手术治疗纠正型大动脉错位

目的　总结治疗纠正型大动脉错位的手术方法和经验。方法　 1990年 1月～ 1998年12月 ,手术治疗纠正型大动脉错位 (C TGA) 31例 (占同期手术 0 .6 % )。男性 2 1例 ,女性 10例。手术年龄 2～ 9岁 (平均 5 .2 8± 1.76岁 ) ,体重 7～ 2 2 .5kg(平均 15 .96± 3.78kg)。其中伴左室流出道梗阻2 7例 ,肺动脉高压 4例。 2 4例做双心室修补术 ,其中 12例经形态左心室切口修补室缺 ,死亡 3例 ;12例经形态右心室切口修补室缺 ,死亡 1例。Fontan纠治手术 5例 ,死亡 1例。双向腔肺动脉吻合术 2例 ,无死亡。结果　全组手术死亡率 16.1%。长期随访的疗效不理想 ,主要形态右心室不能长期耐受体循环压力 ,易发生三尖瓣反流。结论　C TGA伴肺动脉高压 ,必须在 1岁以内早期手术。目前双心室手术方法的远期结果尚不理想 ,应以解剖纠治方法为好。     

纠正型大动脉转位的治疗进展

纠正型大动脉转位是一种较为罕见的复杂先天性心脏病,常伴有室间隔缺损、肺动脉狭窄或闭锁、三尖瓣Ebstein样畸形以及中位心等.其病理解剖的基本特征是心房心室连接不一致,心室大动脉连接不一致.1957年开展了生理性纠正手术,二十世纪八十年代末期开展了解剖性纠正手术,即双调转手术,包括心房调转加动脉调转手术和心房调转加心室...     

73例新生儿先天性心脏病的手术时机选择分析

目的 探讨新生儿期先天性心脏病的内外科治疗结果及经验.方法 2010年1月至2011年6月间73例在我院就诊的先天性心脏病新生儿,体重1.6～5 kg,平均(3.1±0.7) kg,早产儿有14例.54例实施了手术治疗,其中45例在体外循环下手术,9例在非体外循环下实施手术.结果 73例患儿中12例自动放弃治疗,7例经治疗后随诊择期手术治疗,54例手术患儿中近期死亡5例,病死率9％.结论 开展新生儿期复杂性先天性心脏病的手术治疗应遵循早期诊断、专科治疗、及时手术的原则.对于无明显的心功能不全和反复肺部感染症状的患儿,可密切门诊随访;新生儿期就出现明显的心功能不全表现者必须及时手术.对于严重低氧血症或伴有循环梗阻的先天性心脏病及气道压迫明显的肺动脉吊带患儿应积极完善术前准备,早期手术,可明显降低病死率,改善治疗效果.     

小于6个月先天性心脏病患儿的外科治疗

目的 回顾总结小于6个月婴幼儿先天性心脏病的外科治疗.方法 2000年1月～2006年12月,手术纠治年龄小于6个月先天性心脏病1831例.包括:室间隔缺损并肺动脉高压453例,完全性大动脉错位214例、法乐四联症119例、完全性肺静脉异位引流106例、右室双出口69例、肺动脉瓣狭窄65例,完全性房室通道55例、动脉导管未闭53例、主动脉缩窄伴室间隔缺损46例、肺动脉闭锁伴室间隔缺损36例、肺动脉闭锁室间隔完整型31例、主动脉弓中断24例、右室双出口伴肺动脉瓣下室间隔缺损22例、其他538例.根据不同病种采取相应的手术方法纠治.结果 手术死亡98例,手术死亡率5.35%.随着手术方法不断改进,手术总死亡率从2000年的8.23%降至2006年的4.91%.随访:完全性大动脉错位术后发生室间隔残余漏1例,肺动脉瓣上狭窄5例,主动脉瓣上狭窄2例,主动脉瓣下狭窄1例,其中再次手术4例,远期死亡1例;完全性肺静脉异位引流心内型4例在术后出现肺静脉回流梗阻,2例死亡,2例再次手术解除梗阻.早期室间隔缺损术后发生残余漏5例,分流量小,不需再次手术;其余病例随访不完整.结论 婴幼儿先天性心脏病的手术处理时间非常重要,危重复杂型先天性心脏病如不早期手术,将失去手术机会,增加术后危险性和死亡率.     

中央分流术在治疗复杂先天性心脏病中的应用

目的 总结68例中央分流术的应用及随访,探讨中央分流术在复杂先天性心脏病治疗中的应用及效果.方法 68例患儿,男36例,女32例,年龄1 d～17岁,体重(9.75±5.83)kg.患儿在不同时期进行了中央分流手术,比较手术前后血氧饱和度的变化.术后进行随访,了解肺动脉发育和后续治疗的情况.结果 死亡8例,病死率11.76%.存活患儿症状改善,术后血氧饱和度比术前显著提高(P＜0.05).随访病例肺动脉比术前显著增粗(P＜0.05).26例患儿进行了二次手术,21例为根治手术,5例为再次姑息性手术,2例死亡,再次手术病死率7.7%.结论 作为姑息性手术的一种,中央分流术损伤小、操作简单,能改善症状,提高生活质量,为根治手术创造条件.     

肺动脉环缩术在儿童复杂先天性心脏病中的应用

目的 探讨肺动脉环缩术在儿童复杂先天性心脏病中的应用经验,并对手术效果进行评价.方法 回顾性分析2002年1月～2007年3月进行的32例肺动脉环缩术.男22例,女10例,手术年龄平均9.2个月,体重平均6.1 kg.肺动脉环缩后肺动脉周径平均(28.0±4.1)mm,术后估测环缩近远端压差(43.7±9.0)mmHg.结果 围术期死亡1例,2例患儿再次调整环缩.4例患儿出现围术期并发症.31例患儿随访1个月～5年,22例患儿进行Ⅱ期手术.结论 肺动脉环缩术可限制肺血过多,锻炼左心室,为Ⅱ期施行双心室矫治及双向腔肺分流术赢得时机.     

新生儿危重先天性心脏病的手术治疗

目的 探讨新生儿危重先天性心脏病的病种组成、手术必要性以及手术治疗的方法和效果.方法 回顾性分析2012年1～6月广东省人民医院小儿心外科收治的新生儿危重先天性心脏病51例的临床资料.所有病例均行手术治疗,术后转心脏监护室.结果 本组51例中,术后成功出院47例(92.2％),死亡4例,病死率为7.8％.其中,完全性肺静脉异位引流死亡1例,死因为肺动脉高压危象;肺动脉闭锁死亡1例,死因为术后严重低氧血症、恶性心律失常;Taussig-Bing畸形死亡1例,死因为心力衰竭、低心排出量综合征;三尖瓣闭锁死亡1例,死因为术后低心排出量综合征.结论 新生儿危重先天性心脏病及时手术治疗,是挽救患儿生命的有效手段.根治手术是新生儿危重先天性心脏病主要手术方式,延迟关胸、体外膜肺氧和、腹膜透析等治疗的应用,可以减少危重心脏病新生儿的死亡.     

先天性心脏病外科修补术后患儿心理行为研究

目的 探讨先天性心脏病(CHD)患儿的心理行为状况,并对照研究外科修补手术与介入封堵治疗对CHD患儿心理行为的影响.方法 应用Aehenbach儿童行为量表分别对79例行外科修补手术和82例介入治疗的CHD患儿在术前和术后1年进行心理行为检测.选取80名与CHD患儿一般特征相匹配的健康儿童为对照组.结果 CHD儿童术前心理行为异常检出率及心理行为总粗分均显著高于对照组(P＜0.01),心理行为粗分均值亦高于对照组(P＜0.05).男孩主要表现为抑郁、交往不良、体诉、社会退缩、违纪及攻击方面,女孩主要表现为抑郁、社会退缩、体诉、违纪方面.术后1年测试,男、女二治疗组心理行为总粗分分别显著低于治疗前(P＜0.01),男、女二介入组总粗分与对照组比较差异无统计学意义;外科组男孩术后总粗分及攻击行为粗分分别高于介入组(P＜0.05),外科组女孩术后心理行为总粗分及抑郁、社会退缩和违纪行为粗分分别高于介入组(P＜0.05).心理行为因子异常检出率与病程呈正相关,病程越长检出率越高.结论 CHD患儿存在着明显的心理行为问题,宜早期治疗.与介入治疗相比,外科修补术后较长时间内患儿仍存有明显的心理行为问题,应引起更多重视.     

小儿三尖瓣下移畸形的手术效果分析

目的 小儿Ebstein畸形的手术纠治是当前的难点,本文意在探讨小儿Ebsetin畸形手术纠治的方法和分析手术效果.方法 回顾性分析2000年1月至2014年12月间于复旦大学附属儿科医院心血管中心就诊并手术的Ebstein畸形患儿45例,其中男28例,女17例,年龄0.2～169个月,平均(44.2±41.1)个月,含新生儿6例.体重2.4～60 kg,平均(17.6±12.8)kg,SPO2在60％～99％,平均(87.8±8.8)％,其中26.7％(12/45)存在较明显的紫绀,22.2％(10/45)存在明显心功能不全,合并Wolff-Parkinson-White预激综合征3例,房性早搏2例.结果 全组45例患儿接受了心脏手术,6例新生儿中5例在非体外循环下接受了改良B-T分流术,2例于术后早期死亡.39例患儿在体外循环下行三尖瓣成形术,Danielson法12例,Carpentier法21例,Cone法6例,无早期和晚期死亡.37例患儿于门诊密切随访,随访1.5个月至15年,7例因为三尖瓣反流加重进行了再手术.5、10、15年免再干预率分别为86.5％、81.1％、81.1％.按照不同手术方式分为3组进行比较,Carpentier组和Danielson组比较发现,Carpentier组手术年龄偏小[(45.0±33.7)个月比(80.8±40.2)个月,P=0.010],体重偏轻[(17.3±11.3)kg比(29.5±10.5)kg,P=0.004],所需主动脉阻断时间长[(44.9±4.7)min比(30.8±5.3)min,P=0.000],术后恢复时间无差异,随访术后再干预率差异无统计学意义.Cone组和Carpentier组比较发现,Cone组手术年龄更小[(12.2±7.3)个月比(45.0±33.7)个月,P=0.000],所需的体外循环时间[(89.2±12.0)min比(73.0±15.5)min,P=0.027],主动脉阻断时间更长[(56.3±6.1)min比(44.9±4.7)min,P=0.000],术后恢复时间差异无统计学意义,随访三尖瓣反流轻,无一例需要再干预,但是与Carpentier组相比差异无统计学意义,可能与病例数少(仅有6例),随访时间偏短有关.结论 Cone手术较其他手术方式更为复杂,手术时间较长,但是适用于小年龄儿童Ebstein畸形的矫治,早中期效果满意,仍需密切随访.     

3个月内复杂先天性心脏病患儿术后延迟关胸的因素分析

目的 探讨3个月内的复杂先天性心脏病患儿术后行延迟关胸的危险因素.方法 2000年1月～2006年10月收治119例胸骨正中切口并进行体外循环的患儿,男97例,女22例,年～龄1～90 d,平均(44±26)d,体重2.2～7.1 kg,平均(3.9±0.9)kg,分析年龄、性别、体重、术前胸围、诊断、是否为早产儿、术前是否机械通气、SpO2、胸片测得的心胸比例、心超测得的左心射血分数、体外循环时间、主动脉阻断时间、深低温停循环等因素与延迟关胸的相关性.结果 延迟关胸47例,占39.5%,主动脉弓离断或完全性肺静脉异位引流(心下型)或TGA/VSD的延迟关胸的比例高(延迟关胸率＞75%),单因素分析结果:年龄、体重、胸围、术前机械通气、体外循环时间、主动脉阻断时间、深低温停循环差异有统计学意义,Logistic回归分析发现:年龄、术前机械通气、体外循环时间、深低温停循环是独立的相关因素.结论 延迟关胸对于复杂先天性心脏病患儿来说是一种简单、安全、有效的手术技术,延迟关胸与主动脉弓离断或完全性肺静脉异位引流(心下型)或TGA/VSD有关,低年龄、术前机械通气、长时间体外循环、深低温停循环是延迟关胸的危险因素.     

动脉调转术治疗新生儿室间隔完整型大动脉转位及危险因素

目的 分析动脉调转术治疗新生儿室间隔完整型大动脉转位(D-TGA/IVS)临床结果及危险因素.方法 回顾分析上海儿童医学中心2000年1月至2006年12月共62例行动脉调转术治疗的D-TGA/IVS新生儿围术期临床结果.手术年龄15 h～28 d,平均(8.91±8.04)d,体重2.30～4.40 kg,平均(3.34±0.44)kg.手术均在深低温低流量体外循环下和深低温停循环下进行.结果 全组死亡6例,住院病死率9.7%.其中急诊手术组和非急诊手术组病死率分别为15.6%(5/32)和3.3%(1/30),小年龄组(年龄≤14 d)和大年龄组(年龄15～28 d)病死率分别为8.0%(4/50)和16.7%(2/12),冷晶体停搏液组和冷含血晶体停搏液组病死率分别为4.0%(1/25)和13.5%(5/37).典型冠状动脉组和非典型冠状动脉组病死率分别为8.3%(4/48)和14.3%(2/14).对应各组间病死率差异均无统计学意义.急诊手术组(148.52±193.98)与非急诊手术组(66.38±34.58)的术后插管时间差异有统计学意义(P=0.039),重症监护时间(P=0.088)、术后住院时间(P=0.100)差异无统计学意义;小年龄组与大年龄组、晶体停搏液组与含血停搏液组、典型冠状动脉组与非典型冠状动脉组的术后插管时间、重症监护时间、术后住院时间差异均无统计学意义.结论 新生儿DTGA/IVS行动脉调转术治疗,效果满意.术前明确诊断,纠正酸中毒、维持动脉导管开放,积极改善术前一般情况,术中精心的手术设计和操作,术后应用综合治疗措施,积极防治各种并发症,是降低围术期病死率的关键.早期诊断,早期手术,尽量减少急诊手术,可减少术后呼吸机辅助时间,有利患儿术后顺利康复.     

完全性大动脉转位患儿外科术后早期并发症和近期随访

Objective To present the short-term outcomes of the patients with transposition of the great arteries after repair. Methods Between 2008 and 2009,71 patients with transposition of the great arteries (TGA) underwent corrective surgeries at this center. The 71 TGA patients were divided into TGA/IVS group whose ventricle septum was intact, and TGA/VSD group who complicated with ventricle septum defect. Their clinical data and postoperative short-term outcomes were analyzed and compared. Results The postoperative complications included 7 (9. 9% ) patients presented with low cardiac output syndrome, 12 ( 16. 9% ) with pulmonary stenosis, 21 ( 29. 6% ) with arrhythmia, 6 (8. 5%) with diaphragmatic paralysis, 6 (8. 5%) with airway abnormalities, and 13 ( 18. 3% ) with bacteria in sputum culture. Three patients (4. 2%) died after surgery. Fifty four out of the 68 patients were followed up for an average period of 12. 0 ± 7. 4 months. Four (7. 4%) patients died during follow-up. Three patients (5. 6%) underwent interventional therapies for pulmonary stenosis. The survival rate of 57 patients was 87. 7% at the end of the first postoperative year. No differences of the postoperative complications and survival rate were noted between the TGA/IVS and TGA/VSD group.Conclusions The postoperative short-term outcomes of the patients with transposition of the great arteries are good.     

三房心的诊断和外科治疗

目的总结14例小儿三房心合并其他心脏畸形的外科治疗经验。方法全组均在中度低温体外循环下进行手术,平均年龄(5.6±3.5)岁,单纯型1例,复杂型13例(完全型11例,非完全型3例)。采用右房-房间隔径路显露左心房纤维隔膜,仔细辨认其解剖关系,充分剪除纤维隔膜疏通左心房通道,保护好二尖瓣膜。采用自体心包补片尽量扩大左心房容积,同时彻底矫正合并的其他心脏畸形。结果无一例手术死亡,14例全部存活,随访5个月~5年,心功能正常。结论三房心手术治疗取得早期和晚期良好疗效,手术关键在于仔细辨认,彻底矫正合并畸形。     

Aortic atresia and tricuspid atresia occurring in complete transposition of the great vessels

Summary A unique case of aortic atresia and tricuspid atresia associated with transposition of the great vessels is described in a newborn infant. The clinical, echocardiographic, and heart catheterization data are presented. The embryological and surgical implications are discussed.     

婴幼儿心脏病术后上腔静脉梗阻综合征的治疗

目的 总结小儿心脏外科术后并发上腔静脉梗阻综合征的原因、治疗策略及经验.方法 2009年1月至2010年5月北京阜外心血管病医院小儿心脏中心共发生3例心脏术后并发上腔静脉梗阻综合征.均根据临床表现、胸片及超声确诊,经内科治疗及外科手术缓解或解除梗阻.结果 2例患儿外科手术解除梗阻后均恢复了上腔静脉血流,胸腔积液于解除梗阻后减少,循环稳定,另1例不全梗阻经保守治疗后好转.结论上腔静脉梗阻综合征是小儿心脏外科少见的并发症,主要发生在新生儿低体重患儿中,由于其所致后果严重,应积极给予外科解除,并应注意预防产生上腔静脉梗阻的常见原因如血块及止血材料的压迫、上腔静脉荷包缝合及插管避免损伤上腔静脉内膜,同时预防深静脉所致的血栓.

Abstract：

Objective To summarize therapeutic experience of superior vena cava obstruction (SVCO) following corrective surgery for congenital heart disease in infants. Methods From January 1999 to May 2010, 3 infants were complicated with SVCO after congenital heart disease corrective surgery at this center. The diagnosis was made based on clinical manifestations, chest X-ray radiography and ultrasonography. Once the diagnosis was made, conservative therapy or surgery was performed to relief the obstruction of superior vena cava. Results Two patients underwent surgery, and the other one with incomplete SVCO received conservative management. After treatment, their blood circulation was stabilized, pleural effusion was also reduced. Conclusions SVCO is a rare complication following pediatric cardiac surgery. The infants with low birth weight have higher risks for developing postoperative SVCO. To prevent SVCO, blood clots and hemostatic agents should be carefully removed during surgery. Intimal injury of superior vena cava should be avoided during cannulation or placing purse string on the superior vena cava. Deep vein thrombosis should also be prevented.     

Follow-up outcomes 10 years after arterial switch operation for transposition of the great arteries: comparison of cardiological health status and health-related quality of life to those of the a normal reference population

The purpose of this study was to investigate the cardiological health status and health-related quality of life after the arterial switch operation (ASO) for transposition of the great arteries (TGA) in comparison with a normative reference group. Chart review and cross-sectional systematic follow-up, including echocardiography, exercise testing, and electrocardiography, were performed on all survivors of ASO for TGA between 1990 and 1995. Health-related quality of life (HRQOL) was assessed using a standardized questionnaire. A normative reference group was included. Forty-nine survivors [median age at operation 13 days, mean age at follow-up 11 +/- 2 years (37/49 with intact ventricular septum] were identified. Thirty-three of 49 patients (67%) [22/33 TGA with intact ventricular septum (IVS)] participated in cross-sectional follow-up. Cumulative 10-year event-free survival was 88% and the re-intervention rate 6%. Aortic root dilatation occurred in 70% of patients; none had severe aortic regurgitation. Left ventricular function was normal. Exercise performance (85% of reference capacity, p = 0.02), maximal oxygen uptake (85%, p < 0.01) and peak heart rate (95%, p < 0.01) were decreased. Exercise electrocardiogram was normal as was rhythm status. Unfavourable outcomes on HRQOL were found for motor functioning and positive emotional functioning. Overall there were no significant differences between TGA/IVS and TGA/VSD. We conclude that at mid- to long-term follow-up after ASO, major events and re-interventions (6%) occur infrequently. Exercise capacity and maximal oxygen uptake are lower than those in a reference population, which could not be related to diminished ventricular function. Aortic root dilatation is frequent, irrespective of the anatomical subgroup. Severe aortic regurgitation or left ventricular dilatation was not found. The unfavourable health-related quality of life deserves further attention.     

婴儿完全性大动脉转位外科根治术前血液动力学的内科调整研究

为探讨球囊房隔造口术（ＢＡＳ）及前列腺素Ｅ１（ＰＧＥ１）对婴儿完全性大动脉转位（ＴＧＡ）外科根治术前血液动力学的调整作用，对３２例年龄２～５６天，平均２６天的ＴＧＡ患儿行ＢＡＳ，对其中１１例于ＢＡＳ前给予ＰＧＥ１，然后进行血液动力学观察。结果：ＢＡＳ后动脉血氧饱和度（ＳａＯ２）由０．５３±０．１４上升至０．７４±０．１１。３０例患儿左右心房压差均＜０．２７ｋＰａ（１ｋＰａ＝７．５ｍｍＨｇ）。单纯ＴＧＡ术后２４个月内左室压力＜５．３０ｋＰａ。ＰＧＥ１应用后ＳａＯ２由术前０．６０±０．１９上升至０．８６±０．０５。心导管检查测得左右室压力比为０．８６。提示，ＢＡＳ和ＰＧＥ１的应用可缓解新生儿ＴＧＡ低氧血症及维持其左右心室良好的压力比值，从而为ＴＧＡ解剖转位术作准备     

个体化手术方案矫治儿童右室双出口的体会

目的 探讨儿童右室双出口(DORV)的外科治疗效果.方法 自1984年8月至2010年6月共手术治疗118例DORV,其中男62例,女56例,年龄10 d～12岁,平均(3.2±2.6)岁,体重3.0～22.5 kg,平均(9.6±3.7)kg.根据SIS-EACTS数据库分类:VSD型86例,TOF型22例,TGA型3例,VSD远离大动脉型7例.手术方法包括采用单纯心室内隧道修补21例,心室内隧道修补+右室流出道扩大补片84例,其中82例自体心包扩大补片,2例行同种带瓣外管道;Switch术4例,双向格林术6例,Fontan术1例,肺动脉环缩术1例,B-T分流术1例.回顾性分析DORV的手术方法和效果.结果 住院死亡9例,病死率7.6%,主要死亡原因为低心排综合症.2004年前手术51例,死亡7例(病死率13.7%),2005年后手术67例,死亡2例(病死率2.9%).存活病例随访2个月～10年,超声心动图检查未见左室流出道梗阻,1例患儿术后2年发现右室流出道残余梗阻,再次手术疏通右室流出道,余患儿效果良好,无晚期死亡病例.结论 根据DORV患儿的解剖特点和年龄,制订个体化的手术方案,有助于提高手术成功率.

Abstract：

Objective To summarize our experience of surgical treatment for double-outlet right ventricle (DORV) in children. Methods From August 1984 to June 2010, 118 patients with DORV underwent surgical repair at this center. Among the 118 patients, 62 were males and 56 were females.Their ages ranged from 10 days to 12 years old (mean age, 3. 2 ± 2. 6). Their weight ranged from 3. 0to 22. 5 kg (mean weight, 9. 6 ± 3. 7 kg). According to the STS-EACTS international nomenclature,86 patients were DORV associated with ventricular septal defect (VSD), 22 were DORV with tetralogy of Fallot (TOF), 3 were DORV with transposition of the greater arteries (TGA), and 7 were DORV with remote VSD. Corrective surgeries included 22 interventricular repairs, 84 interventricular repairs plus right ventricular outflow tract reconstruction (pericardium was used for the reconstruction in 82 cases, and valved extracardiac conduits was used in 2 cases), 4 switch procedures, 6 bidirectional Glenn procedures, 1 Fontan procedure, 1 Pulmonary artery banding procedure, and 1 Blalock-Taussig Shunt procedure. The surgical safety and efficacy were studied by retrospectively analyzing the clinical data of the 118 cases. Results Nine patients died after surgery (7. 6%). The leading cause of death was low cardiac output syndrome. Of the 51 patients underwent corrective surgery before 2004,7 died after surgery (13. 7%). However, only 2 died in the 67 patients who had surgery after 2005(2. 9%). The patients were followed up for 2 months to 10 years. Cardiac ultrasonography didn't show any obstruction of left ventricular outflow tract, but 1 patient developed left ventricular outflow tract obstruction 2 years after surgery and received corrective surgery. No long-term death and other complications were noted. Conclusions Surgical strategy for the children with double-outlet right ventricle should be made based on patients individual anatomic anomalies.