儿童医院经产前超声心动图诊断的64例TAPVC胎儿围生期一体化管理的临床资料。围生期一体化管理是指对产前诊断为TAPVC的胎儿联合产科、胎儿医学科、超声科、小儿心脏外科、小儿麻醉科及新生儿科进行多学科联合诊治。结果 64例TAPVC胎儿中,心上型29例,心内型27例,心下型2例,混合型6例。42例胎儿进行了染色体核型分析,均未见明显异常。64例TAPVC胎儿中,37例被引产,27例随访至足月出生。27例TAPVC患儿中,2例放弃治疗,2例转诊至外院治疗后失访,余23例进行一期根治手术。1例因低心排综合征于术后6个月内死亡,余22例随访(2.1±0.3)年,预后均良好(2例分别因吻合口狭窄、肺静脉狭窄在术后1年内接受了第2次手术)。结论 TAPVC胎儿经围生期一体化管理多可获得良好预后。

完全型肺静脉异位引流的DSCT诊断价值及研究进展

完全型肺静脉异位引流(TAPVC)指全部肺静脉未正常回流至左心房的一类左向右分流型先天性心脏病,约占新生儿先心病发病率的1%-3%,其多变的解剖类型及肺静脉狭窄程度直接影响病人的临床症状、外科修复方式及预后。双源CT(DSCT)因时间及空间分辨率高、明显减低的辐射剂量及强大的后处理功能,能对TAPVC患者异位肺静脉起源、走行路径、连接位置、血管床状态、其他伴发畸形及心腔大小进行详细评估,成为TAPVC术前、术后随访必要的无创性检查。本文现就TAPVC的发生发展、病理生理学改变及在DSCT检查中的影像特征、价值及研究进展做一简要综述。

产前超声对肺静脉异位引流合并心脏畸形的诊断价值

目的探讨胎儿肺静脉异位引流(anomalous pulmonary venous connection,APVC)合并心脏畸形的产前超声心动图特点及漏误诊分析,为提高APVC的超声诊断准确率提供参考依据。方法回顾性分析2016年至2021年湖北省妇幼保健院产前超声心动图诊断APVC合并复杂心脏畸形患者29例的临床资料,其中病理解剖证实的APVC 12例,17例失访。分析对比12例APVC胎儿期超声心动图图像及病理解剖结果,总结超声心动图特征及漏误诊原因。结果29例APVC胎儿中,完全型APVC 24例:心上型15例,心下型8例,心内型1例。部分型APVC5例,心上型3例,心下型1例,心内型1例。2例存在共同肺静脉腔及门静脉汇合处梗阻;主要合并的心脏畸形有心内膜垫缺损11例,无脾综合征12例,肺动脉闭锁7例,单心房、单心室7例。病理解剖12例,其中完全型APVC 8例:心上型4例,心下型3例,混合型1例;部分型APVC 4例:心上型2例,心下型1例,心内型1例。合并心脏畸形中,完全性心内膜垫缺损8例,肺动脉狭窄7例,单心房和(或)单心室5例,右心房异构5例;心外畸形中脾脏缺如7例,内脏反位7例,腹主动脉与下腔静脉位于脊柱同侧,符合无脾综合征特征7例。结论产前超声可准确诊断APVC及分型,APVC合并心脏畸形多伴有脾脏缺如,可有特征性无脾综合征表现。

完全性肺静脉异位引流潜在致病基因PDX1突变对其基因功能的影响

目的·采用全外显子测序技术筛查完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的可能致病基因,并对其功能进行验证。方法·选择2014—2019年在上海交通大学医学院附属新华医院及上海儿童医学中心确诊的100例TAPVC患儿(患儿组)以及120例健康儿童(对照组)为研究对象。收集2组儿童的血液样本,抽提全血基因组DNA并行外显子测序,以筛查TAPVC的潜在致病基因。通过Mutation Taster、SIFT、PolyPhen-2网站筛选致病基因的有害突变位点,并行Sanger测序。构建PDX1野生型(野生组)及突变型(突变组)质粒,转染入HUVEC细胞后,分别采用实时荧光定量PCR (quantitative real-time PCR,qPCR)和蛋白质印迹法检测突变对PDX1的mRNA和蛋白水平的影响。采用STRING数据库进行蛋白与蛋白之间的相互作用分析,并采用qPCR研究PDX1调节的下游基因的表达。结果·在TAPVC患儿中发现致病基因为PDX1, Sanger测序显示该基因存在2个新发突变,即c.C237A(P33T)和c.C237G(P33A)。与野生组相比,2个突变组(CA组、CG组)的PDX1 mRNA水平没有显著变化,但蛋白相对表达量有明显增加,即分别是野生组的2.9倍和3.4倍(P=0.000,P=0.001)。蛋白质相互作用分析的结果显示,PDX1与SOX17相关联;且qPCR结果显示,PDX1过表达可下调HUVEC细胞中SOX17的表达。结论·PDX1的2个新发错义突变可影响其转录后翻译,且PDX1可能是通过调控SOX17来参与TAPVC的发生与发展。

婴儿完全性肺静脉异位引流外科治疗风险因素和随访研究

目的探讨婴儿期外科治疗完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)的风险因素,分析不同类型TAPVC外科治疗随访结果的差异。方法总结广州市妇女儿童医疗中心2009年1月至2019年12月婴儿期手术治疗的TAPVC患儿的资料,同时对这些患儿进行随访。将术前有关因素与住院手术死亡进行单因素相关性分析和多因素相关性分析;将全部患儿按TAPVC的类型分组,分别用Kaplan-Meier法生成术后各组的近-中期生存曲线,并对这些生存曲线用log-rank法进行总体差异性检验,同时对各组间的差异性进行检验。结果手术患儿246例,手术死亡16例,手术病死率为6.50%。心内型、心上型、心下型、混合型分别为91例、106例、34例、15例,分别手术死亡2例、5例、6例、3例,病死率分别2.20%、4.72%、17.65%、20.00%。单因素分析显示与手术死亡有相关性的因子有TAPVC类型、术前休克/酸中毒、体外循环时间、主动脉阻断时间、手术过程中有深低温停循环;多因素分析显示与手术死亡有相关性的因子有TAPVC类型、术前休克/酸中毒、体外循环时间、主动脉阻断时间、手术过程中有深低温停循环、手术时低体质量。全部手术存活患儿随访死亡19例,用Kaplan-Meier法生成生存曲线,术后1年、3年、6年、9年总体累积生存率分别为89%、86%、85%、85%。随访死亡患儿中,心上型、心内型、心下型、混合型分别为6、5、8、0例。各组术后1年、3年、6年、9年累积生存率分别为心内型92%、92%、92%、92%;心上型93%、90%、89%、89%;心下型70%、54%、54%、54%;混合型80%、80%、80%、80%。术后各组生存曲线比较,差异有统计学意义(P<0.05):心下型术后累计生存率显著低于心内型和心上型。结论婴儿期TAPVC的外科治疗独立风险因子有TAPVC类型、术前休克/酸中毒、体外循环时间、主动脉阻断时间、手术过程中有深低温停循环、手术时低体质量;婴儿期TAPVC的心内型、心上型、混合型、心下型术后近中期累计生存率总体上有显著差异;心下型术后累计生存率显著低于心内型、心上型。

137例婴幼儿完全性肺静脉异位引流各年龄段临床特点和外科疗效分析

目的:探讨婴幼儿完全性肺静脉异位引流(TAPVC)各年龄段的临床特点和外科治疗效果。方法:2000-01至2006-12诊断TAPVC并在我院进行手术治疗的婴幼儿(除外合并法乐四联症、右心室双出口、完全性房室通道、单心室等复杂畸形的患儿)共137例。分为≤28天新生儿组(组1,n=7)、>28天～≤6个月组(组2,n=70)、>6个月～≤1岁组(组3,n=26)、>1岁～≤3岁组(组4,n=34)4个年龄组。137例患儿伴房间隔缺损119例,卵圆孔未闭18例,动脉导管未闭27例,室间隔缺损2例,三尖瓣关闭不全103例。合并重度肺动脉高压50.4%(69/137),其中≤6个月患儿(组1、组2)占71.0%(49/69)。心上型49.6%(68/137),心内型37.2%(51/137),心下型1.5%(2/137),混合型11.7%(16/137)。合并肺静脉狭窄18.2%(25/137),其中≤6个月患儿(组1、组2)占88.0%(22/25)。结果:手术死亡率11.7%(16/137),组1和组2占87.5%(14/16)。主要死亡原因为肺部感染和低心排综合征。有随访资料99例,随访率81.8%(99/121),随访期限2～86个月,平均(25.5±22.3)个月。晚期死亡9例,均为组1和组2患儿,晚期死亡率9.1%(9/99)。结论:年龄>6个月的TAPVC患儿可获得满意的外科治疗效果,而年龄≤6个月尤其是新生儿患儿病情重,合并限制性房间隔缺损、肺静脉狭窄和重度肺动脉高压比例高,往往需要急诊手术,手术及远期死亡率较高。

完全性肺静脉畸形引流的彩色多普勒超声诊断

目的 :探讨彩色多普勒超声诊断完全性肺静脉畸形引流 (TAPVC)的价值。方法 :应用HP2 5 0 0、Angilet5 5 0 0型彩色多普勒超声诊断仪检查 7例TAPVC患者 ,常规行二维超声心动图及彩色多普勒血流显像检查 ,主要观察心腔大小、肺静脉左房开口、房间隔缺损部位、大小及分流方向 ,跟踪扫查共同静脉干的走行及开口部位。结果 :7例TAPVC患者中 ,心上型 5例 (ⅠA1例 ,ⅠB1例 ) ,心内型 2例 (ⅡA1例 ,ⅡB1例 ) ,检查发现本组患者均有右房、右室明显增大 ,左房、左室较小 ;继发孔型房间隔缺损 ;左房内不能探及肺静脉开口 ,在心脏后方探及一粗大管道 (CPV) ,其结果与手术完全一致 ,彩色多普勒超声正确显示TAPVC部位。结论 :应用彩色多普勒超声可确定TAPVC的诊断和分型 ,为制定手术方案提供重要依据。

混合型肺静脉异位引流的彩色多普勒超声心动图诊断

目的:探讨彩色多普勒超声心动图对混合型肺静脉异位引流(MTAPVC)的诊断价值。方法:经心导管及手术证实为MTAPVC的11例患者为研究对象,回顾性分析其超声心动图。结果:患者可分三种类型:A型(心上型合并心内型)8例;B型(心上型合并心下型)2例;C型(心上型,合并有两个回流部位)1例。彩色多普勒超声心动图对MTAPVC诊断的正确率为81%(9/11)。结论:如能多部位、多切面仔细观察,彩色多普勒超声心动图可以比较准确地诊断混合型完全性肺静脉异位引流。

心下型完全性和部分性肺静脉异位引流的超声诊断经验

目的:与外科手术结果对比分析,探讨超声心动图在心下型完全性和部分性肺静脉异位引流诊断中的应用。方法:2008-01至2012-12,经外科手术证实为心下型肺静脉异位引流的7例患者为研究对象,其中6例为完全性肺静脉异位引流,1例为部分性肺静脉异位引流,分析其超声心动图表现及诊断要点,与外科手术结果对比分析。结果:6例心下型完全性肺静脉异位引流患儿中,超声心动图均明确诊断,超声心动图诊断要点为共同肺静脉干走行向右下穿过膈肌,汇入门静脉或下腔静脉,但4例患儿合并动脉导管未闭,2例合并肺静脉狭窄,超声心动图仅提示1例肺静脉流速偏快。1例心下型部分性肺静脉异位引流患者,初次超声漏诊,术前复查时参考磁共振成像结果,发现异位引流的肺静脉走行途径。结论:超声心动图可以发现心下型完全性肺静脉异位引流的异常血流走行及汇入部位,可以准确诊断心下型完全性肺静脉异位引流,并具有无创、简便等优点,但应注意肺静脉狭窄等合并畸形的诊断。应加强对心下型部分性肺静脉异位引流的认识,有意识去剑突下切面探查以提高其诊断率。

完全性肺静脉异位引流诊断探讨(附91例报告)

目的 探讨完全性肺静脉异位引流 (TAPVC)诊断方法。方法 总结 1986年 10月 -2 0 0 2年 2月收治入院的91例TAPVC的临床及辅助检查资料。结果 超声心动图与心导管造影比较诊断符合率 10 0 % ,引流位置准确率为 96.7%( 88/91)。 91例进行心导管造影检查明确了类型。 1例心下型TAPVC行MRI检查 ,清楚地显示了肺静脉回流情况及伴发畸形 ,与心导管造影结果一致。结论 心导管造影对本病是一个重要的检查手段 ,尤其对一些复杂型TAPVC、年龄大、临床症状重的患儿 ,为其外科手术提供了正确的解剖及生理数据。超声心动图能较准确地反映完全性肺静脉异位引流部位及其类型 ,但必须在超声心动图能明确排除有混合性异位引流或复杂畸形时才可直接手术。磁共振 (MRI)新技术应用于临床为本病的诊断提供了新方法并弥补了心导管造影的不足 ,尤其是心下型者。