半旋转动脉干调转术治疗完全性大动脉转位合并室间隔缺损和肺动脉狭窄

目的 报道半旋转动脉干调转术治疗完全大动脉转位(TGA)合并室间隔缺损(VSD)和肺动脉狭窄(PS)的近期疗效.方法 2例病儿男、女各1例,分别为16岁和19个月.均为TGA/VSD/PS.采用半旋转动脉干调转术治疗.结果 2例病儿均生存.女病儿术后发生低心排出量综合征和毛细血管渗漏综合征,治疗后痊愈.男病儿术后恢复良好.术后分别随访10、9个月,心功能Ⅰ级.结论 半旋转动脉干调转术可有效治疗TGA合并VSD和PS.长期效果仍需观察.     

完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

目的 分析和评估纠正型大血管错位伴肺动脉狭窄病儿采用双调转(Double-Switch)手术的治疗效果.方法 2001年8月至2008年12月采用Double-Switch手术行纠正型大血管错位伴肺动脉狭窄21例.其中男11例,女10例;年龄3.5个月至6.3岁,平均(31±18)个月;体重6～28 kg,平均(13.1±6.5)kg.室间隔缺损(VSD)为膜周型15例,远离大动脉1例,肺动脉下5例.轻度二尖瓣反流2例,中度反流2例.采用Senning+Rastelli手术方法 .13例右心室与肺总动脉的连接采用牛颈静脉管道.结果 全组均生存.体外循环转流156～287 min,平均(202.0±35.8)min;主动脉阻断93～161min,平均(138.0±19.8)min.均无完全性房室传导阻滞.随防2～5年,上腔静脉流速增快3例(1.8～2.2)m/s,其中1例术后2.6年再次手术解除上腔静脉梗阻,余2例在随防中;肺静脉回流均无明显梗阻;牛颈静脉管道通畅,解剖右室流出道连接处梗阻2例,压力阶差在30～45 mm Hg(1 mm Hg=0.133kPa),尚在随防中.结论 Double-Switch手术纠治纠正型大血管错位伴肺动脉狭窄的复杂型先心病,取得了较好效果,但仍需进一步随访,以观察这类复杂手术方法 的长期疗效.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

Objective To compare the results of surgical repair for congenitally corrected transposition of the great arter-ies(ccTGA) with pulmonary stenosis(PS), and to analyze the risk factors that may affect early results and surgical technique. Methods From Aug. 2001 to Dec. 2008, 21 patients with ccTGA and PS were treated in our hospital. They aged 3.5 months to 6.3 years [(31 ± 18) months], weighted 6.28 kg [(13.1± 6.5) kg]. Fifteen cases had paramembranous ventricular sep-tal defect (VSD), 1 noncommitted VSD and 5 subpulmonary VSD. The repairs comprised of Senning and Rastelli operation. RV-PA reconstruction was done by xenopericardial conduit in 13 patients. RV-PA direct anastomosis plus pericardial patch in 6 patients and homograft patch with autopulmonary valve in 2 patients. Results All the patients were alive. During 2 - 5 years follow-up, blood flow rate in superior vena cava increased to 1.8 - 2.2 m/s in 3 cases. One of them needed reoperation to re-lease the stenosis and the rest 2 were in follow-up. Tow patients had right ventricular outlet obstruction with a pressure gradient of 30 -45 mmHg were in follow-up. There was no other stenosis and valve insufficiency. Conclusion Double-Switch opera-tion is practical and impressive in treating of CCTGA with PS. It is important to evaluate the size of VSD and the reconstruction of RV-PA. But it still need more cases and longer time to evaluate the long-term effects.     

双调转术纠治小儿纠正型大血管错位伴肺动脉狭窄

Objective To compare the results of surgical repair for congenitally corrected transposition of the great arter-ies(ccTGA) with pulmonary stenosis(PS), and to analyze the risk factors that may affect early results and surgical technique. Methods From Aug. 2001 to Dec. 2008, 21 patients with ccTGA and PS were treated in our hospital. They aged 3.5 months to 6.3 years [(31 ± 18) months], weighted 6.28 kg [(13.1± 6.5) kg]. Fifteen cases had paramembranous ventricular sep-tal defect (VSD), 1 noncommitted VSD and 5 subpulmonary VSD. The repairs comprised of Senning and Rastelli operation. RV-PA reconstruction was done by xenopericardial conduit in 13 patients. RV-PA direct anastomosis plus pericardial patch in 6 patients and homograft patch with autopulmonary valve in 2 patients. Results All the patients were alive. During 2 - 5 years follow-up, blood flow rate in superior vena cava increased to 1.8 - 2.2 m/s in 3 cases. One of them needed reoperation to re-lease the stenosis and the rest 2 were in follow-up. Tow patients had right ventricular outlet obstruction with a pressure gradient of 30 -45 mmHg were in follow-up. There was no other stenosis and valve insufficiency. Conclusion Double-Switch opera-tion is practical and impressive in treating of CCTGA with PS. It is important to evaluate the size of VSD and the reconstruction of RV-PA. But it still need more cases and longer time to evaluate the long-term effects.     

Nikaidoh术治疗完全型大动脉错位伴室间隔缺损和肺动脉狭窄的早期结果

目的回顾性研究Nikaidoh术治疗完全型大动脉错位伴室间隔缺损和肺动脉狭窄(TGA/VSD/PS)的早期结果。方法在2004年1月至2005年12月期间,有8例TGA/VSD/PS患者在我院接受Nikaidoh术矫治,手术年龄4～29个月(11.4±7.6个月),体重5.2～11.0kg(8.0±1.9kg);所有患者房室连接一致,其中伴房室瓣骑跨1例,左肺动脉狭窄1例,本组患者术前均未行其他手术,手术均采用改良Nikaidoh术,即主动脉根部移位和重建左、右心室流出道,除1例冠状动脉同时移植和使用Homograft重建右心室流出道外,用自体心包补片扩大重建右心室流出道。结果手术死亡1例,无1例出现明显左室流出道梗阻(LVOTO)和右室流出道梗阻(RVOTO),轻度肺动脉反流3例,中度4例,除1例死亡患者外,其余左心功能均正常;随访时间平均8.8个月(3～18个月),7例存活;心功能状况佳,射血分数(EF)0.64±0.02;短轴缩短率(FS)0.33±0.02。未出现进展性主动脉瓣反流、LVOTO、RVOTO和肺动脉反流加重。结论Nikaidoh术适宜于治疗TGA/VSD/PS,尤其当解剖上存在不宜行Rastelli术的患者,早期结果良好。     

完全型大动脉错位伴室间隔缺损、肺动脉狭窄的外科治疗

治疗完全型大动脉错位伴室间隔缺损、肺动脉狭窄(TGA/VSD,PS)的手术方式有Rastelli手术、Lecompte手术、Nikaidoh手术、Yamagishi手术以及动脉转位 左心室流出道扩大术等多种术式,Rastelli手术和Lecompte手术损伤小,但远期并发症多;Nikaidoh手术和Yamagishi手术解剖纠治完全,但手术损伤大;动脉转位术 左心室流出道扩大术适应证相对较狭窄。因此,在TGA/VSD,PS的外科治疗上,应根据患者的自身情况和手术指征采取不同的手术方法。现对上述手术术式的优缺点、适应证、禁忌证、疗效和展望进行综述。     

完全性大动脉错位、室间隔缺损伴重度肺动脉高压的治疗

2001年8月至2005年8月，我们对5例完全性大动脉错位（D—TGA）、室间隔缺损（VSD）伴重度肺动脉高压（PH）患儿施行VSD补片开窗的大动脉调转术，取得了满意疗效，现报告如下。     

自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣狭窄

目的探讨解剖纠治完全性大血管错位（TGA）伴肺动脉瓣狭窄的手术方法。方法1例8月龄、体重6．5kg的TGA伴肺动脉瓣狭窄男病婴，术中将原主动脉瓣移植至肺动脉瓣处形成新的主动脉；再行Switch术，同种带瓣管道连接右心室与肺动脉。另1例10月龄、体重9．8kg的该病男病婴，术中将整个主动脉瓣取下，保留左、右冠状动脉，向后移植，后半部分直接与原肺动脉瓣环连续缝合，前半部分与室间隔缺损之间采用dacron补片连续缝合关闭；肺动脉与右心室切口直接连接。结果2例手术均取得成功。出院时超声检查各吻合口通畅，无残余分流．无主动脉瓣反流。术后随访12个月和6个月，生长发育良好，心胸比率0．75。超声示左心功能良好，前例示主动脉瓣轻度反流．后例左、右心室流出道无残余梗阻．结论采用自体主动脉瓣移植纠治完全性大血管错位伴肺动脉瓣和瓣下狭窄，从解剖上得到彻底纠治．效果良好。由于病例少，随访时间短．还需进一步观察。     

Rastelli手术治疗大动脉转位伴室间隔缺损

目的介绍Rastelli手术治疗大动脉错位伴室间隔缺损的经验.方法全组49例中男29例,女20例.平均年龄5.6岁;平均体重17.2kg.完全性大动脉转位31例,纠正性大动脉转位18例;伴肺动脉狭窄45例,伴肺动脉高压4例.均在低温体外循环下行Rastelli手术.二期根治5例.体外循环灌注(178.5±52.5)min;主动脉阻断(109.2±38.3) min.结果手术早期死亡6例,死亡率12.2%.死因为肺动脉高压危象、肾衰、心律紊乱和严重低心输出量综合征.术后并发症有心律紊乱、肺动脉高压危象、蛛网膜下腔出血、脑功能紊乱、肾衰及多脏器功能衰竭、心包或胸腔积液、感染等.CICU平均监护7.3 d.随访中因同种带瓣大动脉(VHC)感染死亡1例.结论完全性大动脉转位手术中,右室流出道直切口有利于心内隧道修补室间隔缺损;纠正性大动脉转位手术中,解剖右室径路显露缺损较好且易避开传导系统,但不利于术后心功能恢复.而解剖左室径路修补室间隔缺损的房室传导阻滞发生率高;大于4岁者手术宜选择大号VHC可减少再次手术几率;对无长段左室流出道狭窄的完全性大动脉转位病婴,可在动脉换位术基础上行肺动脉瓣叶交界切开或Konno术以解除左室流出道梗阻.为防止术后功能性二尖瓣反流,对伴肺动脉狭窄的纠正性大动脉转位病儿,提倡心房-大动脉双换位手术.     

合并室间隔缺损的大动脉转位所致肺动脉高压的可逆性程度及其分子机制

目的 探讨合并室间隔缺损(VSD)的完全性大动脉转位(TGA)所致肺动脉高压的可逆性程度及其分子机制.方法 24例平均肺动脉压力大于30 mmHg(4.0 kPa)病儿,其中大动脉转位伴室间隔缺损10例,单纯室间隔缺损14例.在体外循环开始前取其右中叶肺组织,标本经HE及弹力纤维染色,评估肺血管病变程度.ELISA检测标本中eNOS、iNOS、ET-1、ET-AR、ET-BR、MMP-2、MMP-9及TIMP的表达情况.结果 两组病儿年龄、身高、体重、VSD大小、术前肺动脉压力筹异均无统计学意义,而血红蛋白浓度、主动脉及肺动脉内氧饱和度及术后肺动脉压力下降值筹异明显,P<0.05.两组肺标本Heath-Edwards分级0～Ⅱ级,ELISA检测发现TGA组的eNOS及MMP-2表达,分别为(280.13 ±101.92)ng/mg和(31.68±15.36)ng/mg,明显低于单纯VSD组的(488.41±249.6)ng/mg和(69.28±49.12)ng/mg,P<0.05.两组iNOS、ET-1、ET-AR、ET-BR、MMP-9及TIMP表达差异无统计学意义.结论 NOS系统、ET系统及MMP/TIMP系统的失衡参与TGA合并VSD者的肺高压形成过程,相对于单纯VSD者,TGA者肺循环的高氧合状态使MMP-2和eNOS表达下调,在一定程度上影响肺动脉高压的进展及其肺血管病变的可逆性程度.     

大动脉转换术在复杂先天性心脏病治疗中的应用

目的 回顾总结我院近3年采用大动脉转换术治疗小儿复杂先天性心脏病(先心病)的临床经验。方法2000年1月至2003年5月,采用大动脉转换术(Switch手术)治疗小儿复杂先心病61例。其中完全性大血管错位(TGA)45例,包括室间隔完整型(TFGA-IVS)26例,伴室间隔缺损(TGA,VSD)19例;右心室双出口伴肺动脉瓣下室间隔缺损(Taussig-Bing)16例。结果TGA,IVS死亡2例;TGA-VSD死亡4例;Taussig-Bing死亡4例;总手术病死率16％。术后随访3个月-3年,所有病例青紫症状消失,活动能力明显增强。1例Taussig-Bing术前二尖瓣轻～中度返流,术后仍为中度返流。2例TGA术后出现主动脉和肺动脉瓣上狭窄,压差40mmHg,1例肺动脉瓣下狭窄和残余室间隔缺损,3个月后再次手术治愈。结论大动脉转换术应用于完全性大血管错位的纠治,手术效果满意;应用于右室双出口肺动脉瓣下室间隔缺损的纠治,不但可早期纠治,防止其肺血管阻塞性病变发生,而且避免了心内修补左室流出道梗阻的远期并发症。     

合并室间隔缺损的大动脉转位所致肺动脉高压的可逆性程度及其分子机制

Objective Explore the reversibility and potential molecular mechanisms of pulmonary hypertension in pa-tients with complete transposition of the great arteries (cTGA) combined with ventricular septal defect (VSD) in comparison with those with simple VSD. Methods Twenty-four patients with pulmonary hypertension (mean pulmonary arterial pressure was greater than 30 mmHg) were enrolled in our study, in which 10 patients suffered from cTGA with VSD, and the rest 14 pa-tients suffered from simple VSD. Lung specimens were taken from the right middle lobe of lung before cardiopulmonary bypass. The extent of pulmonary hypertension was then graded according to the Heath-Edwards classification. ELISA was used to exam-ine the expression of eNOS, iNOS, ET-1, ET-AR, ET-BR, MMP-2, MMP-9 and TIMP in all the specimens. Results No statistically significant differences in age, height, weight, the size of VSD, and the pulmonary artery pressure before operation were found between the groups. The level of hemoglobin, aortic and pulmonary arterial oxygen saturation, and the reduction value of pulmonary arterial pressure after surgery were significantly higher in the cTGA patients than that in the simple VSD pa-tients (P < 0.05). All patients had grade 0 - Ⅱ Heath-Edwards changes in their lung biopsy samples. The expression of eNOS and MMP-2 was significantly lower in the TGA group than that in the simple VSD group [eNOS: (280.13 ± 101.92) ng/mg vs. (488.41±249.6) ng/mg, P<0.05; MMP-2:(31.68±15.36)ng/mg vs. (69.28±49.12)ng/mg, P<0.05]. There were no statistically significant differences between the two groups regarding the expression of iNOS, ET-1, ET-AR, ET-BR,MMP-9 or TIMP. Conclusion The imbalance of the NOS/ET system and the MMP/TIMP system involves in the development of pulmonary hypertension in patients with TGA combined with VCD. In patients with cTGA, the high oxygenation state in pul-monary circulation may decrease the expression of MMP2 and eNOS, and may affect the progress of pulmonary hypertension to a certain extent.     

合并室间隔缺损的大动脉转位所致肺动脉高压的可逆性程度及其分子机制

Objective Explore the reversibility and potential molecular mechanisms of pulmonary hypertension in pa-tients with complete transposition of the great arteries (cTGA) combined with ventricular septal defect (VSD) in comparison with those with simple VSD. Methods Twenty-four patients with pulmonary hypertension (mean pulmonary arterial pressure was greater than 30 mmHg) were enrolled in our study, in which 10 patients suffered from cTGA with VSD, and the rest 14 pa-tients suffered from simple VSD. Lung specimens were taken from the right middle lobe of lung before cardiopulmonary bypass. The extent of pulmonary hypertension was then graded according to the Heath-Edwards classification. ELISA was used to exam-ine the expression of eNOS, iNOS, ET-1, ET-AR, ET-BR, MMP-2, MMP-9 and TIMP in all the specimens. Results No statistically significant differences in age, height, weight, the size of VSD, and the pulmonary artery pressure before operation were found between the groups. The level of hemoglobin, aortic and pulmonary arterial oxygen saturation, and the reduction value of pulmonary arterial pressure after surgery were significantly higher in the cTGA patients than that in the simple VSD pa-tients (P < 0.05). All patients had grade 0 - Ⅱ Heath-Edwards changes in their lung biopsy samples. The expression of eNOS and MMP-2 was significantly lower in the TGA group than that in the simple VSD group [eNOS: (280.13 ± 101.92) ng/mg vs. (488.41±249.6) ng/mg, P<0.05; MMP-2:(31.68±15.36)ng/mg vs. (69.28±49.12)ng/mg, P<0.05]. There were no statistically significant differences between the two groups regarding the expression of iNOS, ET-1, ET-AR, ET-BR,MMP-9 or TIMP. Conclusion The imbalance of the NOS/ET system and the MMP/TIMP system involves in the development of pulmonary hypertension in patients with TGA combined with VCD. In patients with cTGA, the high oxygenation state in pul-monary circulation may decrease the expression of MMP2 and eNOS, and may affect the progress of pulmonary hypertension to a certain extent.