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外科治疗的进展

完全性肺静脉异位引流外科治疗的进展马旺扣综述汪曾炜审校完全性肺静脉异位引流（ＴＡＰＶＣ）是一种罕见的先天性心脏病，约占“先心病”发病率的１．５％～３．０％，其中７５％～８０％病儿在１岁内死亡［１，２］。因此，必须及早手术治疗。解剖类型Ｅｄｍｕｎｄｓ统...     

外科治疗完全性肺静脉异位连接31例

目的 总结31例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 31例患者均在中度低温体外循环下行TAPVC矫治术.其中心上型16例,心内型13例,混合型2例.所有患者均合并继发孔型房间隔缺损,合并动脉导管未闭4例,肺动脉瓣狭窄1例,二尖瓣关闭不全1例,三尖瓣关闭不全15例.结果术后无早期(30 d)死亡,发生并发症8例(25.8%),其中阵发性结性心律1例,Ⅰ度房室传导阻滞1例,频发性房性早搏、短阵性房性心动过速1例;肺部感染2例,肺不张1例,气胸1例,左侧膈肌麻痹并肺部感染二次插管1例,均经治疗出院.本组31例均获得随访,随访时间2.8±1.5年.随访期间1例心上型患者于术后8个月死于心力衰竭,生存的30例患者中28例(93.3%)手术效果良好,心功能基本正常,生活工作无明显受限;治疗效果较差2例,为反复发作房性心律失常,经内科治疗无效.结论 TAPVC手术方式的选择依据TAPVC畸形的特点而不同,手术的关键是完全矫治,避免出现远期肺静脉梗阻和心律失常,其治疗效果满意.     

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     

完全性肺静脉异位引流的外科治疗及疗效

１９８８年１１月至１９９５年１２月外科治疗１１例完全性肺静脉异位引流（ＴＡＰＶＣ）病人，其中心上型７例，心内型４例。术后死亡１例。术后１０例生存者心功能较术前明显恢复，无远期吻合口狭窄和肺静脉梗阻发生。ＵＣＧ检查发现右房、右室内径较术前明显缩小（Ｐ≤０．０００１），左室舒张末期内径明显增大（Ｐ＜０．００２），心脏指数和射血分数较术前明显提高（Ｐ＜０．０００１）；左房内径略有增大但无统计学意义，共同肺静脉干术后无明显变化。结论：ＴＡＰＶＣ外科治疗成功的关键取决于术前左心室发育及其顺应性；手术吻合口通畅和无远期吻合口狭窄或肺静脉梗阻     

心上型完全性肺静脉异位引流手术132例的治疗效果分析

目的总结无内膜接触缝合（Sutureless）技术应用于矫治心上型完全性肺静脉异位引流（TAPVC）的临床疗效。方法2007年7月至2013年12月在广东省心血管病研究所行外科矫治术的所有心上型TAPVC婴幼儿共132例,排除合并单心室、心房异构等患儿,均行双心室根治性手术。全部患儿均行超声心动图检查,绝大部分患儿行CT检查了解肺静脉发育情况,进行术前评估,并术中探查确诊。按不同的手术方式分为传统手术组和Sutureless手术组。其中传统手术组69例,男54例（78．3％）、女15例（21．7％）;中位年龄60（30,225）d;中位体重4．85（3．50,6．35）kg。Sutureless手术组63例,男48例（76．2％）、女15例（23．8％）,中位年龄90（30,210）d;中位体重4．58（3．72,6．20）蝇。随访至2014年1月1日。结果Sutureless手术组住院死亡率（4．8％VS．7．2％,x^2=1．414,P=-0．720）、术后总死亡率（4．8％VS．13．0％,x^2=2．733,P=-0．098）均低于传统手术组,但差异无统计学意义。Sutureless手术组术后梗阻率低于传统手术组（1．6％VS．10．1％,x^2=4．236,P=-0．040）,差异有统计学意义。COX多因素分析发现,传统手术方式、术前肺静脉梗阻与术后肺静脉梗阻的发生显著相关（P=0．023,P=0．016）。传统手术方式与术后死亡无明显相关性（P=0．060）。结论对于心上型TAPVC,Sutureless技术可明显降低术后肺静脉梗阻发生率。     

新生儿完全性肺静脉异位连接手术治疗结果

目的:分析预测新生儿完全性肺静脉异位连接(total anomalous pulmonary venous connection,TAPVC)矫治术预后相关危险因素。方法:回顾性分析2009年1月至2018年1月收治的105例接受TAPVC手术治疗的新生儿。心上型42例(40%,42/105)、心内型21例(20%,2...     

完全性肺静脉异位连接的外科治疗

目的　总结 18例完全性肺静脉异位连接的外科治疗经验。　方法　全组均在全身麻醉中度低温体外循环下进行手术 ,11例心上型患者除早期 1例经后径法矫治外 ,其他 10例经右心房切口径路矫治 ;7例心内型采用自体心包片将冠状静脉窦口经扩大的房间隔缺损隔入左心房 ,后 5例用 5 - 0 Prolene线连续缝合房间隔粗糙面后再矫治。结果　无手术死亡 ,2例心内型患者术后 6个月和 8个月再次手术 ,发生心律失常 7例 ,一过性肺水肿 2例 ,经治疗均痊愈。 16例随访 4个月～ 5年 ,心功能正常。　结论　提高手术成功率和远期疗效的关键为 :术中吻合口要足够大 ,术后及时处理心律失常、肺水肿和低心排血量 ,心内型矫治时消除房间隔粗糙面可防止术后肺静脉梗阻。     

混合型完全性肺静脉异位引流1例

病儿　男 ,14个月。查体 :中央性发绀 ,经皮氧饱和度为0 85 ,胸骨左缘第 2、3肋间闻及 1/ＶＩ级收缩期杂音 ,Ｐ2 亢进 ,心功能Ｉ级。心电图示双室大 ,右室为主。Ｘ线胸片示肺淤血 ,心影增大 ,右上纵隔增宽。多普勒超声心动图示多发性房间隔缺损 ,一处近上腔静脉 ,直径 10ｍｍ     

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。     

A surgical technique for correction of total anomalous pulmonary venous drainage

A technique was employed successfully for correction of total anomalous pulmonary venous drainage into the upper right superior vena cava. A J-shaped right atriotomy was performed; the posterior flap was sutured to the anterior border of a previously enlarged atrial septal defect. The right superior vena cava was divided above the site of drainage of the pulmonary veins, and its proximal opening closed with a suture. The pulmonary venous return was directed to the left atrium in this way. The right atrial-right superior vena caval continuity was then reestablished by an anastomosis between the previously opened right atrial appendage and the distal end of the right superior vena cava. Finally the remaining atriotomy was closed. The azygos vein must be ligated to avoid systemic unsaturation. For correction of anomalous pulmonary venous drainage into the azygos vein with this technique, ligature of the azygos vein must be placed distally to the site of anomalous drainage. Three patients, aged 2 months, 7 years, and 16 years, respectively, with different anatomic types of the anomaly, were successfully operated on with this procedure. Findings displayed from the postoperative hemodynamic, echocardiographic, and clinical evaluation are encouraging, after a follow-up period that ranges from 4 months to 4 years. The advantages of the repair are discussed.     

应用无内膜接触缝合技术治疗心上型全肺静脉异位引流

Objective Retrospectively analyze surgical correction supracardiac total anomalous pulmonary venous con-nection (TAPVC) using sutureless technique to prevent post-repair pulmonary vein stenosis. Methods Between December 2007 and December 2008, 25 children cases of total anomalous pulmonary venous connection underwent primary surgical cor-rection. The anatomic types of TAPVC were supra cardiac in nine patients, inha cardiac in one, mixed in 3 and intra cardiac in 12. Five of nine supra cardiac TAPVC underwent correction using sutureless technique. There were 2 male and 3 female. Their age at surgeries ranged from 2 months to 13 years, and the body weight were from 4.5 kg to 2.1 kg with an average of (7.9±6.4) kg. After median sternotomy and opening the pericardium, the heart was arrested by delivering cold crystal ear-dioplegia. The heart was then positioned toward the patient' s right and under the right henri sternum. A generous incision across the posterior wall of the left atrium and one on the common pulmonary vein was made. The latter was extended upwards to the midpoint of the vertical vein. The left atrium was subsequently connected to the pulmonary venous confluence by suturing the edge of the atrium to the posterior mediastinal pericardium that surrounding the common pulmonary vein and the vertical vein with 7-0 PDS. The vertical vein was partially ligated after conclusion of CPB, leaving a diameter of 5mm shunt. Routine follow-up with echocardiogram were at diacharging, 3 months, 6 months and 1 year after surgery. Results All 5 cases survived uneventfully excopt 1 baby on ventilation over 7 days after surgery, who had bilateral lung consolidation before the operation. Echocardiogram showed satisfactory results with maxium velocity acrossing the anastomosis of 0.65 -0.85 m/s. Conclusion Sutureleas technique can avoid trauma to the pulmonary venous endothelium and minimize the tension of anastomasis. It may play an important role to prevent post-repair pulmonary vein stenosis. More patients with long-term follow-up are necessary to draw a definite conclusion of this technique.     

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.     

应用无内膜接触缝合技术治疗心上型全肺静脉异位引流

Objective Retrospectively analyze surgical correction supracardiac total anomalous pulmonary venous con-nection (TAPVC) using sutureless technique to prevent post-repair pulmonary vein stenosis. Methods Between December 2007 and December 2008, 25 children cases of total anomalous pulmonary venous connection underwent primary surgical cor-rection. The anatomic types of TAPVC were supra cardiac in nine patients, inha cardiac in one, mixed in 3 and intra cardiac in 12. Five of nine supra cardiac TAPVC underwent correction using sutureless technique. There were 2 male and 3 female. Their age at surgeries ranged from 2 months to 13 years, and the body weight were from 4.5 kg to 2.1 kg with an average of (7.9±6.4) kg. After median sternotomy and opening the pericardium, the heart was arrested by delivering cold crystal ear-dioplegia. The heart was then positioned toward the patient' s right and under the right henri sternum. A generous incision across the posterior wall of the left atrium and one on the common pulmonary vein was made. The latter was extended upwards to the midpoint of the vertical vein. The left atrium was subsequently connected to the pulmonary venous confluence by suturing the edge of the atrium to the posterior mediastinal pericardium that surrounding the common pulmonary vein and the vertical vein with 7-0 PDS. The vertical vein was partially ligated after conclusion of CPB, leaving a diameter of 5mm shunt. Routine follow-up with echocardiogram were at diacharging, 3 months, 6 months and 1 year after surgery. Results All 5 cases survived uneventfully excopt 1 baby on ventilation over 7 days after surgery, who had bilateral lung consolidation before the operation. Echocardiogram showed satisfactory results with maxium velocity acrossing the anastomosis of 0.65 -0.85 m/s. Conclusion Sutureleas technique can avoid trauma to the pulmonary venous endothelium and minimize the tension of anastomasis. It may play an important role to prevent post-repair pulmonary vein stenosis. More patients with long-term follow-up are necessary to draw a definite conclusion of this technique.