静脉窦房间隔缺损的外科治疗

目的:总结我科1999年7月至2004年4月26例静脉窦房间隔缺损手术病例的临床资料,探讨静脉窦房间隔缺损的外科手术方式和经验。方法:采用浅低温体外循环心脏不停跳技术行静脉窦房间隔缺损及合并畸形的手术治疗。其中男性11例,女性15例,年龄6个月～27岁,平均(12.1±5.7)岁。合并部分肺静脉异位引流19例,其中引流入上腔静脉8例,引流入右心房11例;合并动脉导管未闭1例、左上腔静脉引流入冠状静脉窦2例。合并室间隔缺损1例,三尖瓣返流3例,二尖瓣重度返流1例。体外循环转流时间25～98min,平均(56.4±6.2)min,全组均用自体心包补片修补,术中8例用右心房外侧切口避开窦房结延长至上腔静脉,3例用另外心包加宽上腔静脉与右心房结合部;5例术中扩大房间隔缺损利于异位引流的肺静脉回流,1例同期行二尖瓣置换。结果:术后平均重症监护时间(4.2±2.1)d,平均住院时间(15±6.5)d。全组无死亡病例。1例术后早期窦房结功能失调,经使用临时起搏器辅助3d后恢复,术后1例转为心房颤动心律,余均为窦性心律。1例出现上腔静脉梗阻,二次手术加宽上腔静脉后好转。结论:运用经食道超声心动图较易诊断静脉窦房间隔缺损和确定有无肺静脉异位引流,外科手术治疗静脉窦房间隔缺损的效果满意,选择适当的手术方式可以降低上腔静脉梗阻、窦房结功能失调等并发症。     

胎儿冠状静脉窦扩张的超声心动图诊断及误诊原因分析

目的:探讨胎儿冠状静脉窦扩张的超声心动图诊断临床意义及误诊原因分析。方法:2010年3月至2013年5月间,7 639例孕妇于我院接受产前胎儿超声心动图检查。结合出生后随访结果,回顾性分析冠状静脉窦扩张胎儿的超声心动图表现。结果:7 639例中共检出胎儿冠状静脉窦扩张60例(0.8%),其中单纯左位上腔静脉(PLSVC)28例(47%,28/60);合并心内其他畸形32例(53%,32/60)。初诊时误诊为原发孔房间隔缺损3例。结论:产前胎儿超声心动图可以诊断冠状静脉窦扩张及其合并心内畸形,对妊娠期胎儿的管理具有重要诊断价值和意义。     

无顶冠状静脉窦综合征的外科处理策略

目的:总结无顶冠状静脉窦综合征(UCSS)的手术方法,为术中处理此类疾患提供借鉴.方法:入选UCSS 患者14例,男 8例,女6例,年龄2个月～35(10.55±10.63)岁,体重3.5～60(26.17±19.60)kg,其中完全型心内膜垫缺损4例,部分型心内膜垫缺损6例,室间隔缺损1例,法洛四联症2例,镜像右位心4例,伴中至重度肺动脉高压11例.14例均为永存左上腔静脉(PLSVC)直接汇入左心房,即完全型冠状静脉窦间隔缺损(Ⅰ型).心脏彩超明确诊断3例,提示伴左上腔静脉(LSVC)5例.14例中未行手术处理1例,采用心外管道连结PLSVC至右心耳5例,心内隧道重建冠状窦顶4例,建立心房内隔板2例,限制性环缩LSVC 1例,直接结扎LSVC 1例.结果:全组死亡3例,出现脑部并发症2例,延迟关胸致伤口延迟愈合1例,其余患者远期效果满意.结论:心内膜垫缺损或镜像右位心伴PLSVC时,要警惕UCSS的存在.根据PLSVC汇入左心房的位置选择不同的手术方法,可获得满意的手术效果.限制性环缩LSVC可能是处理该类畸形的又一方法.     

31例完全性肺静脉异位连接的外科治疗

目的探讨31例完全性肺静脉异位连接的手术方法及术后疗效。方法31例患者（心上型16例、心内型13例、混合型2例）均在体外循环下行矫治术。心上型6例采用传统双心房联合切口，10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肺静脉干行侧一侧吻合；心内型切开房间隔缺损与冠状静脉窦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口隔入左房；混合型则根据不同的病理解剖特点行相应的矫治术。结果早期死亡2例，其他患者都痊愈出院。术后随访3个月至14年，恢复良好，无吻合口狭窄发生，心功能均为Ⅰ级，无因心肺功能衰竭死亡患者。结论完全性肺静脉异位连接早期诊断和及早行矫治术极为重要，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。     

冠状静脉窦闭锁侧支循环途径的多排螺旋CT诊断和临床价值探讨

目的:探讨冠状静脉窦闭锁(CSOA)侧支循环途径的多排螺旋CT(MDCT)诊断价值。方法:对收集的37例CSOA患者影像学资料进行回顾性分析,了解冠状静脉的回流途径。患者在行MDCT前均常规行经胸超声心动图检查。比较MDCT和超声心动图对CSOA的检出率,分析不同CSOA类型在性别和回流方式上的差异。结果:37例CSOA患者中,MDCT诊断率100%,超声心动图7例提示冠状静脉窦扩张,1例诊断无顶冠状静脉窦综合征,无一例诊断CSOA。按CSOA长度不同,将37例患者分为隔膜型闭锁7例、节段型闭锁25例和弥漫型闭锁5例。按闭锁后心脏静脉回流途径分有5种方式,本组中11例通过心大静脉—左侧垂直静脉—上腔静脉—右心房;6例通过直接侧支静脉回流入右心房;6例通过冠状静脉窦末端直接与左心房相通,即无顶冠窦方式;2例通过单支或多支冠状静脉侧支回流入左心房;12例分别通过不同侧支既与左心房相通,也同时存在连接右心房,即混合型。不同CSOA类型在性别和回流方式上差异均无统计学意义(P均>0.05)。结论:不同CSOA类型在性别和回流方式上差异均无统计学意义。MDCT检查对CSOA的检出率明显高于超声心动图检查,可作为超声心动图检查的补充,二者结合可提供精准的诊断,为临床治疗保驾护航。     

部分性肺静脉异位引流矫治术后致顽固性引流液1例

患者,女,27 岁,因体检发现心脏杂音 4 年于2003年 5 月 29 日入院。患者一般情况良好, HR84次/min,律齐,胸骨左缘第 2 肋间可闻及Ⅲ级收缩期杂音,P2 亢进;心脏彩色 B超:左心房 43 mm,右心房86 mm,房间隔中上部24 mm回声中断,冠状静脉窦明显扩张,右肺上、下静脉引流入右心房。术前检查无手术禁忌证,在全麻体外循环下行部分肺静脉异位引流矫治术,术中见心包内淡黄色心包积液约 300 ml,右房明显扩大。常规建立体外循环,切开右房,见冠状静脉窦明显扩张,静脉血回流异常增多,发现永存左上腔静脉,遂游离之并套临时阻断带;房间隔缺损为继发孔…     

冠状静脉窦闭锁的诊断与临床意义

目的:评价冠状静脉窦闭锁(CSOA)患者的诊断与临床特点。方法:回顾分析2005-02至2014-05我院诊断的22例CSOA患者,其中男性15例,女性7例,年龄1.5~76[平均(50.88±14.97)]岁。22例患者均行心电门控心脏增强计算机断层摄影术(CT)。结果:22例患者经心电门控心脏增强CT诊断为CSOA。按患者冠状静脉内血液回流方向分类:9例患者血液回流入左心房[3例伴永存左上腔静脉(LSVC)且其内血液亦入左心房,其中2例合并复杂先天性心脏畸形];5例血液回流入右心房(直接回流入右心房2例,间接经LSVC回流入右心房3例);8例经细小分支血液既入左心房亦入右心房属混合型(4例伴LSVC)。CSOA患者9例伴LSVC。按患者LSVC内血液回流方向分:3例患者LSVC血液直接引流入左心房;6例患者LSVC血液经无名静脉-右上腔静脉-引流入右心房。超声心动图均未诊断CSOA。心电图显示5例患者有心律失常,其中3例射频消融成功,1例射频消融失败,1例行永久起搏器植入术。22例患者中6例合并先天性心内畸形(6/22,27.27%),其中1例伴房间隔缺损,5例伴复杂先天性心脏病(先心病)。复杂先心病患者中3例术前行心电门控心脏增强CT检查发现CSOA;另2例复杂先心病患者术后仍存在症状,经CT检查发现CSOA。5例复杂先心病患者均行手术,本组无因单纯CSOA行外科手术者。结论:明确诊断CSOA患者中心律失常、LSVC和心内畸形等并发情况,对预防心血管意外的发生有重要意义。心电门控心脏增强CT对CSOA的诊断有重要价值。     

200例胎儿先天性心脏病尸体解剖分析

目的:对胎儿先天性心脏病(先心病)进行尸体解剖分析,提高该疾病诊断的精准性。方法:回顾近几年我院经超声心动图畸形筛查,发现胎儿先天性心脏畸形引产而进行尸体解剖病例,分析胎儿心血管畸形不同类型的发病情况及特点,有关心血管畸形按心脏节段分析方法进行分析观察。结果:胎儿先心病尸体解剖200例,男性93例,女性107例,孕12~38周,胎儿先天性心脏病类型:心房/心耳畸形16例,体静脉畸形45例,肺静脉畸形28例,冠状静脉窦畸形4例;房间隔畸形55例,房室瓣畸形及骑跨24例,房室间隔缺损26例,室间隔畸形87例,左心发育不良综合征17例,右心发育不良综合征9例,法洛四联症12例,心室双出口21例,大动脉转位20例,心室单出口21例,主动脉瓣畸形14例;肺动脉瓣畸形28例;主动脉近段畸形73例,肺动脉畸形26例,动脉导管畸形18例;冠状动脉畸形6例;右旋心5例。常见畸形为室间隔缺损56例(28%)、房间隔缺损36(18%)和永存左上腔静脉35(17.5%),单心室31(15.5%),主动脉弓发育不良25(12.5%),右位主动脉弓23(11.5%)。结论:胎儿先心病室间隔缺损、房间隔缺损、永存左上腔静脉、单心室及主动脉弓发育不良发病率较高;胎儿心血管畸形变异较多,血管较细小,解剖时应仔细寻找每一个分支。     

永存左上腔患者起搏导线植入路径探讨:附28例分析

正永存左上腔静脉(persistent left superior vena cava,PLSVC)在正常人群中发病率为0.3%~0.5%,其中约12%的人群合并其他心血管异常[1-2]。最常见PLSVC为PLSVC直接流入冠状静脉窦,开口于右房,占PLSVC的80%~92%[1,3]。文献对PLSVC有多种分型报道[1,4-5]。但根据PLSVC流入心脏部位的不同,常见的为4种类型:Ⅰ型PLSVC流入冠状静脉窦,右上腔静脉缺如;Ⅱ型PLSVC左右上腔静脉均存在,两者之间不存在交通支;Ⅲ型PLSVC左     

无顶冠状静脉窦综合征分型超声诊断及临床价值探讨

目的:回顾性分析不同类型无顶冠状静脉窦综合征(UCSS)患者的超声心动图特征,合并畸形特点,分析漏误诊原因,提高该病术前诊断水平。方法:回顾性收集2014年10月至2019年10月,经我科手术并证实为UCSS的68例患者资料,总结其超声心动图特征并与手术结果相比较。结果:68例UCSS中,Ia型30例(44%),Ib型12例(18%),IIa型5例(7%),IIb型7例(10%),IIIa型6例(9%),IIIb型7例(10%),IIa+IIIa混合型1例(2%)。合并永存左上腔静脉(PLSVC)42例(62%),继发孔房间隔缺损(ASD)27例(40%),单心房12例(18%),心内膜垫缺损(EDC)17例(25%),室间隔缺损23例(34%),单心室5例(7%),心房异构8例(12%),右位心6例(9%),右心室双出口7例(10%),肺静脉异位引流5例(7%)。术前超声准确诊断并分型正确64例,准确率94%(64/68);漏诊4例,漏诊率6%(4/68)。结论:超声心动图是诊断不同类型UCSS及其合并畸形的首选方法,掌握UCSS不同类型超声特征及合并畸形特点有助提高术前诊断率,为临床治疗提供可靠依据。     

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.     

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.     

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/...