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。     

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。     

完全性肺静脉异位引流的外科治疗体会(附16例报告)

完全性肺静脉异位引流 ( TAPVC)为一种少见的先天性心脏畸型 ,发病率占先天性心脏病的 1 %～3% ,自然病程预后极差 ,其中 5 0 %于生后 3个月内死亡 ,80 %于 1岁以内死亡 ,存活至 1岁以上的患儿常合并较大的房间隔缺损。 1 995年 6月至 2 0 0 2年 6月 ,我院共实施 TAPVC矫正术 1 6例 ,取得较好的效果。现总结报告如下。1　资料与方法1 .1　临床资料　本组 TAPVC患儿 1 6例 ,男 7例 ,女 9例 ;年龄 3个月至 6岁 ,平均 1 .2岁。均经心脏超声和多普勒检查明确诊断 ,1例行心导管检查和心血管造影。按 Darling分类法均为心上型 ,均合并房间…     

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,...     

1岁以内完全性肺静脉异位引流外科治疗86例疗效分析

目的:回顾性总结婴儿完全性肺静脉异位引流(TAPVC)外科治疗经验及其效果分析,提高手术疗效。方法:回顾分析河北省儿童医院2012年1月至2015年12月TAPVC患儿86例的临床资料,其中男性66例,女性20例,年龄5d~12个月,平均5.11个月;体质量2.3~9 kg,平均(5.67±2.08)kg,均经心脏彩超、胸部X线片、心电图检查确诊,矫治手术均在常规体外循环中低温(28℃左右)下完成。结果:当时住院死亡4例,术后肺静脉狭窄再手术死亡1例,总病死率5.81%。术中心力衰竭死亡2例,严重感染、多器官衰竭1例,低心排出量综合征2例。81例存活者术后复查结果满意,出院后随访3~36个月,身体发育、活动能力正常,无二次手术者。结论:完全性肺静脉异位引流是一种危重先天性心脏病,尤其伴梗阻性的需急症手术治疗,手术矫治可以明显改善患儿的生存质量。     

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外科治疗

目的 :探讨完全性肺静脉异位引流 （TAPVD）的手术方法 ,评价其效果。方法 :本组患者 2 9例 ,均在体外循环下行矫治术。心上型采用心房横断法 ,应用补片扩大左房 ;心内型及混合型采用扩大房间隔缺损 ,用大补片将异位引流的肺静脉隔至左房 ;混合型上腔静脉用心包补片加宽。结果 :全组无手术死亡 ,出院时心功能 1级。随访 （2 2 /2 9例 ） 3～ 15年 ,心功能均正常。结论 :按 TAPVD的类型采用不同的手术方法。对 TAPVD的外科治疗只要诊断明确、手术方法得当、术后严密监护可获良好的治疗效果     

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。     

单心室合并完全性肺静脉异位引流的外科治疗

目的:探讨单心室合并完全性肺静脉异位引流(TAPVC)的外科治疗效果。方法:2009-05至2011-09,我科共收治单心室合并TAPVC患儿11例,年龄(30.1±21.4)个月,体重(11.0±3.2)kg。例2行改良B-T分流术,例1、3～6、8、9、11行一侧或双侧双向腔肺分流术,例7、10行一期改良Fontan术。例1、6～9、11同期行TAPVC矫治术。结果:例6、10围术期死亡(18.2%,2/11),死亡原因分别为中枢神经系统感染和多器官功能衰竭。存活的患儿随访1～29个月,例4死亡,余患儿氧饱和度改善,未发现残余肺静脉梗阻,例5行二期改良Fontan术并顺利出院。结论:单心室合并TAPVC患儿仍具有较高早期死亡率,术前明确诊断TAPVC并制定合理的个体化治疗方案是提高手术疗效的关键。     

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。     

Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is an uncommon congenital anomaly in which the anatomical presentations vary widely among patients. We hereby present two newborns with TAPVC associated with asplenia syndrome; both had severe esophageal varices due to infradiaphragmatic pulmonary venous drainage. Ultrafast computed tomography (CT) scanning was superior to color Doppler echocardiography and cardiac catheterization as it provided a detailed portrait of the pulmonary drainage. The remarkable radiographic manifestations are presented.     

Partial anomalous pulmonary venous connection and pulmonary arterial hypertension

Background and objective: Isolated partial anomalous pulmonary venous connection (PAPVC) has been implicated as a cause of pulmonary arterial hypertension (PAH); however this condition is often overlooked in the diagnostic work up of patients with PH. We studied the prevalence of PAH both in patients with isolated PAPVC or associated with other congenital heart diseases (CHD) such as atrial septal defect (ASD). We also aimed to identify factors related to the presence of PAH in these patients. Methods: We retrospectively analyzed data from the Adult CHD database at the Cleveland Clinic, USA between October 2005–2010. We included all patients diagnosed with PAPVC with or without other CHD. We excluded all patients with previous corrective surgeries. Results: We identified 14 (2.5%) patients with PAPVC. Group I included patients with PAPVC (with or without patent foramen ovale (PFO)). Group II included patients with PAPVC associated with other CHD. PAH was seen in six (6/14, 42.8%) patients, two (2/7, 28.5%) in group I and four (4/7, 57.1%) in group II (P = 0.3). The mean pulmonary artery pressure in all patients (n = 14) was 29.5 ± 13.8 mm Hg. Group I had a mean PAP of 23.6 ± 6.6 mm Hg as compared to 33.7 ± 16.5 mm Hg for group II (P = 0.34). The two patients in group I with PAH had either two anomalous pulmonary veins or a condition (sickle cell disease) that could potentially explain the haemodynamic findings. Conclusions: Patients with PAPVC (with or without PFO) in the absence of other CHD had normal pulmonary arterial pressure (PAP) unless they have two pulmonary veins with anomalous return or associated conditions known to cause PAH.     

改良左心房上吻合法矫治心上型完全性肺静脉异常回流

目的总结从左心房顶部行改良左心房-共同静脉吻合(左心房上吻合法)治疗心上型完全性肺静脉异常回流(total anomalous pulmonary venous connection,TAPVC)的经验。方法 2005年1月至2011年8月高州市人民医院收治心上型TAPVC 17例,17例肺静脉均是经垂直静脉、无名静脉、右上腔静脉后回流至右心房,其中男7例,女10例,年龄中位数为1岁(40 d～24岁);体质量中位数为12.5 kg(3.1～31.0 kg),全组12例合并房间隔缺损,3例合并卵圆孔未闭,2例合并房间隔缺损及动脉导管未闭,均采用左心房上吻合法。结果手术死亡2例,1例死于低心排血量综合征;1例术后反复肺感染,死于呼吸衰竭。1例因术后急性肺水肿,二次手术重新开放垂直静脉延迟闭合治愈;2例心律失常(频发房性期前收缩),治疗一周恢复正常。无肺动脉高压危象。术后随访3个月至6年,平均3.5年,无吻合口狭窄和心律失常,发绀消失,活动耐力显著增加,生长发育良好。结论改良左心房上吻合法治疗心上型TAPVC,手术暴露好、操作简单、心律失常发生率低,术后中期随访结果良好。     

Stent implantation as a stabilization technique in supracardiac total anomalous pulmonary venous connection.

A 6-week-old male presented in-extremis with obstructed supra-cardiac total anomalous pulmonary venous connection. Balloon dilation and stent implantation in the stenotic segment of the vertical vein relieved the obstruction until the patient was stable enough to undergo surgical repair. An unusual form of extrinsic vertical vein compression was found at surgery.     

The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus

BACKGROUND—Infants with isolated totally anomalous pulmonary venous return often present severely decompensated, such that they are at high risk for surgical repair. On the other hand, if surgical repair can be safely accomplished, the outlook is usually good. Thus prenatal diagnosis would be expected to improve the prognosis for the affected child.
OBJECTIVE—To describe the features of isolated totally anomalous pulmonary venous drainage in the fetus.
DESIGN—Four fetuses with isolated totally anomalous pulmonary venous connection were identified and the echocardiographic images reviewed. Measurements of the atrial and ventricular chambers and both great arteries were made and compared with normal values.
SETTING—Referral centre for fetal echocardiography.
RESULTS—There were two cases of drainage to the coronary sinus, one to the right superior vena cava, and one to the inferior vena cava. Right heart dilatation relative to left heart structures was a feature of two cases early on, and became evident in some ratios late in pregnancy in the remaining two.
CONCLUSIONS—Ventricular and great arterial disproportion in the fetus can indicate a diagnosis of totally anomalous pulmonary venous connection above the diaphragm. However, in the presence of an atrial septal defect or with infradiaphragmatic drainage, right heart dilatation may not occur until late in pregnancy. The diagnosis of totally anomalous pulmonary venous drainage in fetal life can only be reliably excluded by direct examination of pulmonary venous blood flow entering the left atrium on colour or pulsed flow mapping.


Keywords: congenital heart disease; fetus; total anomalous pulmonary venous drainage; echocardiography     

Evaluation of total anomalous pulmonary venous drainage with cross-sectional colour-flow Doppler echocardiography

Three patients with total anomalous pulmonary venous drainage(TAPVD) were studied by real-time cross-sectional colour-flowDoppler echocardiography. Serial suprasternal, parasternal andsubcostal scans were obtained. In all cases surgical or angiocardiographicconfirmation was available. Two patients had supracardiac drainage(to the left vertical vein or to the right superior vena cava)and one patient had infracardiac drainage. An abnormal forwardflow in the left innominate vein and vertical vein was visualizedin those patients with supracardiac TAPVD. Abnormal venous flowwas also imaged in one patient with mixed drainage. In the patientwith infradiaphragmatic TAPVD characteristic flow signals wereidentified in the inferior vena cava (retrograde flow) and inthe descending aorta and anomalous pulmonary venous channel(forward flow). In all patients the patterns of pulmonary venousflow allowed us to distinguish TAPVD from contiguous structuresand to validate two-dimensional cross-sectional imaging.     

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。