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...     

心上型完全性肺静脉异位回流3例

心上型完全性肺静脉异位回流３例赵文增徐敬薛金熔胡伟作者单位：４５００５２河南医科大学第一附属医院胸心外科完全性肺静脉异位回流是少见的、预后较差的先天性紫绀型心脏病。１９８５～１９９３年我院共收治３例，报告如下：临床资料３例病人中男１例，女２例。年龄１...     

右腋下直切口手术治疗先天性心脏病

目的 通过右腋下直切口治疗先天性心脏病可以达到创伤小、疼痛轻、美观的目的。方法 本组４０例患者中,进行单纯型继发孔房间隔缺损修复１８例,房间隔缺损并二尖瓣关闭不全修复３例,室间隔缺损修复１５例,法洛四联症心内台术２例,心内型完全性肺静脉异位引流和部分房室管畸形修复各１例,结果 全组无手术死亡。体外循环时间１８～６６分３０秒,主动脉阻断时间３～５２分,术后６～１２天出院。结论 微创伤切口先天性心脏病     

心上型完全性肺静脉异位引流外科治疗24例临床分析

目的总结心上型完全性肺静脉异位引流的外科治疗经验。方法1995年7月至2004年6月,手术治疗心上型完全性肺静脉异位引流24例,其中男11例,女13例;年龄40d～35岁,其中6周岁以下23例,体重3．8～54．0kg。经右心房、房间隔切开左心房后壁和肺静脉共干吻合10例;横行切开双心房与肺静脉共干吻合8例;2例分别用人造血管补片和自体心包补片将垂直静脉入口和扩大的房间隔切口一并隔人左心房;1例行左心房后壁和肺静脉共干吻合的同时用自体心包补片将肺静脉共干人口和扩大的房间隔切口一并隔入左心房;3例行左心房顶和肺静脉共干吻合。术后呼吸机辅助呼吸,多巴胺、硝普钠辅助循环2～5d。结果手术死亡1例,死于低心排出量综合征;术后心包填塞1例,再次手术止血后治愈;心律失常6例,均治愈;急性肺水肿1例,二次手术重新开放垂直静脉延迟闭合治愈。术后随访3个月至7年,平均2．5年。术后2周严重室上性心律失常1例,治愈;远期因心包补片挛缩致肺静脉严重回流障碍二次手术1例,治愈;其余患者均未见远期并发症。结论心上型完全性肺静脉异位引流可采用不同的手术方法,正确的手术方法和对并发症的及时处理可以获得良好的治疗效果,单纯应用右心房内补片应选用合适的修补材料,左心房顶和肺静脉共干吻合法暴露好、操作简单、心律失常发生率低。     

完全性肺静脉异位连接术后引起心律失常的原因探讨

目的 探讨完全性肺静脉异位连接(TAPVC)术后心律失常的原因和治疗措施。方法 回顾性分析1999年1月～2002年6月TAPVC26例患者的临床资料。心上型16例(61．54％)．心内型9例(34．61％)，混合型1例(3．85％)。结果 术后早期(30d)死亡2例(7.69％)。术后心律失常为室上性心律失常12例(46．15％)，其中窦性心动过缓5例(19.23％)，交界区性心律5例(19．23％)，房性过早搏动1例(3．84％)，Ⅱ度房室传导阻滞1例(3．84％)。心上型完全性肺静脉异位连接矫治术后的心律失常发生率为56．25％(9／16例)。结论 完全性肺静脉异位连接早期手术疗效满意。术后心律失常发生率较高，应及时发现和处理。     

房间隔缺损并部分肺静脉及肝静脉右房异位引流2例

房间隔缺损并部分肺静脉及肝静脉右房异位引流２例舒涛，江泽熙１９８６年３月至１９９３年１０月，我们共施行房间隔缺损及部分肺静脉异位引流根治术７例，其中２例肝静脉直接开口于右房，在深低温停循环下矫治，疗效满意，现介绍如下：例１男，６岁。生后３个月发现心脏...     

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     

左心房内吻合治疗心下型肺静脉异位引流效果分析

目的探讨左心房内吻合术治疗心下型完全性肺静脉异位引流的效果。方法对32例心下型完全性肺静脉异位引流新生儿实施深低温体外循环下左心房内吻合术。结果本组手术顺利,23例患儿术后延迟关胸,无手术死亡病例。5例心脏复跳后心率较慢安装临时心脏起搏器,术后1~5 d心率恢复正常。除2例因低心排综合征于术后早期死亡,其余患儿均治愈出院。3例患儿术后反复出现肺部感染,2例出现气胸,给予抗感染,胸腔闭式引流均匀恢复。1例心脏超声提示发现主动脉弓有缩窄,再次手术解除主动脉弓缩窄,痊愈出院。28例随访3个月~5 a,行心脏超声、胸片检查。患儿心功能良好,吻合口血流速度正常。结论对心下型完全性肺静脉异位引流新生儿实施左房后壁心内吻合手术,吻合口充分,有利于提高术后恢复效果。     

鲁登巴赫综合征合并右肺静脉异位引流1例

鲁登巴赫综合征合并右肺静脉异位引流１例关敬堂，郭好学，林武英，刘书勇，吴志强，罗静病人女，３８岁。唇绀、活动后心慌、胸闷３０余年。查体：轻度唇绀，轻度杵状指。心界扩大，律齐，胸骨左缘第２～４肋间可闻及３级收缩期杂音，心尖可闻及４级舒张期杂音。Ｘ线胸片...     

57例婴幼儿复杂心脏畸形的外科治疗

报告１９９１年９月至１９９４年８月期间收治的５７例婴幼儿复杂先天性心脏病，包括右室双出口１６例、完全性大动脉转位８例、完全性心内膜垫缺损７例、完全性肺静脉畸形引流６例、肺动脉闭锁４例、单心房４例、三尖瓣闭锁和主动脉弓中断各３例、左冠状动脉右室瘘２例、单心室和镰刀综合征各１例及其他复杂畸形２例。６例行姑息性手术，手术早期死亡１例（１６．７％）；５１例行一期矫治术，手术早期死亡９例（１７．６％）。总手术早期死亡为１７．５％，最近２年早期死亡率为１２．５％。主要死亡原因为肾功能衰竭和低心输出量综合征。     

Total anomalous pulmonary venous connection. Long-term results following repair under 3 months of age

The surgical experience with total anomalous pulmonary venous connection (TAPVC) at the University of Louvain (Brussels) between the years 1975 and 1986 is reviewed. Nineteen patients aged two days to three months with TAPVC were studied. The types of TAPVC were supracardiac in 9 patients, cardiac in 4, infracardiac in 4 and mixed in 2. Profound hypothermia induced by surface cooling, limited cardiopulmonary by-pass and total circulatory arrest were used in all cases. The 4 early deaths concerned the first four neonates who were critically ill. All operative survivors are followed for a mean of 3.5 years (12 months to 8 years). There are two late deaths due to reoperation for pulmonary venous obstruction. All 13 survivors are well at last review. Eleven of them have been recatheterized 4 to 33 months after repair (19 months in average). The pulmonary artery and capillary pressures fell to a normal level after a few months. Ventricular function which was markedly depressed preoperatively, was evaluated by quantitative angiocardiography and echocardiography. It returned to normal late postoperatively. The hospital mortality for the repair of TAPVC in the neonates remains appreciable. Total correction at one operation is advisable. The incidence of postoperative pulmonary venous obstruction is of particular concern. The late postoperative functional and hemodynamic results are excellent. The repair of TAPVC can be considered curative.     

Total anomalous of pulmonary venous connection in infants less than 3 months old]

We examined the surgical results of total anomalous pulmonary venous connection (TAPVC) retrospectively in 6 infants, who were less than 3 months old and underwent a total repair at Ehime Prefectural Central Hospital between May, 1993 through May, 1998, in terms of the pre, peri, and postoperative management, the site of connection, and the surgical procedures. Aged at operation ranged from 1 day to 86 days (mean 39 days), and body weight ranged from 2.4 kg to 5.5 kg (mean 3.4 kg). All 6 patients had echocardiographic diagnosis and cardiac catheterization but one. In operative procedure, cut back method was done in a patient of paracardiac type of Darling's classification and posterior approach was used in total correction for 4 supracardiac and 1 infracardiac type. There were 3 hospital deaths who had poor conditions before operation, but no late deaths. Surgical results of TAPVC might have been improved with advances in non-invasive diagnosis by echocardiography, and pre and perioperative management. And we should take care of these patients of TAPVC in long term period to make sure that they have no pulmonary venous obstruction.     

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。     

婴儿期完全性肺静脉畸形引流矫治术近期疗效分析

目的 分析婴儿完全性肺静脉畸形引流矫治术的近期疗效,总结外科治疗经验,分析围手术期死亡的影响因素.方法 2001年1月至2008年7月共有145例1岁以内患儿因完全性肺静脉畸形引流接受矫治术,其中男性94例,女性51例;首次手术的平均年龄为(7±3)个月,平均体质量为(6.3±1.6)kg.肺静脉的引流方式心上型77例(53.1%),心内型47例(32.4%),心下型9例(6.2%),混合型12例(8.3%).术前超声评价21例有明确肺静脉梗阻(心上型12例,心内型3例,心下型3例,混合型3例).结果 全组病例均行根治术治疗(合并复杂畸形不能行双心窜矫治已剔除).围手术期死亡17例,病死率为11.7%.因2006年1月后手术技术和术后策略的综合改进,以此时间点为界分为两组(此前为A组,此后为B组),病死率从A组的19.0%降至B组的6.2%(P=0.020).回归分析显示病死率相关危险因素为组别、是否有肺静脉发育不良致梗阻以及房间隔交通的大小.手术技术以及围手术期治疗策略的综合改进是近3年围手术期终点结果满意的原因.全组无围手术期因肺静脉梗阻再次手术病例.结论 手术技术的改进和围手术期的积极综合治疗可降低完全性肺静脉畸形引流矫治术围手术期病死率.术前肺静脉发育不良致梗阻仍是影响患儿预后的重要因素.     

Outcome of primary repair of infracardiac total anomalous pulmonary venous connection using a right-sided approach to the left atrium

Abstract Objective: Pulmonary venous obstruction (PVO), the major postoperative complication in patients with infracardiac total anomalous venous connection (TAPVC), compromises the surgical outcomes of TAPVC repair. Here, we report our experience using a right‐sided approach to the left atrium to repair this anomaly variant. Method: Eleven patients with infracardiac TAPVC underwent this surgical modification from September 2005 to December 2009. After a medium sternotomy, bicaval venous cannulation was performed for cardiopulmonary bypass (CPB). By adequate exposure of the surgical field, incision of the left atrium was located and anastomosed to the corresponding incision in the pulmonary venous confluence through the right side. Medical records consisting of preoperative and postoperative data were retrospectively reviewed to analyze the efficiency of this strategy. Results: Total correction was achieved in all 11 patients and there were no operative deaths. Postoperative low cardiac output persisted in six patients (54.5%), including five patients with preoperative PVO. No late deaths were noted in a mean follow‐up of 26.7 months (range, 1 to 51 months). Both residual atrial shunt and severe anastomotic obstruction were excluded by transthoracic echocardiography. Moderate residual obstruction was confirmed in one patient. At the end of the follow‐up, all patients had normal biventricular function and were in New York Heart Association (NYHA) Functional Class I. Conclusion: The right‐sided approach for repair of infracardiac TAPVC contributes to produce favorable outcomes. This modified technique is useful for enhancing exposure during surgical repair and providing adequate patent anastomosis. (J Card Surg 2011;26:102‐106)     

Primary sutureless repair for simple total anomalous pulmonary venous connection with a risk of postoperative pulmonary venous stenosis

Late mortality following surgical repair of total anomalous pulmonary venous connection (TAPVC) is often associated with pulmonary venous stenosis. We describe here two successful cases of primary sutureless repair for simple TAPVC in patients who had a potential risk of postoperative pulmonary venous stenosis. A 10-day-old neonate with mixed-type TAPVC and a 30-day-old infant with supracardiac TAPVC underwent primary sutureless repair with our modification. In the early follow-up, both patients are now doing well and have no signs of pulmonary venous stenosis. The sutureless repair can be applied as a primary surgical option to prevent postoperative pulmonary venous stenosis in selective patients with simple TAPVC.     

心上型完全性肺静脉异位引流的单中心治疗结果

目的 分析心上型完全性肺静脉异位引流的手术治疗结果.方法 回顾性分析2014～2019年在本中心行外科手术治疗的98例心上型完全性肺静脉异位引流患者的临床资料,其中男64例、女34例,中位手术年龄3.0(1.5,7.0)个月,中位体重5.0 (4.0,6.0) kg.术前肺静脉梗阻23例(23.5％).传统手术技术治疗...     

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。