大于6月龄伴室间隔缺损并重度肺动脉高压心室大动脉连接异常的大动脉调转术

目的 总结大动脉调转术(ASO)根治>6月龄伴室间隔缺损(VSD)并重度肺动脉高压(PH)心室大动脉连接异常者手术疗效,探讨ASO适应证.方法 2000年5月至2008年10月治疗86例VSD并PH的心室大动脉连接异常者.男51例,女35例;年龄7月龄～19岁,平均(24±22)月龄;体重2.6～48.0 kg,平均(9.0±7.0)kg.肺动脉平均压50.0～97.0(64.9±13.0)mm Hg,肺血管阻力46.0～1261.9(324.0±249.0)dyn·s·cm ~(-5).手术在全麻低温(18～22℃)低流量(50 ml·kg~(-1)·min~(-1)体外循环下完成,同期矫治合并畸形.结果 手术死亡率7.0%(6/86例),均为2005年12月以前病例(40例),其中仅1例7岁病儿死因与PH有关;2006年1月以后连续46例无死亡.随访1～84个月,80例生存.2例(2岁及13岁)分别于术后2个月和1年半不明原因猝死,余78例心功能明显改善,无再手术及并发症.结论 年龄>6月龄并重度PH的TGA/VSD或TBA,如一般状况尚好,经皮血氧饱和度>0.60,X线胸片示两肺血尚多,肺血管阻力<1200 dyn·8·cm~(-5),ASO根治仍可取得满意效果,扩大了根治性ASO适应证.     

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。     

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.     

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.     

完全性大动脉转位合并重度肺动脉高压增加调转术后病死率

目的 探讨完全性大动脉转位(TGA)术前肺动脉高压与术后近、中期结果的关系.方法 将101例行动脉调转术的病儿根据术前平均肺动脉压力(mPAP)分为3组,肺动脉压力正常(对照)组,轻度肺动脉高压组和重度肺动脉高压组,比较分析不同组间的术后近中期结果.结果 手术后肺动脉高压组病儿肺动脉压力均有明显下降.术后各组间并发症和手术死亡比例差异无统计学意义.但是重度肺高压组中期病死率较高.结论 mPAP＜50 mm Hg(1 mm Hg=0.133 kPa)的TGA合并肺动脉高压病儿可以进行大动脉调转术,并可取得较好的术后结果,但mPAP≥50 mm Hg者虽然行大动脉调转术后肺动脉压力明显下降,且术后早期结果良好,但随访中期病死率较高.     

快速二期大动脉转位手术前等待期的临床观察

目的探讨超龄完全性大动脉转位(D-TGA)病婴经历了肺动脉环缩(PAB)和体肺分流(BTS)的左心室准备术后,如何安全度过快速二期大动脉转位术(ASO)前的等待期。方法2002年9月至2006年8月,21例D-TGA病婴实施了快速二期ASO前的准备(PAB和BTS)。结果等待期中,2例行BTS再通,2例行PAB松解,1例行再次PAB;12例并发低心排出量综合征,7例并发室上性心动过速,5例行心肺复苏,死亡率14.3%(3/21例)。18例度过准备期接受二期手术,等待期9(1～150)d,平均(22.4±36.7)d,死亡率16.7%(3/18例)。结论快速二期ASO前的等待期中,平衡左、右心室的压力和容量负荷,及时逆转低心排出量综合征,判断左心室准备完成、适时行二期ASO,是安全渡过准备期的关键。     

主动脉移位术纠治婴幼儿完全性大动脉错位伴室间隔缺损和肺动脉狭窄

目的探讨主动脉移位术纠治完全性大动脉错位伴室间隔缺损和肺动脉狭窄(TGA/VSD/PS)的临床应用。方法2004年8月到2005年7月,采用主动脉移位术连续纠治6例TGA/VSD/PS,其中男5例,女1例;年龄4~24个月,平均(11.33±6.86)个月;体重5.6~11.0kg,平均(8.43±2.19)kg。结果无手术死亡。体外循环转流112~204min,平均(153.83±33.70)min,主动脉阻断73~139min,平均(89.16±18.61)min。术后随访2~12个月。6例病儿活动良好,X线胸片示心影较术前略大,肺血增多;心电图示窦性心律,心脏超声检查示主动脉瓣反流轻微2例,轻度1例。射血分数0.74~0.86,短轴缩短率0.34~0.52。结论当前TGA/VSD/PS普遍采用Rastelli手术,但长期随访发现术后并发症较多,结果尚不理想。主动脉移位术重建左心室流出道和右心室流出道,避免了Rastelli手术后左心室流出道梗阻和心外管道梗阻的并发症。     

169例动脉调转手术治疗大动脉转位的早、中期结果

目的 探讨大动脉调转手术(ASO)治疗完全性大动脉转位(TGA)的早、中期结果,分析其随访死亡、术后主动脉瓣反流及肺动脉狭窄的危险因素.方法 2004年1月至2007年12月,169例行动脉调转术病儿入选,其中男129例,女40例,平均年龄(11.7±26.3)个月.病儿分两组:Ⅰ组为室间隔完整组(56例),Ⅱ组为室间隔缺损组(113例).所有术后生存病儿均进行超声随访,平均随访时间(27.7±14.6)个月.危险因素采用Logistic回归模型分析.结果 全组住院死亡19例(11.24%),两组间差异无统计学意义.随着整体治疗水平的提高,住院病死率由2004年的16.67%下降到2007年的3.92%.1、3及5年生存率分别为94.00%、91.33%及91.33%,两组间差异无统计学意义.Logistic回归分析发现,ASO术后随访死亡的危险因素为手术年龄大于6个月;术后主动脉瓣反流的危险因素为合并室间隔缺损、年龄大于6个月、术后新主动脉瓣Z值>1;术后肺动脉狭窄的危险因素为手术年龄小于1个月和肺动脉采用补片成形.结论 ASO手术具有良好的早、中期结果,是治疗完全性大动脉转位的理想术式.TGA病儿应该尽早手术治疗,手术年龄大于6个月是随访死亡及主动脉瓣反流的重要危险因素;新主动脉根部与主动脉远端不匹配是术后主动脉瓣反流的危险因素;病儿的生长发育与肺动脉成形材料无生长特性的矛盾是导致术后肺动脉狭窄的危险因素.     

完全性大动脉转位的个体化外科治疗

目的 研究不同类型完伞性大动脉转位(TGA)的个体化外科治疗策略及其效果.方法 1998年3月至2009年10月,共收治各类TGA患者127例(158例次),男性97例,女性30例.年龄生后4 h～17岁,平均(25±37)个月,其中<3个月56例66例次;体质量2.7～47.5 kg,平均(8±8)kg.初期手术行Glenn手术14例(其中3例行双侧Glenn手术),主-肺动脉分流术14例,肺动脉环缩术8例,房间隔缺损扩大+肺动脉环缩+主-肺动脉分流术15例.终期手术包括Seaning手术5例,一期Switch手术32例,二期Switch手术11例,Switch手术+室间隔缺损修补术20例,Switch手术+室间隔缺损镶嵌手术1例,Nikaidoh手术3例,Rastelli手术13例,Fontan手术18例,心内隧道等其他手术4例.结果 20例患者接受初期手术后现正在等待或已放弃二期手术,21例患者接受2期手术,5例接受3次及以上手术.66例次患者术后延迟关胸.采用腹膜透析9例,呼吸机使用时间2 h～16 d.全组早期死亡12例(病死率9.4%),死因包括术后低心排出最5例,肺动脉高压危象2例,术中出血2例,冠状动脉畸形1例,感染合并自发性肝破裂1例,肾功能衰竭1例.109例患者术后随访1个月～12年,6例失访,远期2例死亡.随访患者中,10例有不同程度的并发症,3例已再次手术,随访效果好.余存活病例,心功能正常,生长发育良好.结论 根据TGA患者解剖条件,采用个体化治疗策略,制定不同的手术方案,可以明显提高患者的手术成功率和远期生存率.     

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.     

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.