CT血管成像与经胸超声心动图诊断部分型肺静脉异位引流

目的 对比分析CT血管成像（CTA）与经胸超声心动图（Trans Thoracic Echocardiography，TTE）对部分型肺静脉异位引流（PAPVC）的诊断价值。方法 回顾性分析21例PAPVC患者的CTA及TTE特点，并将二者检出情况与手术结果进行对比分析。结果 单纯性PAPVC 2例，CTA及TTE均检出。复杂性PAPVC 19例，CTA误诊1例，TTE误诊6例。CTA与TTE对PAPVC检出率分别为95.2%（20/21）和71.4%（15/21），两者差异具有统计学意义（P＜0.05）。对于已检出的病例，CTA漏诊的合并其他畸形主要为房室间隔缺损和细小血管畸形，而TTE主要为心外血管畸形。结论 TTE的优势在于心内细致结构、尤其是瓣膜病变的检出，可作为PAPVC首选筛查方法。CTA的优势在于心外大血管畸形的检出，可作为TTE的有效补充手段。对于部分肺静脉回流显示不清、疑有心外血管异常及合并其他畸形者应联合两种检查方法，才能最大程度降低漏诊误诊的发生。     

经胸和经食道超声心动图评价成人部分型肺静脉异位连接

目的通过经胸和经食道超声心动图对研究对象进行全面检查,探讨部分型肺静脉异位连接（partial anomalous pulmonary venous connection,PAPVC）的解剖学特征及其与手术的相关性。方法对2008年6月至2011年7月36例（男12例,女24例,年龄15—59岁,中位年龄37岁）经外科手术证实的先天性PAPVC患者的临床资料进行回顾性分析,所有患者均行经胸超声心动图检查。27例进一步行经食道超声心动图检查,所得诊断结果与外科手术诊断结果进行比较。结果36例PAPVC均为右肺静脉异位连接,其中两条右肺静脉21例（58．3％）,单纯右上肺静脉11例（30．5％）,单纯右下肺静脉4例（11．1％）。合并静脉窦型缺损33例（91．6％）。经胸及（或）经食道超声心动图联合检查,与手术结果对比,正确诊断PAPVC31例（86．1％）。结论超声心动图在对PAPVC的诊断中起到重要作用,为外科术前提供足够的信息,可作为PAPVC诊断的首选检查方法。     

右前侧胸小切口心内直视手术106例

<正> 自1997年5月至1999年5月,我们采用右前侧胸部小切口径路,选择性在体外循环下施行心内直视手术106例,效果满意,现报告如下.1 临床资料与方法1.1 一般资料全组共106例,男41例,女65例.年龄5～34岁,平均11岁.成人24例,体重14～55Kg,平均19Kg.病种有室间隔缺损(VSD)73例(膜周部室间隔缺损69例,嵴上型4例,缺损直径4～12mm,平均8mm);房间隔缺损(ASD)21例(中央型16例,下腔型5例);房间隔缺损并室间隔缺损5例;部分型肺静脉畸形引流3例(PAPVC);风湿性二尖瓣狭窄并关闭不全(MSI)及部分性房室管畸形(PAVC)各2例.本组患者平均心胸比率0.53;心功能Ⅰ级2例,Ⅱ级92例,Ⅲ级12例.     

先天性主动脉弓病变合并心内畸形新生儿期手术的临床分析

目的:探索先天性主动脉弓病变合并心内畸形新生儿期手术的治疗策略。方法:回顾性分析我院2013年1月1日至2016年12月31日,先天性主动脉弓病变合并心内畸形21例新生儿期手术的临床资料,其中男性11例,女性10例;年龄7~28d,平均天数(13±5)d;体质量2.1~5.5kg,平均体质量(3.5±1.2)kg。其中18例为一次性根治;3例为其他手术方式。结果:主动脉弓病变合并心内畸形21例新生儿中,19例成功治愈出院。手术成功率90.5%,治疗效果较显著。结论:个体化治疗方案,可以有效提高主动脉弓病变合并心内畸形的新生儿的治疗成功率。     

迷宫Ⅲ型手术治疗房颤时的体外循环管理

1995年10月至2003年11月,我们对37例心脏病合并房颤的患者,实施心内直视手术同时加行迷宫Ⅲ型手术治疗房颤,现将手术过程中的体外循环管理报告如下。     

右前胸小切口治疗先天性心脏病65例

目的总结应用右前胸小切口进行心内直视手术的治疗体会.方法1999年3月-2003年6月,我们采用右前胸小切口行心内直视下修补先天性房、室间隔缺损65例,其中房间隔缺损37例,室间隔缺损28例.结果所有患者无手术死亡及手术并发症.结论经右前胸小切口进行心内直视手术是一种安全可靠的微创手术,对一些简单的先天性心脏病有良好的手术和美容效果,但不适合比较复杂的心脏病手术.     

不同类型无顶冠状静脉窦综合征手术治疗13例临床观察

目的:分析无顶冠状静脉窦综合征(UCSS)的外科手术方法,包括经右腋下剖胸小切口入路手术的治疗经验,为此类疾病的手术处理提供参考。方法 :回顾性分析2010年3月至2013年6月于北京安贞医院小儿心脏中心行手术矫治的13例UCSS患者的临床资料,本组病例术前经超声确诊率为54%(7/13),经手术中探查,其中I a型7例,II b型1例,III a型4例,III b型1例。合并左上腔静脉(LSVC)引流入左心房11例,采用自体心包缝制心内隧道9例,左心房后壁折叠式隧道窦顶重建1例,直接结扎LSVC 1例。全组病例均合并其他心内畸形,手术同期根治合并畸形。10例经右腋下剖胸小切口入路行根治术。结果:本组手术无死亡病例。1例术后并发肺部感染,1例术后并发右侧膈肌麻痹行膈肌折叠术,分别治疗18天和24天后痊愈出院。其余11例患儿术后早期恢复顺利,术后呼吸机使用时间平均33.8小时;住院天数(11.6±5.3)天;全组病例均行随访,随访时间平均18.9个月,其中10例经右腋下剖胸小切口入路行矫治术,无死亡及并发症。结论:UCSS的术前漏诊率较高,需经术中仔细探查。根据分型及合并畸形选择最佳处理方法。右腋下剖胸小切口入路手术根治多数心内畸形合并UCSS,治疗效果良好。     

完全性肺静脉异位连接的外科治疗

目的:探讨完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)的手术方法及疗效。方法:选取5例TAPVC患者,其中1例心上型患者行左心房共同静脉吻合;1例心内型患者将房间隔缺损扩大,应用补片将异位引流的肺静脉隔至左心房;3例混合型患者中有2例垂直静脉与左心耳吻合,补片将异位引流的肺静脉共同腔隔至左心房,1例扩大房间隔缺损后行心包片补片将异位引流的肺静脉隔至左心房。结果:所有患者均痊愈并获得随访,随访时间8个月～2年,心功能基本正常。结论:TAPVC患者一旦诊断明确应及早手术,正确认识肺静脉复杂的解剖,选择适当的手术方法可获良好的治疗效果。手术治疗的关键在于肺静脉流入左心房足够宽畅,避免狭窄。     

低体重危重婴幼儿先天性心脏病的早期心内直视手术治疗

目的:回顾性分析低体重婴幼儿危重先天性心脏病早期心内直视手术的效果.方法:对17例出生体重<2 500 g的患儿在中低温体外循环下行心内直视矫治术.结果:手术效果满意,手术成功16例.结论:患先天性心脏病的低体重患儿,若临床心脏症状危重,早期行体外循环心内直视手术可有较满意的效果.     

42例婴幼儿完全性肺静脉异位引流的治疗体会

目的:总结婴幼儿完全性肺静脉异位引流的早期诊断和治疗经验。方法:1995年3月至2005年3月,我院共完成42例完全性肺静脉异位引流的手术治疗,男33例,女9例,年龄18天～3岁,平均(4.3±3.2)月,体重3.2～11kg,平均(5.4±1.2)kg。心上型27例,心内型10例,心下型3例,混合型2例。所有患者均由超声心动图确诊,11例行心导管检查,3例心下型行磁共振成像检查。均在全麻低温体外循环下行矫治术。6例心上型采用左心房顶部入路。结果:死亡4例,死亡率9.52%(3例心上型围术期死亡,1例心内型术后1年因肺静脉回流梗阻死亡)。3例心下型无死亡。心律失常7例中,6例为心上型双心房横切口,1例为心内型。随访39例,随访时间1月～10年,存活者心功能明显改善。结论:完全性肺静脉异位引流可由超声心动图确诊,必要时辅以心导管检查及磁共振成像检查。新生儿及婴儿期手术可获得良好效果。心上法矫治心上型可减少心律失常的发生率。     

Diagnostic value of real-time three-dimensional transesophageal echocardiography for preoperative assessment of sinus venosus atrial septal defect

Background Sinus venosus atrial septal defect（SVASD） is a rare atrial septal defect. The right-sided pulmonary veins may drain into the right atrium directly or via vena cava. Two-dimensional transthoracic echocardiography（2D-TTE） is certainly the initial choice of evaluation of SVASD but is unable to show its spatial relation to the surrounding structures and the configuration of the veins accurately. The purpose of this study was to evaluate the diagnostic accuracy of real-time three-dimensional transesophageal echocardiography（RT3D-TEE） for detecting SVASD and partial anomalous pulmonary venous connection（PAPVC）. Methods2D-TTE and RT3D-TEE were performed in 25 patients with the SVASD before surgical repair. Records of these patients were compared with surgical assessments. Results Twenty-three of the 25 patients with SVASD were detected by 2D-TTE. All the 23 patents were associated with PAPVC. Two of the 25 patients with SVASD were misdiagnosed as the secundum atrial septal defect by 2D-TTE. SVASD was demonstrated in all the 25 cases by RT3D-TEE, and 25 patents were associated with PAPVC. Conclusions RT3D-TEE is highly accurate, scientific method for displaying the location, size, shape of the defect and its spatial relation to the surrounding structures, and provides additional value to the surgeon and physicians for better understanding of spatial intracardiac morphology and making more accurate diagnosis and treatment.     

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.     

Unusual venacaval anomalies in a patient with atrial septal defect

An unusual systemic venous drainage pattern was found in a 30-year-old man with ostium secundum atrial septal defect and pulmonary stenosis. He had the rare association of absent right superior vena cava, persistent left superior vena cava draining into the coronary sinus, and a left-sided inferior vena cava draining into a left superior vena cava through the hemiazygous vein.     

Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Sinus venosus atrial septal defect (SV‐ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV‐ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio‐CT was finally conclusive. This diagnostic approach allowed the surgical planning.     

The Warden Procedure through a Posterolateral Thoracotomy Approach

The Warden procedure for the correction of a right-sided partial anomalous pulmonary venous connection to the high superior vena cava is well established. It has the advantages of avoiding sinoatrial node dysfunction and pulmonary and systemic venous obstruction. In the case related here, a 3-year-old girl presented with a superior vena cava type of sinus venosus atrial septal defect and an anomalously draining right upper pulmonary vein, with bilateral superior venae cavae. Our approach to the Warden procedure was through a right posterolateral thoracotomy, which provided additional advantages.     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流（total anomalous pulmonary venous drainage，TAPVD）的外科治疗经验。方法TAPVD27例（心上型15例，心内型12例）均在体外循环下手术。心上型6例采用传统双心房联合切口，9例采用心上法，后者经上腔静脉与升主动脉之间显露左心房与共同静脉干行-一侧吻合：心内型切开房间隔缺损与冠状静脉开口之间的残余房间隔壁，自体心包将肺静脉开口与冠状静脉窦隔入左心房。结果早期死亡2例，其他病人痊愈出院，术后随访3个月-4年，恢复良好，无吻合口狭窄发生，出院后无因心、肺衰竭死亡病人。结论TAPVD早期诊断极为重要，手术治疗的关键在于左心房与肺总静脉吻合口应足够大，避免狭窄的发生。     

Successful Correction of a Total Anomalous Venous Connection in a 63-year-old Male—Case Report and Review of the Literature

Total anomalous pulmonary venous connection is a rare variant of cyanotic congenital heart disease and usually requires surgical correction within the first few months of life. We report midterm results of a 63-year-old male with intracardiac total anomalous venous return into the coronary sinus who presented with congestive predominantly right heart failure and underwent corrective surgery with unroofing of the coronary sinus and patch closure of the secundum atrial septal defect.     

Subdivided left atrium: an expanded concept of cor triatriatum sinistrum.

Twenty-four hearts with cor triatriatum were studied. On the basis of this material and a review of the literature a new classification of "subdivided left atrium" is proposed. Tye A, the most common form of subdivided left atrium, is the classic cor triatriatum with its multiple variations of partial anomalous pulmonary venous drainage; the fossa ovalis can be related to the proximal left atrial chamber (type A, a) or the distal left atrial chamber (type A, b). Type B hearts are related to (but not identical with) total anomalous pulmonary venous drainage into the coronary sinus; the coronary sinus opening is atretic in these hearts; abnormal defects connect the proximal left atrial chamber usually with the right atrium only, rarely also with the distal left atrial chamber. The Type C heart, first reported in this paper, has a superiorly and medially situated proximal chamber, located between the right and distal left atrium; it does not receive any pulmonary veins; the coronary sinus is normally formed. Current morphogenetic hypotheses that satisfactorily explain the Type A b and B heart fail to account for the Type A a and C heart.     

无顶冠状静脉窦综合征的超声影像学及临床特征分析

目的:分析无顶冠状静脉窦综合征(unroofed coronary sinus syndrome,UCSS)患者超声影像学及临床特征.方法:回顾我院2007年1月至2020年1月临床确诊的140例UCSS患者超声影像资料,对其超声影像特征、合并畸形及继发改变进行分析.结果:140例UCSS患中,女性占62.1％(87/...     

Percutaneous Occlusion of Right Partial Anomalous Pulmonary Venous Connection with Dual Drainage to the Innominate Vein and the Left Atrium: A Unique Anatomical Finding

A 43-year-old woman with a past medical history of aortic coarctation surgically repaired at the age of 3 years using an end-to-end anastomosis, presented with 2 years complain of increasing dyspnea and fatigue with exercise associated to frequent palpitations. During extensive work-up, she was found to have a partial anomalous pulmonary venous connection (PAPVC) with “dual drainage” represented by a communication between the right pulmonary veins draining into the left atrium and the innominate vein via an anomalous vein due to a persistence of early connections between the sinus of the right pulmonary veins and the cardinal veins system in the splanchnic plexus and also a persistence of the proximal portion of the left anterior cardinal vein. She was successfully treated through a percutaneous implantation of a vascular plug occluding the vertical portion of the anomalous vein diverting the flow to the left atrium. To the best of our knowledge, this anatomical variant of partial anomalous pulmonary venous connection with dual drainage has not been previously reported.