新生儿梗阻型完全性肺静脉异位引流的治疗

目的 评估新生儿完全性肺静脉异位引流( TAPVC)不同类型矫治手术方法和预后.方法 1999年至2011年,共收治68例新生儿梗阻型TAPVC急诊手术治疗,平均年龄16天,其中心上型21例,心内型8例,心下型36例和混合型3例.心上型和心下型TAPVC是将肺静脉共汇与左心房后壁作侧侧吻合,心内型TAPVC在心房内将扩大的冠状窦去顶将异位的肺静脉隔入左心房.结果 术后早期死亡2例,占2.9％.随访6个月至3年,经超声心动图随访,肺静脉吻合口均无明显狭窄,血液流速1.10 ～ 1.42 m/s.结论 早期的梗阻型TAPVC的纠治中左心房后壁与肺静脉共汇的侧侧吻合远期效果良好,肺静脉的梗阻情况需要远期进一步随访.     

心内型完全性肺静脉异位引流的类型与外科治疗

心内型完全性肺静脉异位引流(ＴＡＰＶＣ) ,因其开口位置不定 ,类型较多 ,给手术带来困难。我院 1981年 4月～ 2 0 0 0年 4月共收治ＴＡＰＶＣ 11例 ,其中心内型 7例 ,均经手术治愈。1.临床资料 :本组男 4例 ,女 3例 ;年龄 16个月～ 11岁 ,平均 (5 7± 4 5 )岁 ;体重 9 0～ 2 1 0ｋｇ ,平均 (13 9± 4 7)ｋｇ。患者均有反复呼吸道感染、活动后气急、发绀、多汗、易疲劳 ,无蹲踞、缺氧史 ;经皮血氧饱和度 76 %～ 94% ;有杵状指、趾 ;胸骨左缘第 2、3肋间可闻Ⅱ～Ⅲ级收缩期喷射性杂音 ,Ｐ2亢进 ;肝右肋下 1～ 5ｃｍ。ＥＣＧ显示 :右心房右心…     

完全性肺静脉异位引流术后肺静脉狭窄手术干预9例近中期结果北大核心CSCD

目的总结阜外医院过去10年完全性肺静脉异位引流(TAPVC)术后肺静脉狭窄(PVS)患者行再次手术干预的近中期结果。方法纳入2009~2019年于阜外医院完成手术治疗的9例TAPVC术后PVS患者,其中男4例、女5例,手术时年龄(5.10±5.00)岁。根据肺静脉成形手术方式将患者分为2组:无缝线缝合(sutureless)组(n=3)和非无缝线缝合(non-sutureless)组(n=6)。分析患者的临床资料。结果本组患者原发TAPVC类型包括:心上型4例,心内型2例,心下型1例,混合型2例。全组患者中位体外循环时间95(63,208)min,中位主动脉阻断时间58(30,110)min,术后中位ICU滞留时间24(24,2 136)h。早期院内死亡1例(11.1%)。1例(11.1%)合并单心室患者发生住院并发症,术后行血液滤过治疗。随访时间11.9(2.2,18.0)个月,随访期间死亡2例,死因分别为肺静脉再狭窄及脑卒中。Sutureless组与non-sutureless组术后结果及随访结果差异无统计学意义(P>0.05)。结论外科手术是TAPVC术后出现PVS的有效治疗手段,但仍存在较高的并发症发生率及死亡率,sutureless缝合技术在此类患者中应用的优势仍有待验证。     

心脏右侧径路纠治心下型肺静脉异位回流

目的 评估经心脏右侧径路纠治心下型完全性肺静脉异位回流方法的价值.方法 2005年9月至2007年12月,采用经心脏右侧进路方法纠治心下型完全性肺静脉异位回流7例.平均年龄(70.57 -44.67)天;平均体重(4.07±0.87)kg.其中3例肺静脉回流梗阻,2例卵圆孔未闭较小.结果 无手术死亡.术后并发症包括:低心排5例,发生肺动脉高压危象3例,呼吸机辅助7天以上2例,肺部感染2例.术后随访6个月至2年,超声检查心房内无残余分流,吻合口无明显梗阻,流速1.20～1.47m/s;心功能:射血分数0.70～0.79,短轴缩短率0.32～0.44;1例出现肺静脉回流的流速增快,其右上肺静脉2.60m/s,右下肺静脉2.12m/s,继续随访中.结论 采用经心脏右侧径路方法纠治心下型完全性肺静脉异位回流,取得了较好效果.手术成功取决于左房与垂直静脉的吻合口大小,保证肺静脉回流无梗阻.

Abstract：

Objective Infracardiac total anomalous pulmonary venous connection, a rare congenital cardiac defect, is associated with high mortality. A modification was designed for the procedure to reduce the post-operative obstruction in the pulmonary venous. Methods From September 2005 to December 2007, seven patients with infracardiac total anomalous pulmonary venous connection were treated with repair surgery through right side approach. The patients' age at operation was (70.57 ±44.67) days , the weight was (4.07 ±0.87) kg. Three patients had pulmonary venous obstruction, and 2 with small PFO. A modified right - side approach for repairing this defect was used. Results No death occurred after the operation. The postoperative complications included low cardiac output in 5 patients (71.43%), pulmonary hypertension crisis in 3patients ( 42.86% ) , mechanical ventilation for more than 7 days, which happened in 2 patients (28.58%) and pulmonary infection. All of the patients received follow-up. No residual shunt and pulmonary venous return obstruction were identified on echocardiogram(with a velocity from 1.2 m/s to 1.47 m/s). The heart function of patients was within the normal range(EF 0.70 -0.79, FS 0.32 -0.44). Conclusion The modified surgical procedure for the correction of infracardiac total anomalous pulmonary venous connection by right side approach was associated with favorite clinical outcomes, The post-operative outcomes depended on the size of anastomosis between the common vein and left atrium and the patency of the pulmonary venous return. Adequate size of anastomosis and maintenance of the spatial structures in adjacent regions were helpful in decreasing the adverse effect of postoperative obstruction.     

17例心下型完全性肺静脉异位引流的外科治疗

目的 评估新生儿、小婴儿心下型完全性肺静脉异位引流的纠治方法及效果.方法 2000年7月至2009年6月,共纠治心下型完全性肺静脉异位引流17例.病婴出生15天～3.7月龄,其中1月龄以下9例,占52.9%;体重(4.10±0.95)kg,其中5 kg以下15例,占全组88.2%.4例低温低流量灌注下,经右心房切口,切开房间隔,左房与肺静脉吻合;13例深低温停循环灌注,采用心脏上翻法,左心房斜形切口与肺静脉无张力缝合,其中吻合口宽度保证在2.5～4.0 cm.结果 全组手术顺利,无手术死亡.6例术后延迟关胸,5例发生肺高压危象,2例出现暂时性Ⅲ度房室传导阻滞,2例急性肾功能衰竭,4例肺水肿,所有病婴均治愈出院.1例术后反复出现肺部感染,心脏超声发现吻合口流速明显增快(3.05 m/s)、严重肺动脉高压、三尖瓣重度反流,1年后失随访.16例随访1个月～9年,平均(51.8±35.0)个月.随访期均行心脏超声、X线胸片、心电图检查.多数病婴心功能良好,心影较术前明显缩小,肺充血消失,心电图示窦性节律,吻合口血流速度正常.结论 新生儿及婴幼儿心下型完全性肺静脉异位引流病情危重,早期诊断、早期手术效果良好,手术治疗的关键在于左心房与肺静脉吻合口应足够大,避免狭窄的发生.

Abstract：

Objective To evaluate the effectiveness of urgent surgical correction for infra-cardiac total anomalous pulmonary venous drainage (TAPVD) in infants and children. Methods From July 2000 to April 2009, seventeen patients with infra-cardiac type of total anomalous pulmonary venous connection received surgical correction in our department. The age of patients ranged from 15 days to 3.7 months at admission, 9 patients ( 52.9 % ) were younger than 1 month of age. The mean body weight was (4.10 ±0.95) kg and was less than 5 kg in 15 patients (88.2%). Deep hypothermic circulatory arrest (DHCA) without cerebral perfusion was used in 13 patients (76%), and continuous hypothermic low-flow perfusion was used in4 (24%). Incisions on the left atrium and pulmonary veins were sutured without tension, and the width of the anastomosis was 2.5 to 4.0 cm. Results Urgent operations were performed in all patients and no early death occurred during surgery.Mean bypass time was 121.9 minutes ( ranging from 70 to 277 minutes) and mean aortic cross-clamp time was 44 minutes ( ranging from 30 to 74 minutes). 6 neonates had delayed wound closure after surgery and 4 had obvious pulmonary edema. Inhalation of nitric oxide was given to 5 cases for the management of significant postoperative pulmonary hypertension, while peritoneal dialysis was performed in 2 cases with temporary renal failure. Transient atrioventricular block of grade 3 occurred in 2patients and acute renal failure occurred in 2 cases. All patients were uneventfully discharged. The mean ventilation time was 45 h (ranging from 12 to 430 h). Mean duration in ICUs and hospitals were 15 days ( ranging from 4 to 40 days) and 22. 5days ( ranging from 10 to 42 days), respectively. During the period of follow up, one patient presented with pulmonary venous obstruction and recurrent pulmonary infection with increased flow speed at the site of anastomosis, pulmonary hypertension and tricuspid regurgitation on echocardiogram. No improvement was observed in the patient spite of medical treatment and he was lost during follow up one year after operation. 16 patients received postoperative follow up for 1 month to 9 years, mean (51.8 ± 35.0) months. Echocardiography, chest radiography, and ECG were performed during this period. As a result, most children had good cardiac function, with sinus rhythm on ECG and apparently reduced pulmonary congestion on radiography.No obstructive pulmonary venous return was observed on echocardiography. Most patients had good cardiac functions. Conclusion Corrective operation for infra-cardiac TAPVC on urgent basis may provide favorite outcomes. The prognosis is associated with the size of anastomosis between the pulmonary vein and left atrium, as large anastomosis may prevent the obstruction of pulmonary venous retum.     

混合型完全性肺静脉异位引流的外科治疗

目的总结手术治疗混合型完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的经验。方法 2006~2018年,我院共完成51例混合型TAVPC患者(排除合并单心室、法洛四联症等患者)的外科治疗,其中男35例、女16例,中位年龄102.0(59.0,181.0)d,中位体重5.0(4.1,6.4)kg。根据解剖形态将患者分为3类:3+1型(38例,3支肺静脉回流入同一个部位,而另一支肺静脉回流另一侧部位);2+2型(9例,两侧的肺静脉分别回流入不同的位置);怪异型(4例,无法归入以上两类的怪异解剖类型)。结果无患者院内死亡,中位随访时间41.0(18.0,86.5)个月。术后发生肺静脉梗阻10例。Kaplan-Meier生存曲线示3种类型术后肺静脉梗阻率差异无统计学意义(P=0.239)。Cox分析发现术前肺静脉梗阻与术后肺静脉梗阻明显相关(P=0.024)。结论混合型TAPVC解剖形态多变,需要个体化手术方法。     

完全性肺静脉异位引流术后肺静脉梗阻的随访和再干预

目的:总结完全型肺静脉异位引流(TAPVC)术后肺静脉梗阻(PVO)的随访和再干预结果。方法:回顾2013年10月至2019年10月174例单纯TAPVC患儿资料,总结术后发生PVO患儿的围术期和随访期间症状、生长发育等临床表现,分析超声心动图、CT心血管造影检查等资料。对符合干预指征的采取内外科镶嵌、原位心包无内膜接...     

右房异构单心室伴完全性肺静脉异位引流的外科治疗

目的 介绍右房异构、单心室伴完全性肺静脉异位引流（TAPVC）的外科治疗经验和TAPVC在右房异构纠治手术中意义。方法 1999年6月于2000年3月手术治疗右房异构、单心室伴TAPVC5例。其中4例为心上型,1例为混合型。所有病儿均施行一侧或双侧的双向腔肺血管吻合术（BCPC）,4例同时进行TAPVC纠治术。结果 手术死亡1例,原因为 诊断不明确、体外循环时间过长、术后严重低心排和低氧血症。生存4例术后均无残余解剖梗阻,术后血流动力学稳定,血氧饱和度明显增高,随访结果满意。结论 术前明确TAPVC的诊断对指导手术具有重要意义;右房异构、单心室伴TAPVC在进行分流术时应同时施行TAPVC纠治术。     

完全性肺静脉异位引流的手术治疗经验

为评估影响完全性肺静脉异位引流（ＴＡＰＶＤ）手术纠治的因素，本组纳入了２８例在中度低温体外循环和１５例在深低温停循环下手术纠治者。结果手术死亡４例，死亡率９．３％。随访３２例，２例肺静脉回流梗阻分别于术后５个月和２年３个月死亡。结论认为，ＴＡＰＶＤ必须早期手术防止肺血管阻塞性病变；术后定期随访；改进手术方法，防止心房内补片粘连所致肺静脉回流梗阻。     

混合型完全性肺静脉异位引流1例

病儿　女 ,10岁。出生后即口唇发绀、发现心脏杂音 ,反复肺部感染。查体 :血压双上肢 70 5 0ｍｍＨｇ(1ｍｍＨｇ =0 133ｋＰａ) ,双下肢 10 0 6 0ｍｍＨｇ。口唇轻度发绀 ,轻度杵状指 ,胸骨左缘第 2、3肋间可闻及 3 ＶＩ级吹风样收缩期杂音 ,Ｐ2 亢进。心电图 :电轴右偏 114° ,不完全右束支传导阻滞 ,右室肥厚。Ｘ线胸片示心脏增大 ,心胸比率 0 70 ,主动脉结小 ,肺动脉段突出。多次彩色超声心动图示继发孔房间隔缺损 (房缺 ) ,右向左分流 ,诊断法洛三联症 ,不除外肺静脉异位引流。右心导管造影检查见肺动脉、肺静脉、右房、右室顺序显…     

应用无内膜接触缝合技术治疗心上型全肺静脉异位引流

目的 探讨无内膜接触缝合技术应用于初次心上型全肺静脉异位引流(TAPVC)的外科矫治,以顶防术后肺静脉梗阻发生的可行性.方法 自2007年12月至2008年12月,25例TAPVC病儿接受体外循环下畸形矫治手术,其中心上型TAPVC 9例手术中5例采用无内膜接触缝合技术.男2例,女3例;年龄为2个月～13岁;体重为4.5～21.0 kg,平均(7.9±6.4)kg.手术选择心包斜窦入路,沿共同静脉长轴横向剖开,并将此切口上延至垂直静脉的心包返折处,使用7-0 PDS缝线将左心房后壁切口与共同静脉切口周边的心包组织吻合,通过"控制性出血技术"将肺静脉回流的血液引流进入左心系统;部分结扎垂直静脉.结果 5例采用无内膜接触缝合技术进行外科矫治的病儿全部生存.1例病儿因术前并发双侧肺实变,术后机械辅助呼吸超过7天其他病儿均顺利康复,无并发症.术后行心脏超声随访,随访1～13个月,中位数为7个月,常规随访时间点分别设在出院前、术后3个月、半年及1年.至目前为止,5例病儿肺静脉回流通畅,血流速度0.65～0.85 m/s.结论 在治疗心上型全肺静脉异位引流方面,无内膜接触缝合是一项切实可行、易于操作的外科技术,可以预防术后早期肺静脉梗阻的发生;但其大宗病例的远期疗效,尚有待进一步的研究和探索.     

完全型肺静脉异位引流的诊断和外科矫正

目的总结完全型肺静脉异位引流(TAPVD)的诊断和外科治疗经验.方法回顾近16年收治TAPVD17例,其中男10例,女7例;年龄2～37岁.心上型11例,心内型6例.心上型者经右、左心房联合切口矫正,心内型者切除肺静脉共干与左心房之间的组织,补片修补房缺.同时矫正合并畸形.结果超声检查误诊4例,其中3例为心内型,外院曾误诊为纵隔肿瘤1例.造影检查除3例心内型者均明确诊断.2例术中始发现合并的动脉导管未闭(PDA),1例心上型者再次手术矫正.1例术后死于呼吸衰竭.16例随访1.5～17.5年,心功能良好.结论超声心动检查是诊断本病的主要手段,但有时需行心血管造影,甚至经术中探查方可确诊.本病确诊后应尽早手术,术中应常规探查和处理PDA,及早切开肺静脉共干引流,同时完全矫正心内畸形.手术成功率较高,远期效果良好.     

心上法矫治小儿完全性心上型肺静脉畸形引流(附11例报告)

目的介绍心上法矫治心上型完全性肺静脉畸形引流(TAPVR).方法 1998年6月至2001年8月,采用心上法矫治心上型TAPVR 11例,其中男7例,女4例;年龄5月龄～15岁,平均(5.33±4.98)岁;体重6.4～33.0kg,平均(15.09±8.78)kg.在全麻、低温体外循环下行矫治术,正中切口纵劈胸骨入胸,经横窦,上腔静脉与升主动脉之间显露共同肺静脉干及左心房,将其侧侧吻合.结果术后早期(30 d)无死亡,随访4～36个月,无远期死亡及心律失常发生.结论心上法矫治心上型TAPVR可获得良好的显露和足够大的吻合口,手术对心脏的损伤小,可降低术后心律失常的发生率.     

38例完全性肺静脉异位引流的外科治疗

目的：总结３８例完全性肺静脉异位引流的外科治疗经验。资料和方法：３８例中男１７例,妇女１例。年龄２个月￣２２岁,３岁以内婴幼儿１２例。体重４．５￣３６．０ｋｇ。心上型２０例、心内型１５例、混合型３例。全组均在中度低温体外循环下将完全性异位引流的肺静脉直接或通过房间隔缺损隔入左房。结果：手术死亡３例（７．９％）,其中２例术后严重心律失常、１例多次缝合止血致吻合口狭小。术后并发心律失常１２例、呼吸道感     

Histopathology of anastomotic stenosis after total anomalous pulmonary vein connection

We report a case of a 10-month-old girl who was diagnosed with pulmonary vein stenosis after total anomalous pulmonary vein connection repair and underwent release of an anastomotic stenosis. Histopathological examinations of the resected anastomotic tissue revealed intimal hyperplasia at the anastomotic site. Predominant lesion cells were identified as myofibroblasts and had the characteristics of fibroblasts and synthetic smooth muscle cells. These cells could be a useful target for preventing anastomotic stenosis after total anomalous pulmonary vein connection repair.