完全性肺静脉畸形连接的放射诊断

本文分析56例经手术和或尸检证实的完全性肺静脉畸形连接(TAPVC)的造影所见及亚型变异,并对该畸形造影方法的选择进行了探讨。文内还结合造影征象评估了各型TAPVC的平片诊断价值及限度。     

完全性肺静脉异位连接

完全性肺静脉异位连接山东医科大学附属医院（２５００１２）宋惠民完全性肺静脉异位连接（ＴＡＰＶＣ）的主要病理解剖是左、右全部肺静脉血在心背侧汇合成肺后静脉，注入冠状静脉窦；肺总静脉经垂直静脉转入锁骨下静脉至上腔静脉，经肝、门静脉等转入右心系统。１分型根...     

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。     

完全性肺静脉异位连接的外科治疗

手术治疗５例完全性肺静脉异位连接（ＴＡＰＶＣ）患者，均经Ｂ超声和彩色多普勒检查确诊。认为本病手术成功的关键是汇总静脉和左房的吻合口要足够大，术中在左心耳尖端缝１针牵引线牵拉可充分暴露术野，停止体外循环后左室压高者可部分放开垂直静脉。术后延长辅助呼吸时间并应用呼气末正压，限制液体入量为术后顺利过渡的重要措施。     

完全性肺静脉异位连接33例诊断体会

本文总结我科1990年1月-1996年12月共收治完全性肺静脉异位连接（TAPVC）33例。全部做彩色多普勒超声心动图检查，27例做心导管检查，21例做手术根治。心导管诊断符合率100％；超声心动图的诊断符合率96％，连接部位符合率96％，估测肺动脉压力为8．53±2．19kPa，与心导管对比无显著性差异。死亡4例，其中3例为＜1岁的婴幼儿。心导管仍是最准确的诊断方法，超声心动图有一定的价值，早期诊断、及时手术根治是治疗的唯一方法。     

完全性肺静脉异位连接的外科治疗

完全性肺静脉异位连接(TAPVC)是一种少见的复杂先天性心脏畸形。1999年2月至2004年6月,我们收治6例TAPVC,手术治疗均获成功。现报告如下。     

完全性肺静脉畸形引流的外科治疗

本院自1976年1月至1989年12月手术治疗完全性肺静脉畸形引流(TAPVC)58例。心上型38例,心内型19例,混合型1例。死亡5例,死亡率8.6%。本文对该病的发生率、诊断和手术方法进行讨论,着重介绍作者采用左右房联合切口矫治本病的经验。     

混合型完全性肺静脉畸形引流的外科治疗

目的 :对两例患儿实施混合型完全性肺静脉畸形引流矫治术。方法 :2 0 0 1年 3月～ 11月 ,我院分别为 1例 9个月的婴儿和 1例 4岁儿童成功实施混合型完全性肺静脉畸形引流矫治术。两患儿左肺静脉通过垂直静脉流入无名静脉 ,右肺静脉回流至冠状静脉窦。结果 :患儿术后恢复顺利 ,术后 7天出院。结论 :两患儿经外科矫治取得了良好的结果     

22例完全性肺静脉畸形引流的外科治疗

本文分析22例完全性肺静脉畸形引流手术常见并发症肺水肿的产生原因和预防,并对各种手术方法的优缺点作了探讨,认为心上型手术经心内途径进行左房和肺静脉总干吻合的方法优于心后途径。     

完全性肺静脉异位连接的手术治疗

目的观察完全性肺静脉异位连接（TAPVC）患者的手术治疗效果。方法在中度低温体外循环下对23例TAPVC患者行TAPVC矫治术。结果术后早期死亡1例;并发阵发性结性心律、短阵性房性心动过速、左侧膈肌麻痹各1例。22例出院后均获随访（2.8±1.5）a,1例心上型患者术后8个月因左肺静脉回流梗阻再次入院经手术治愈,1例反复发作房性心律失常经内科治疗无效。结论手术是治疗TAPVC的有效手段。     

Cross sectional echocardiographic diagnosis of total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection can be diagnosed by cross sectional echocardiography. Information is, however, lacking concerning the diagnostic accuracy of this imaging method and any factors which may influence it. To predict the pulmonary venous connection 463 patients with congenital heart disease who had angiographic confirmation were prospectively examined. Total anomalous pulmonary venous connection was present in 34 (7%) patients and correctly detected in 33 (97% sensitivity). There were two false positive results (99% specificity). All 23 patients with atrial situs solitus with or without associated congenital heart defects were correctly detected. One false negative result occurred in a patient with right atrial isomerism and complex congenital heart disease with decreased pulmonary blood flow. Diagnosis of the type of total anomalous pulmonary venous connection, including the site and other anatomical details, was analysed and was correct in 24 of 34 (71%) patients. Errors included incorrect prediction of the site of total anomalous pulmonary venous connection in five patients with right atrial isomerism, atrioventricular canal defect, and pulmonary atresia, details of confluence interconnection in three of four patients with the mixed type of connection, undiagnosed pulmonary venous obstruction in three of the patients with right atrial isomerism, and failure to predict common pulmonary vein atresia in one patient. Factors which were related to incorrect echocardiographic diagnosis were abnormal atrial situs, mixed total anomalous pulmonary venous connection, and associated congenital cardiac defects, whereas age, weight, sex, clinical condition, and time during the study were not related. It is concluded that cross sectional echocardiography can be used to diagnose accurately total anomalous pulmonary venous connection. This method can be the definitive imaging and diagnostic method in symptomatic infants with total anomalous pulmonary venous connection who have atrial situs solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defect.     

Echocardiographic diagnosis of obstructed supracardiac total anomalous pulmonary venous connection

Obstruction in supracardiac total anomalous pulmonary venous connection (TAPVC) may be seen in 50% of cases. We describe here successful echocardiographic diagnosis of obstruction at an unusual and undescribed location in a supracardiac TAPVC. The patient was successfully managed by surgery.     

Cross sectional echocardiographic diagnosis of total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection can be diagnosed by cross sectional echocardiography. Information is, however, lacking concerning the diagnostic accuracy of this imaging method and any factors which may influence it. To predict the pulmonary venous connection 463 patients with congenital heart disease who had angiographic confirmation were prospectively examined. Total anomalous pulmonary venous connection was present in 34 (7%) patients and correctly detected in 33 (97% sensitivity). There were two false positive results (99% specificity). All 23 patients with atrial situs solitus with or without associated congenital heart defects were correctly detected. One false negative result occurred in a patient with right atrial isomerism and complex congenital heart disease with decreased pulmonary blood flow. Diagnosis of the type of total anomalous pulmonary venous connection, including the site and other anatomical details, was analysed and was correct in 24 of 34 (71%) patients. Errors included incorrect prediction of the site of total anomalous pulmonary venous connection in five patients with right atrial isomerism, atrioventricular canal defect, and pulmonary atresia, details of confluence interconnection in three of four patients with the mixed type of connection, undiagnosed pulmonary venous obstruction in three of the patients with right atrial isomerism, and failure to predict common pulmonary vein atresia in one patient. Factors which were related to incorrect echocardiographic diagnosis were abnormal atrial situs, mixed total anomalous pulmonary venous connection, and associated congenital cardiac defects, whereas age, weight, sex, clinical condition, and time during the study were not related. It is concluded that cross sectional echocardiography can be used to diagnose accurately total anomalous pulmonary venous connection. This method can be the definitive imaging and diagnostic method in symptomatic infants with total anomalous pulmonary venous connection who have atrial situs solitus, unifocal pulmonary venous connection, and no evidence of other major congenital cardiac defect.     

完全性肺静脉异位引流的手术治疗(附44例报告)

目的 探讨完全性肺静脉异位引流（TAPVC）的手术方法和治疗经验。方法对44例TAPVC患者施行手术治疗,其中心上型29例,心内型13例,心下型2例。手术在中度低温体外循环下进行．心上型早期行心脏上翻法吻合、中期行左右房联合切口法,近期改良为心上吻合法。心下型采用心脏上翻纵切口吻合。结果手术死亡2例（心上型和心内型各1例）;心律失常12例,出院时恢复安性心律。结论TAPVC一经确诊．必须立即手术,以防止肺血管阻塞性病变的发生。     

Repair of total anomalous pulmonary venous connection in adults

Three cases are presented of adults who underwent successful surgical correction of total anomalous pulmonary venous connection of the supracardiac type. Adults rarely present with this unusual condition but our experience suggests that correction can be performed, using the same techniques that are used in children, with a low operative risk and achieving similar long-term results.