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.     

新生儿完全性肺静脉异位引流的矫治及预后相关危险因素分析

目的评价单中心的新生儿完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)外科矫治情况,评估手术的危险因素。方法纳入2002年9月至2014年3月在我院行TAPVC外科矫治的新生儿患者74例,其中男59例、女15例,中位手术年龄10.5 d。心上型35例(47.3%)、心内型16例(21.6%)、心下型17例(23.0%)、混合型6例(8.1%)。采用Cox多因素分析死亡的危险因素,用Binary logistic回归分析术后吻合口或肺静脉狭窄的危险因素。结果共有18例患儿死亡。不同分型的死亡率:心上型占25.7%(9/35),心内型占18.8%(3/16),心下型占17.6%(3/17),混合型占50.0%(3/6)(P=0.413)。术后早期发生吻合口或肺静脉狭窄13例,心上型占17.1%(6/35),心内型占12.5%(2/16),心下型占17.6%(3/17),混合型占33.3%(2/6)(P=0.700)。术后发生吻合口或肺静脉狭窄21例,10例患儿死亡[47.6%(10/21)vs.15.1%(8/53),P=0.003],差异有统计学意义。手术后死亡的独立影响因素为体重3 kg(P=0.036)。术后吻合口或肺静脉狭窄的发生与使用Sutureless与否(P=0.010)及机械通气时间相关(P=0.000)。结论 Sutureless技术可有效降低术后吻合口或肺静脉狭窄的几率,术后发生吻合口或肺静脉狭窄的患儿死亡率明显增高,体重3 kg是术后死亡的独立危险因素,应引起临床医生的高度重视。     

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...     

改良Sutureless技术治疗完全性肺静脉异位引流

目的 介绍一种改良Sutureless技术及其治疗完全性肺静脉异位引流的手术效果。方法 回顾性分析阜外医院小儿心外科2014—2019年行改良Sutureless技术治疗11例完全性肺静脉异位引流患者的临床资料,其中男4例、女7例,中位手术年龄1.4(0.3,27.0)个月,中位体重4.3(3.5,8.5)kg。心上型6例（54.5%）、心下型5例（45.5%）。术前重度肺动脉高压5例（45.5%）,术前肺静脉梗阻3例（27.3%）。并将其手术治疗结果与同期行传统手术方法治疗的10例患儿进行比较。结果 中位随访时间12(1～65)个月。随访期间,改良Sutureless组未出现死亡及术后肺静脉梗阻事件,在围术期资料及免除再梗阻等方面均较传统手术组显示出优势,但差异无统计学意义（P>0.05）。改良Sutureless组术后生存优于传统手术组（P=0.049）。结论 包括“先部分缝合再切开”及“肺静脉内膜外翻扩大开口”等创新点的改良Sutureless技术,是一种治疗完全性肺静脉异位引流的安全可靠的方法,术后近期效果良好。     

改良心包斜窦路径在新生儿完全性肺静脉异位引流矫治术中的应用及效果评价

目的观察改良心包斜窦路径在新生儿完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)矫治术中的应用及效果评价。方法回顾性分析2005年7月至2015年12月在广东省心血管病研究所行心上、心下型完全性肺静脉异位引流外科矫治67例新生儿的临床资料,其中男53例、女14例,中位手术年龄12.5(7.0,20.5)d。根据手术入路将患者分为3组:房间沟路径组(6例)、心房顶路径组(14例),改良心包斜窦路径组(47例)。比较三组临床效果。结果改良心包斜窦路径组体外循环时间[88(80.0,107.0) min vs. 135(121.0,157.0)min,P0.05]及主动脉阻断时间[45(39.0,53.0)min vs. 80(73.0,85.0)min,P0.05]均显著短于房间沟路径组。改良心包斜窦组围术期死亡率明显低于心房顶路径组(2.1%vs. 28.6%,P0.05),改良心包斜窦组远期死亡率明显低于房间沟路径组(4.3%vs. 60.0%,P0.05)及心房顶路径组(4.3%vs. 30.0%,P0.05)。改良心包斜窦路径组术后吻合口狭窄发生率明显低于房间沟路径组(2.1%vs. 50%,P0.05)及心房顶路径组(2.1%vs.35.7%,P0.05)。结论在新生儿心上型及心下型TAPVC的矫治中,与传统手术路径相比,改良心包斜窦路径能够提供较为满意的手术空间,缩短手术时间,降低术后肺静脉吻合口发生率、围术期及远期死亡率,可获得较为满意的治疗效果。     

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.     

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。     

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.     

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。     

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.     

787例小于6月龄先天性心脏病患者外科治疗的临床分析

目的回顾性总结婴幼儿先天性心脏病的手术时机和手术治疗方法,以进一步提高手术成功率和远期疗效。方法1988年1月～2003年6月,手术纠治年龄小于6个月的先天性心脏病患者787例。主要病种包括完全性大动脉错位109例,完全性肺静脉异位引流51例,肺动脉闭锁16例,主动脉缩窄33例,室间隔缺损伴肺动脉高压299例,法洛四联症44例,右心室双出口23例,室间隔完整型肺动脉闭锁9例等;对787例患者根据不同病种采取相应的手术方法纠治。结果手术死亡77例,手术死亡率9.78%(77/787)。随着手术方法的不断改进,手术总死亡率从1988～1995年的25%降至2003年的4.11%。随访完全性大动脉错位患者中发生VSD残余漏1例,术后3个月再次手术治愈;随访中发生肺动脉和主动脉瓣上狭窄2例。完全性肺静脉异位引流心内型患者中2例分别在术后4d和2个月出现肺静脉回流梗阻,1例死亡,1例再次手术解除梗阻。室间隔缺损患者中发生残余漏5例,分流量小,不需再次手术。其余病例随访资料不完整。结论对婴幼儿先天性心脏病施行手术治疗的时间非常重要,危重复杂型先天性心脏病患者如不早期手术,将失去手术机会,增加术后危险性和死亡率。     

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)     

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。     

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.     

Surgical palliation of cardiac malformations associated with right isomerism

Between 1985 and 1993, palliative surgery was performed on 13 pediatric patients who had complex cardiovascular anomalies associated with right isomerism. The patients included two neonates, ten infants, and one child who were divided into two groups according to whether or not a total anomalous pulmonary venous connection (TAPVC) was present. Group 1 consisted of six patients with TAPVC and group 2 consisted of seven patients without TAPVC. In group 1, the surgical procedures involved TAPVC repair alone in two patients, combined TAPVC repair with a modified Blalock-Taussig shunt in two, combined TAPVC repair with pulmonary artery banding in one, and a modified Blalock-Taussig shunt alone in one. There were five hospital deaths and one late death in this group: pulmonary venous obstruction in two patients, perioperative myocardial failure in the two neonates, and congestive heart failure caused by increased pulmonary blood flow in two patients. In group 2, all the patients underwent systemic-pulmonary artery shunts, and there was one hospital death and three late deaths, the causes of which were unknown in two patients, and shunt failure and pneumonia in one patient each. These results suggest that surgical palliation for right isomerism produces poor results in young infants with obstructed TAPVC. Thus, we conclude that TAPVC repair should be performed without delay if pulmonary venous obstruction has been diagnosed clinically. Resolving pulmonary venous obstruction without cardiopulmonary bypass (CPB) may be preferable for infants, considering their difficult management. The systemic-pulmonary artery shunt should be of the low-calibrated type, especially if common atrioventricular valve regurgitation exists. If infants survive the surgery, they must be carefully followed up for a long period due to the risk of sudden death or infection.     

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.     

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     

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.     

心下型完全性肺静脉异位引流外科治疗的回顾性队列研究

目的比较无内膜接触缝合(Sutureless)手术和传统手术纠治心下型完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的效果。方法回顾性分析2014年6月至2019年4月于我院行手术纠治的46例心下型TAVPC患者的临床资料,排除合并单心室、法洛四联症等患者。根据手术方式将患者分为传统手术组和Sutureless手术组。传统手术组35例,男28例(80.0%)、女7例(20.0%),中位年龄21(8,42)d,中位体重3.6(3.0,4.0)kg。Sutureless手术组11例,男8例(72.7%)、女3例(27.3%),中位年龄14(6,22)d,中位体重3.5(2.9,3.6)kg。比较两组手术疗效。结果传统手术组死亡5例(10.9%),其中院内死亡4例(8.7%),晚期死亡1例(2.2%)。传统手术组死亡率(14.3%,5/35)高于Sutureless手术组(0.0%,0/11),但差异无统计学意义(P=0.317)。Cox回归模型分析发现性别(P=0.042)、年龄(P=0.028)、体外循环时间(P=0.007)、主动脉阻断时间(P=0.018)、气管插管时间(P=0.042)是术后死亡的危险因素。中位随访时间为18.00(5.00,37.75)个月。术后发生肺静脉梗阻22例,均为传统手术组患者,两组差异有统计学意义(P=0.000)。结论对于心下型TAPVC,与传统手术相比,Sutureless手术可以降低术后肺静脉梗阻发生率。