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135例动脉转位术患者围手术期监护

目的 总结动脉转位术（ASO）围手术期监护的经验，为提高其疗效提供借鉴。 方法 回顾性分析2000年1月至2009年6月广东省人民医院收治的135例完全型大动脉转位（TGA）患者行ASO的临床资料，男110例，女25例；手术时日龄1～160 d（27.24±26.49 d），体重2.4~5.0 kg（3.52±0.66 kg）。室间隔完整61例，室间隔缺损74例。术前动态监测血乳酸水平，纠正心功能不全，对98例（72.59%）明显低氧血症患者给予持续静脉注射前列腺素E1［PGE1，2~200 ng/（kg·min）］。手术在低温体外循环下施行，同期矫治合并畸形。术后严密监测凝血指标、左心功能，以间歇指令通气+容量保证通气（SIMV+VG）模式进行呼吸支持。22例术后应用PGE1［4~20 ng/（kg·min）］。 结果 全组体外循环时间36~423 min（189.20±59.94 min），主动脉阻断时间0~219 min（120.07±31.09 min），118例术后即时血氧饱和度95%~100%。术后机械通气时间24~792 h（168.24±154.80 h），总住院时间1~89 d（30.31±17.21 d）。生存108例，死亡27例（20.00%），主要死亡原因为低心排血量综合征。2001~2003年病死率为50.00%，2004~2006年为36.36%，2007~2009年后为8.43%，明显下降（χ2=18.323，Plt;0.001）。术后并发症：感染30例（22.22%），肺不张35例（25.93%）；心电图检查示：STT改变50例（37.04%），心律失常44例（32.59%）；超声心动图提示：吻合口狭窄36例（26.67%），肺动脉狭窄33例（24.44%）。 结论 ASO应用于TGA手术效果良好，除手术、麻醉操作外，术前维持内环境稳定、控制血乳酸水平，术后注意呼吸管理、维护左心功能是成功的关键。

Investigation of Risk Factors Influencing the Mortality of the Arterial Switch Operation

Objective To analyze the outcome of arterial switch operation （ASO） for surgical repair of complete transposition of the great arteries （TGA）, and to investigate the risk factors influencing the mortality of ASO. Methods The clinical data of patients suffered from TGA and treated with ASO from the January 2003 to December 2004, and the clinical records in hospital including eehoeardiogram and operation record were collected. The clinical data were analyzed by chi-squared test and logistic muhivariable regression analysis, including the age undergone operation, body weight, diagnosis, anatomic type of coronary artery, cardiopulmonary bypass time, aortic crossclamping time, circulation arrest time, assisted respiration time after operation, the delayed closure of sternum and so on. The risk factors influencing the early mortality of the ASO were analyzed. Results Sixty seven patients were operated with ASO, five patients died during the peri-operative period. The outcome of univariate analysis indicated that risk factors influencing the mortality of ASO included： age（P=0. 004）, body weight （P=0. 042）, anatomic type of coronary artery （P= 0. 006） and extracorporeal circulation time （P= 0. 048）, the length of the CICU stay（P= 0. 004） and the hospital stay（P=0. 007） after operation in the TGA/VSD patients were longer than those in TGA/ IVS patients. The logistic muhivariable regression analysis indicated that the age at operation （P= 0. 012）, coronary arteries anomaly （P = 0.001 ）and the longer cardiopulmonary bypass time （P = 0. 002） were correlated with the increase of death rate. Conclusion It could be good results for TGA patients who was repaired with ASO. The age at operation, the coronary arteries anomaly and the longer cardiopulmonary bypass time are the risk factors influencing the mortality.

Aortic Root Reconstruction with the Remaining Coronary Buttons in Arterial Switch Operation

Objective To summarize the clinical experiences of using the remaining coronary buttons to reconstruct the neoaortic root in the arterial switch operation (ASO) and discuss the clinical significance of preserving the morphology of aortic sinus in improving postoperative coronary artery perfusion. Methods From January 2003 to June 2009, 110 patients with transposition of great arteries (TGA) combined with ventricular septal defect (VSD ) or the Taussig Bing anomaly treated in our hospital were enrolled in this study. The patients were at the age between 2 days and 2 years averaged 91.1 days, and their body weight varied between 1.79 and 9.50 kg with an average weight of 4.70 kg. The patients were divided into two groups based on different surgical strategies. For group A (n=78), we reconstructed the neoaortic root with the remaining coronary buttons by shortening the diameter of the proximal neoaorta. While for group B (n=32), we excised the remaining coronary buttons with the aortopulmonary diameter mismatched. The risk factors of hospital mortality were analyzed with stepwise logistic regression. Results Twelve patients died early after operation with a death rate of 10.9% (12/110). Mortality in group A was significantly lower than that in group B ［6.4%(5/78) vs. 21.9%(7/32)，P= 0.019］. Clinical followup was completed in 72 survivors with the followup time of 3 months to 5 years after operation. Late death occurred in 3 patients including 1 in group A and 2 in group B. Five patients underwent reoperations. Univariate logistic regression analysis showed that risk factors for early postoperative death were TaussigBing anomaly (χ2=4.011,P=0.046), aortic arch anomaly (χ2=4.437,P=0.036), single coronary artery pattern (χ2=5.071,P=0.025) and patients in group B (χ2=5.584, P= 0.019). Multivariate analysis confirmed that the aortic arch anomaly (χ2= 5.681, P=0.010 ) and patients in group B (χ2=3.987, P=0.047 ) were two independent risk factors for early mortality after operation. Conclusion The modified technique which uses the remaining coronary buttons to reconstruct the neoaortic root can preserve the morphology of neoaortic root better. The special anatomical morphology of aortic sinus has close relation to the perfusion of coronary arteries. The lowering hospital mortality may be due to the better perfusion of the coronary arteries.

Prognosis of the complete transposition of great arteries with left ventricular outflow tract obstruction after intraventricular repair

Objective To compare the clinical characteristics and prognosis of patients who received two different intraventricular repair. Methods We retrospectively analyzed the clinical data of 24 complete transposition of the great arteries (TGA)/left ventricular outflow tract obstruction (LVOTO) patients who all received intraventricular repair. The patients were allocated into two groups including a REV group and a Rastelli group. There were 13 patients with 9 males and 4 females at median age of 25.2 (6, 72) months in the REV group. There were 11 patients with 10 males and 1 female at median age of 47.9 (14, 144) months in the Rastelli group. Results The age at operation (P=0.041), pulmonary valve Z value (P=0.002), and LVOT gradient (P=0.004), rate of multiphase operation between the REV group and the Rastelli group was statistically different. The mean follow-up time was 17.3 months. And during the follow-up, 1 patient had early mortality, 2 patients had early reintervention, 7 patients had postoperative RVOTO, and received Rastelli and larger VSD inner diameter were associated with postoperative RVOTO. Conclusion As the traditional surgery for TGA/LVOTO patients, the intraventricular repair has a low early mortality and low early reintervention. Modified REV is associated with postoperative peripheral pulmonary vein isolation (PVIS). Patients who received Rastelli operation and with larger VSD inner diameter are more likely to have postoperative RVOTO, but the reintervention for PVI and RVOTO during follow up is very low.

Anaesthesia Management in Rastelli Procedure for Translocation of the Great Arteries Associated with Ventricular Septal Defect and Pulmonary Stenosis

目的：采用Rastelli术治疗合并室间隔缺损、肺动脉狭窄、完全性大动脉转位病人，探讨围手术期麻醉管理特点。方法：选用大剂量芬太尼静脉复合麻醉方案，在深低温、低流量体外循环下实施Rastelli手术并进行多指标监测和临床观察。结果：麻醉经过平稳，围手术期患者生命体征稳定。结论：选择恰当的麻醉方案，有效的心脏保护,保持酸碱平衡和水、电解质稳定是成功进行麻醉管理的必要条件。

The operative strategy after palliative shunt for corrected transposition of great artery (cTGA) with left ventricular outflow tract obstruction and cardiac malpostion

ObjectiveTo explore the operative strategy after palliative shunt for correcting congenitally corrected transposition of great artery (cTGA) patients with left ventricular outflow tract obstruction (LVOTO) and cardiac malpostion.MethodsWe retrospectively analyzed the clinical data of 54 patients with onsecutive cTGA with LVOTO and cardiac malpositon from June 2011 to May 2019. The patients were devided into two groups. There were 24 patients (16 males and 8 females at mean age of 5.4±2.2 years) who underwent one and a half ventricle repair as a one and half ventricle group. And there were 30 patients (19 males and 11 females at age of 8.6±6.2 years) who underwent one ventricle repair operation as a one ventricle group. Follow-up data were collected by telephone interviews.ResultsThere was no statistical difference in systemic atrioventricular valve regurgitation and systemic ventricular ejection fraction between the two groups (P>0.05). Compared with one and a half ventricle group, the cardiopulmonary bypass time (CPB) time, mechanical ventilation time and intensive care unit stay were significant shorter than those in the one ventricle group (P<0.05), but prolonged pleural effusions developed more frequently in the one ventricle repair group (P<0.05). There was no in-hospital death but 1 follow-up death in each group. The follow-up time was 49 (17-38) months in the one and half ventricle group at follow-up rate of 93.9%, and 47 (12-85) months at follow-up rate at 90.9% in the one ventricle group. One and a half ventricle group had better systemic ventricular ejection fraction (EF) than that in the one ventricle repair group. And the rate of heart function (NYHA) class Ⅲ and class Ⅳ in one and a half ventricle group was lower than that in the ventricle group. No significant difference of survival and freedom from re-intervention probability between the two groups was found.ConclusionFor patients of correction of cTGA with LVOTO and cardiac malposition after palliative shunt, the one-and-a-half ventricular repair procedure is ideal operative strategy.

Pulmonary Artery Banding for Morphologic Left Ventricular Training in Corrected Transposition of the Great Arteries

Abstract：　Objective　To evaluate clinical outcomes of pulmonary artery banding for morphologic left ventricular training in corrected transposition of the great arteries.?Methods?A total of 89 patients with corrected transposition of the great arteries underwent surgical repair in Shanghai Children’s Medical Center from January 2007 to December 2011 year. Among them, 11 patients underwent pulmonary artery banding, whose clinical records were retrospectively analyzed. Except that one patient was 12 years, all other patients were 3 to 42 （16.40±11.67） months old and had a body weight of 6 to 32 （11.70±7.20）kg. All the patients were diagnosed by echocardiogram and angiocardiogram.?Results?There was no postoperative death after pulmonary artery banding in 11 patients. The pulmonary arterial pressure/systemic blood pressure　ratio （Pp/Ps） was 0.3 to 0.6 （0.44±0.09） preoperatively and 0.6 to 0.8 （0.70±0.04） postoperatively with statistical　difference （P＜0.01）. Tricuspid regurgitation was mild in 2 （18.2%） patients, moderate in 5 （45.4%）, severe in 4 （36.4%）preoperatively，and none in 2（18.2%）patients, mild in 7 （63.6%），and mild to moderate in 2 （18.2%）postoperatively. Five patients underwent staged double-switch operation after pulmonary artery banding at 15.20±8.31 months, and 1 patient died. The other 6 patients were followed up for 18.83±3.43 months, and echocardiogram showed tricuspid regurgitation as trivial in 2 （33.3%）, mild in 3 （50.0%）, and moderate in 1 （16.7%）patient.?Conclusions?In patients with corrected transposition of the great arteries, pulmonary artery banding is helpful to reduce tricuspid regurgitation, and morphologic left ventricle can be trained for staged double-switch operation with good clinical outcomes. It is important to follow up these patients regularly to evaluate their morphologic left ventricular function and tricuspid regurgitation after pulmonary artery banding.

主动脉弓离断合并完全性大动脉转位一例

一个半心室治疗的新领域——心脏位置异常的复杂矫正型大动脉转位矫治术

目的介绍一种治疗心脏位置异常的复杂矫正型大动脉转位（ cTGA）的新方法。方法中国医学科学院阜外心血管病医院 2011年采用“一个半心室修复”治疗心脏位置异常的 cTGA患者 1例，男，5岁 1个月。检索 PubMed、维普医药信息资源系统、万方数据知识服务平台等文献数据库，复习采用“一个半心室修复”治疗 cTGA的相关文献。结果我院 1例患者术后恢复良好，无明显手术相关并发症，术后第 11 d痊愈出院。出院时心脏超声心动图提示：肺静脉 -左心房及下腔静脉转流通畅，双侧上腔静脉 -肺动脉连接通畅，心室 -大动脉连接正常，心室流出道通畅，心室水平分流消失。共检索 3篇文献报道采用“一个半心室修复”策略治疗 cTGA，其中仅有 1篇文献报道了对心脏位置异常的复杂 cTGA采用一个半心室修复，平均随访 59.2个月，一个半心室修复延长了右心室 -肺动脉外管道的使用寿命，减少了肺静脉梗阻和心律失常、改善了心功能。结论对心脏位置异常的复杂 cTGA患者，一个半心室修复是一种安全有效的治疗策略。

Outcomes of Morphologic Left Ventricle Retraining Procedure for Congenitally Corrected Transposition of the Great Arteries

Abstract：　Objective?To evaluate clinical experiences and long-term outcome of morphologic left ventricle （mLV） retraining for congenitally corrected　transposition of the great arteries （cCTGA）.　Methods　From May 2005 to May 2011, 24 patients with cCTGA anomaly underwent left ventricle retraining by means of pulmonary artery banding in Fu Wai Hospital. There were 13 males and 11 females with their age of 0.17-22.00 （3.73±4.35） years and body weight of 5.10-61.00（15.71±10.95）kg. Major concomitant malformations included tricuspid valve insufficiency （TR）in 23 patients （mild in 11 patients, moderate in 7 patients, severe in 5 patients）, restrictive ventricular septal defect in 18 patients, atrial septal defect in 5 patients, patent foramen ovale in 5 patients, patent ductus arteriosus in 4 patients, mild pulmonary stenosis in 5 patients, and aortic coarctation in 1 patient. All the patients were preoperatively diagnosed by echocardiography, cardiovascular angiography or cardiac catheterization. The mLV end diastolic diameter （mLVEDD） was 8-32（21.56±6.60）mm, posterior wall thickness of mLV was 2-7 （4.29±1.52）mm , mLV to morphologic right ventricle （mRV） pressure ratio （mLV/mRV） was 0.12-0.65 （0.41±0.12）. Pulmonary artery banding operation was performed through upper partial sternotomy or median sternotomy without circulatory arrest.　Results　The mLV/mRV pressure ratio reached to 0.57-0.93 （0.76±0.10） under direct pressure monitoring after surgery. There was no in-hospital death in this group.　Echocardiography before discharge showed that the structure and function of the two ventricles were good, the interventricular septum moved partially towards mRV, mLVEDD was increased slightly, and there was a tendency of reduced TR. Postoperative follows-up was from 1 to 35 months, and there was no late death during follow-up. All the patients were in good general condition with stable vital signs and New York Heart Association （NYHA） classⅠ-Ⅱ. The mLVEDD was 14-40 （26.17±7.11） mm, posterior wall thickness of mLV was 4-9 （4.95±1.44）mm, mLV/mRV pressure ratio was 0.52-0.98 （0.72±0.16） , and TR was significantly decreased. Fourteen patients successfully underwent staged complete double-switch procedure.　Conclusion　Left ventricle retraining is a safe and effective method to train mLV for cCTGA patients. Pressure load and posterior wall thickness of mLV are increased, mLV cavity is dilated, and TR is significantly reduced after the surgery.