完全型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例心内型者均明显诊断。2例术中始发现合并的动脉导管未闭（PDA）,1例心上型是再次手术矫正。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。手术成功率高,远期效果良好。     

全肺静脉异位引流心内修复术后护理

全肺静脉异位引流,是指左、右肺静脉未能直接与左心房相连,而与右心房或体静脉相连接。致使全肺静脉的氧合血,直接流入右心房。其发病率占全部先天性心血管畸形的1～4％。我院自1975年9月～1986年9月,对20例全肺静脉异位引流患者,进行了手术治疗。术后护理得法、无一例死亡。效果满意。一、临床费料全肺静脉异位引流心内修复术20例。其中男性6例、女性14例。年龄4～23岁;心上型13例、心内型7例。有紫绀症状者17例。二、护理要点 (一) 呼吸道的观察与护理全肺静脉异位引流病人,术前因肺静脉氧合血直接或间接的流到右心房,X线照片均示肺静脉充血,加之心上型和心下型手术需将肺静脉总干与左心房作一大的侧侧吻合,并闭合房间隔缺损和结扎垂直静脉。心内型需用人工补     

肺静脉畸形引流的外科治疗

目的探讨肺静脉异位引流（APVC）的外科治疗经验。方法全组患者共10例，其中完全型3例（心上型），部分型7例（4例引流至右心房，3例引流至上腔静脉），男6例，女4例，全组患者均在中度低温体外循环心内直视下采用右心房切口，通过吻合和补片法将异位的肺静脉隔人左心房，同时纠正合并的心内畸形。结果手术死亡1例（10％），其余9例患者均痊愈出院。结论本病确诊后应尽早手术，从而防止肺血管阻塞性病变的进展，手术成功的关键是肺静脉回流是否通畅，随着手术技术的完善以及术后监护水平的提高，肺静脉异位引流的外科治疗可以取得满意的疗效。     

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.     

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.     

电子束CT血管造影及三维重建在肺静脉畸形引流临床诊断中的应用

目的 评价电子束CT血管造影及三维重建在肺静脉畸形引流临床诊断中的作用。方法对20例电子束CT诊断的肺静脉畸形引流病例进行回顾性分析。电子束CT检查采用增强单层容积扫描，层厚1.5-3.0mm，扫描时间100ms。所有病例均行三维重建。20例均行超声心动图检查，有10例尚行心血管造影检查。14例有手术资料对照。结果 在14例有手术对照的病例中，电子束CT对肺静脉畸形引流的定性及定位诊断符合13例，部分符合1例；超声心动图仅诊断2例，手术对照，仅部分符合；怀疑8例，心血管造影6例中诊断4例，与手术对照完全符合，怀疑2例。未行手术治疗的6例中，电子束CT均可见肺静脉畸形引流，血管造影4例中仅有2例发现，超声心动图则均未诊断。结论 电子束CT血管造影及三维重建在肺静脉畸形引流的临床诊断中具有明显优势，是肺静脉畸形引流的有效、无创检查方法。     

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。     

心上路径矫治新生儿期完全性肺静脉异位引流

目的总结新生儿期心上型完全性肺静脉异位引流(TAPVD)的手术治疗经验,推广心上法在TAPVD手术治疗中的应用。方法回顾性分析2008年8月至2013年8月上海儿童医学中心收治新生儿期心上型梗阻型TAPVD患儿7例的临床资料,其中男5例、女2例,年龄8~28(18.3±7.7)d,体重3.1~4.5(3.57±0.47)kg。全部患儿在全身麻醉低温体外循环下行心上法矫治术。结果 7例患儿术后早期(1个月内)无死亡。随访3个月至4年,吻合口均保持通畅,无明显肺静脉回流梗阻,中期随访无心律失常及死亡发生。结论心上法矫治新生儿期心上型TAPVD可获得较大吻合口,减少心房内操作,有效降低术后死亡率、心律失常及吻合口再狭窄率。     

完全性肺静脉异位引流的外科治疗

目的 报道完全性肺静脉异位引流(total anomalous pulmonary venous drainage)的外科治疗疗效和体会. 方法 27例患者中(心上型19例、心内型8例),18例采用全身麻醉体外循环心脏停搏下手术,9例采用心脏不停跳手术.心上型横切右心房和左心房后壁,纵行切开肺总静脉,切口3.5～5.0 cm,与左心房后壁吻合,用自体心包片修补房间隔缺损(ASD),停机后结扎垂直静脉;心内型切开ASD与冠状静脉窦口之间的残余房间隔壁,以扩大ASD,自体心包修补ASD,并将肺静脉异位连接口与冠状静脉窦一并隔入左心房. 结果 结性心律4例,呼吸衰竭3例,全身水肿1例,大量胸腔积液5例;早期死亡1例,死亡原因为低心排血量综合征.其余患者均痊愈出院,随访6个月～10年,恢复良好,无吻合口狭窄发生. 结论 完全性肺静脉异位引流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,该手术对早晚期病变疗效均良好.     

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。     

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。     

Surgical Outcome of Isolated Total Anomalous Pulmonary Venous Connection in Adults: A 14‐Year Experience

Abstract Aim of the study: To investigate the surgical result of adult total anomalous pulmonary venous connection (TAPVC). Methods: From March 1997 to March 2011, 12 adult cases of isolated TAPVC, with an average age of 24.9 ± 6.7 years (from 18 to 41 years), underwent surgical repair in our department. All patients suffered from right‐sided volume overload with clinical manifestations varying from mild cyanosis to severe heart failure. Results: According to Darling's classification, eight cases were classified as supracardiac type, four as cardiac type. Unobstructed connections were established between the left atrium and the pulmonary common vein in all patients with external cardiac approach in four supracardiac cases, and internal cardiac approach in four cardiac and three supracardiac cases, and Warden technique in one supracardiac patient. Concomitant operations included De Vega's tricuspid annuloplasty in six patients, patent arteriosus ductus closure in two. All patients survived the operation, and postoperative follow‐up was 100% complete with a period ranging from 10 months to 14 years. NYHA grade decreased from 2.33 ± 0.49 to 1.08 ± 0.29 (p < 0.01). Three patients had postoperative tricuspid insufficiency. Five patients had cardiac arrhythmia, among two symptomatic cases; one controlled with medication, another received a successful radiofrequency ablation for incision‐related atrial flutter. Conclusions: Surgical correction of isolated adult TAPVC can be carried out safely with acceptable long‐term outcome. Postoperative tricuspid insufficiency and cardiac arrhythmias may have a negative long‐term impact, which should be evaluated preoperatively and managed individually during surgery. (J Card Surg 2012;27:736‐739)     

Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions.

OBJECTIVES: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. METHODS: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). RESULTS: Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3+/-0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7+/-0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005). CONCLUSIONS: Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.     

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.     

Total anomalous pulmonary venous drainage.

Twelve patients with total anomalous pulmonary venous drainage (TAPVD) underwent complete surgical correction. Six were of the supracardiac type, 2 were cardiac and 4 of the infracardiac type. Pulmonary hypertension due to pulmonary vein obstruction was present in 6 patients. There were 6 early deaths, which occurred on the table or soon after surgery. One patient died 8 months after the operation because of a marked obstruction of the pulmonary venous inflow. Early diagnosis and operative correction in these severely ill patients should not be delayed. Postoperative intensive care and long-term follow-up are of the greatest importance.     

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     

Surgical repair of total anomalous pulmonary venous connection in neonates and infants--prevention for pulmonary venous obstruction at the site of anastomosis

From October, 1980, to June, 1987, thirty-eight infants less than one year old underwent correction for total anomalous pulmonary venous connection (TAPVC). Overall operative mortality and late mortality were 13% and 6%, respectively. Residual pulmonary hypertension was noted in 4 patients: three had pulmonary venous obstruction at the site of atrial anastomosis (PVOA) and one had supracardiac (Ia) lesion left after repair of mixed type (IV: Ia + III) of TAPVC. Two late deaths occurred in these with PVOA. Twenty-two patients with supracardiac (I) or infracardiac (III) TAPVC were divided into three groups according to the technical development in atrial anastomosis: the large anastomosis in which venous incision reached into at least one pulmonary vein beyond common pulmonary vein and the continuous running suture were used in 10 patients (group 1), the appropriate size of anastomosis in which venous incision limited within the common pulmonary vein and the continuous running suture used in 4 patients (group 2), and the appropriate size of anastomosis and the interrupted suture in 8 patients (group 3). PVOA were 3 (33%) in group 1, but 0 (0%) in group 2 and 3. Two late death occurred all in group 1 with PVOA. Cardiopulmonary bypass time and aortic clamp time in group 3 were 91 min and 74 min respectively, which did not become longer than those in both group 1 and group 2. Interrupted suture technique does not make operating time longer than continuous running suture one. PVOA is one of the important factors predicting late operative result.(ABSTRACT TRUNCATED AT 250 WORDS)     

Modified repair of mixed total anomalous pulmonary venous connection

Mixed total anomalous pulmonary venous connection (TAPVC) is a rare condition, accounting for only 5% of diagnosed TAPVC. It is associated with a poor prognosis unless surgically corrected by connecting the pulmonary venous sinus to the left atrium and optionally dividing the abnormal connection to the systemic venous system. We report a modified technique of complete intracardiac repair of mixed supracardiac and cardiac TAPVC in a 3-year-old child without pulmonary venous sinus-to-left atrium anastomosis.     

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