53岁心内型完全性肺静脉异位引流手术1例及文献回顾

完全性肺静脉异位引流(total anomalous pulmonary venous connection, TAPVC)是一种罕见的紫绀型先天性心脏病,缺氧和充血性心力衰竭可导致明显症状,通常患者在出生后几个月内需要手术矫正,成人病例在临床上比较少见,尤其是生存至中老年更是罕见。我们成功为1例53岁心内型TAPVC合并房间隔缺损、肺动脉高压患者实施手术。     

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。     

完全性肺静脉异位引流的发病机制研究进展

完全性肺静脉异位引流（total anomalous pulmonary venous connection，TAPVC）是一类罕见而又严重的先天性心脏病。TAPVC是因肺静脉未能与左心房相连，而直接与右心房或体循环静脉系统连接，导致该类患儿生长发育明显受限，生后全身紫绀及缺氧表现逐渐加重，严重威胁患儿的生命，需尽早手术干预。TAPVC的发病原因和机制尚未完全明确，近年来随着现代分子遗传学的发展，对其的发病机制有了初步的认识，本文就TAPVC发病机制研究进展作一概述。     

完全性肺静脉异位引流诊断中超声心动图的应用

目的探讨超声心动图在完全型肺静脉异位引流（TAPVC）诊断中的应用价值。方法22例TAPVC患儿,均经手术证实诊断,术前均行超声心动图检查,比较超声心动图检查结果与手术诊断TAPVC的符合率。结果手术确诊TAPVC为心上型12例、心内型5例、心下型2例、混合型4例。超声心动图检查诊断TAPVC为中心上型12例、心内型5例、心下型2例、混合型3例。超声心动图检查对TAPVC的正确诊断率为100％,回流途径准确诊断率90．9％（1例超声心动图诊断为心内型TAPVC,共同肺静脉通过冠状静脉窦引流入右房,手术结果为心上合并心内型TAPVC。1例超声提示心上型TAPVC,垂直静脉经无名静脉入上腔静脉,术中见双侧垂直静脉汇入上腔静脉）。结论超声心动图可以正确地诊断和评估TAPVC。     

不同手术方法治疗心上型完全性肺静脉异位引流

目的:比较心上型完全性肺静脉异位引流(TAPVC)的各种手术治疗效果,为临床治疗TAPVC提出更合理的手术方式。方法: 2000年8月~2011年4月外科手术治疗60例心上型TAPVC,包括经房间隔手术14例,经左右心房手术19例,心上法27例。结果: 术后死亡4例,其中经房间隔手术死亡2例,经左右心房死亡1例,心上法死亡1例。术后主要并发症为肺静脉梗阻、低心排、手术创伤所致心律失常、左心发育不良所致急性肺水肿、呼吸衰竭。其中心上法手术患者并发症明显低于其他两组。结论: 心上径路治疗心上型TAPVC近、中期效果良好,远期效果有待进一步随访。     

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。     

完全性肺静脉异位引流基因学研究进展

完全性肺静脉异位引流(Total Anomalous PulmonaryVenous Connecting,TAPVC)是一种罕见的先天性心脏病,其发病机制相当复杂,与遗传因素有密切的关系。近年来,随着分子遗传学技术在先天性心脏病领域的应用与发展,人类对TAPVC临床特征、胚胎发育、遗传学角度的认识有了显著的提高。     

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

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。     

完全性肺静脉异位引流的外科治疗

目的:总结完全性肺静脉异位连接(TAPVC)患者的外科治疗经验,以提高手术疗效。方法:20例患者均在中度低温体外循环下行TAPVC矫治术。心上型12例,心内型7例,混合型1例。合并动脉导管未闭2例,肺动脉瓣狭窄及三房心各1例,三尖瓣关闭不全7例。结果:术后早期(30 d)死亡1例,死于术后严重低心排出量综合征,生存19例均获得随访,随访时间1～5年,心功能恢复良好;术后2周超声示右心房室明显缩小,左心房室扩大。结论:完全性肺静脉畸形引流一经确诊应尽早手术,手术的关键是保证吻合口够大和避免吻合口狭窄及心律失常。心上法可为肺静脉主干与左心房顶部吻合提供良好显露并可降低心律失常发生率,是心上型TAPVC左心房与肺静脉总干吻合良好途径。     

Preoperative balloon dilatation of obstructed total anomalous pulmonary venous connection in a neonate.

Prompt recognition and early surgical correction are mandatory in neonates presenting with obstructed total anomalous pulmonary venous connection (TAPVC). Preoperative balloon angioplasty of obstructed TAPVC in a sick neonate is rarely reported. We describe successful short-term palliation with balloon angioplasty of a critically ill neonate with obstructed supracardiac TAPVC.     

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。     

Palliative stent placement in vertical vein in a 1.4 kg infant with obstructed supracardiac total anomalous pulmonary venous connection

Temporary relief of obstructed total anomalous pulmonary venous connection (TAPVC) may stabilize the preoperative clinical status. We report a 1.4 kg infant with obstructed supracardiac TAPVC to right sided superior vena cava, double outlet right ventricle, pulmonary atresia, major aortopulmonary collateral arteries, and atrioventricular septal defect. A palliative percutaneous stent was successfully placed and resulted in immediate clinical improvement. We performed a review of literature regarding interventional procedures for relief of obstructed TAPVC and found 17 cases including stent placement (13), balloon angioplasty (3), and Hybrid procedure (1). We conclude that temporary stent placement in obstructed TAPVC is feasible and efficacious in relieving pulmonary venous obstruction. The procedure can lead to subsequent surgical repair in a more favorable clinical status and has special significance in the low birth weight infant. © 2012 Wiley Periodicals, Inc.     

255例完全性肺静脉异位引流手术治疗的单中心回顾性研究

目的:分析单中心完全性肺静脉异位引流(TAPVC)手术治疗的早中期效果.方法:连续入组2009年1月至2019年10月首都医科大学附属北京儿童医院心脏外科收治并进行手术治疗的0～3岁TAPVC患儿共255例.按照年龄将患儿分为两组:0～6个月组(A组,n=195)和6个月以上～3岁组(B组,n=60),总结其临床特点,...     

Echocardiographic evaluation of total anomalous pulmonary venous connection: Comparison of obstructed and unobstructed type

This study aims to compare the differences between obstructed and unobstructed total anomalous pulmonary venous connection (TAPVC) using echocardiography, and to evaluate the clinical and echocardiographic parameters associated with pulmonary venous obstruction (PVO).We conducted a retrospective study of 70 patients with TAPVC between 2014 and 2019. The morphologic and hemodynamic echocardiographic parameters of patients were observed and measured, and the parameters between obstructed and unobstructed TAPVC were compared. The clinical and echocardiographic parameter differences between the two groups were used for ROC curve analysis.Obstructed TAPVC was found in 30 (42.9%) of 70 patients. Between obstructed and unobstructed TAPVC, there were significant differences in atrial septal defect size, pulmonary artery maximum velocity (PA V_max ), peak E velocity of mitral valve, left ventricular fractional shortening, left ventricular ejection fraction, stroke volume and the incidence of patent ductus arteriosus, but there was no significant difference in birth weight. The first diagnosis age of obstructed TAPVC was earlier than unobstructed type. The ROC curve analysis for the first diagnosis age showed the sensitivity and specificity were 76.7%, 80% respectively. The ROC curve analysis for the PA V_max showed the sensitivity and specificity were 88.5%, 67.6% respectively.Patients with TAPVC had a high incidence of PVO. The presence of PVO can affect the size of atrial septal defect and the closure of the ductus arteriosus, cause significant changes in PA V_max, peak E velocity of mitral valve, left ventricular fractional shortening, left ventricular ejection fraction, stroke volume, lead to earlier symptoms and earlier first diagnosis age. The first diagnosis age and PA V_max were excellent values since they associated with PVO.     

Total anomalous pulmonary venous return in the fourth decade

Most patients with total abnormal venous connection (TAPVC) have no symptoms at birth, yet the majority die within the first year of life if surgical repair is not implemented. TAPVC is usually suspected in the neonatal period, and without surgical repair prolonged survival is quite exceptional. This kind of abnormality can be a real challenge for a non-pediatrics cardiologist today and can be easily misdiagnosed as a large secundum atrial septal defect if there is no sufficient suspicion of TAPVC. Therefore, a case of a 40-year-old female patient referred to our clinic with increasing shortness of breath is of great interest, as this patient was diagnosed with TAPVC.