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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。

老年先天性矫正型大动脉转位行心瓣膜置换术一例

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。

Early and Mid-term Outcomes of Morphologic Tricuspid Valve Replacement with Preservation of Entire Valvular and Subvalvular Apparatus in Corrected Transposition of Great Arteries

Abstract： Objective To investigate the early and mid-term outcomes of morphologic tricuspid valve replacement by means of intravalvular implantation in corrected transposition of great arteries（cTGA）. Methods From January 2009 to January 2012，11 patients with cTGA were surgically treated in Fu Wai Hospital. There were 9 male patients and 2 female patients with their mean of age of（37.8±11.7）years and mean body weight of（73.0±11.3）kg. All the patients underwent morphologic tricuspid valve replacement with preservation of the entire valvular and subvalvular apparatus. Simultaneous surgical procedures included repair of ventricular septal defect in 2 patients，repair of atrial septal defect in 4 patients，pulmonary valvuloplasty in 1 patient，reconstruction of functional right ventricular outflow tract in 4 patients and repair of coronary-pulmonary artery fistula in 1 patient. Postoperative New York Heart Association （NYHA） classification, cardiothoracic ratio, morphological right ventricle ejection fraction, end-diastolic dimension of morphological right ventricle and left atrium were evaluated during follow-up. Results All the 11 patients were successfully surgically treated and followed up for an average duration of（13.0±10.6）months. There was no statistical difference between postoperative and preoperative average cardiothoracic ratio （0.54±0.06 vs. 0.57±0.09，t=1.581，P＞0.05），morphologic right ventricle ejection fraction （52.8%±9.0% vs. 54.9%±9.5%, t =0.712，P＞0.05），and end-diastolic dimension of . morphological right ventricle （54.3±7.5 mm vs. 56.9±9.2 mm，t =0.988，P＞0.05）. There was statistical difference between postoperative and preoperative average end-diastolic dimension of left atrium（42.1±8.9 mm vs. 53.4±11.1 mm，t =3.286，P＜0.05）and NYHA classification（Z = -2.640，P＜0.05）. Conclusion Intravalvular implantation of morphologic tricuspid prosthesis can protect the physiological structure of morphologic right ventricular and prevent furtherdamage to its function caused by morphologic tricuspid valve insufficiency. Postoperative dimension of morphologic left atrium and cardiac function are significantly improved. The early and mid-term outcomes are satisfactory．

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.

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.

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。

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.

Risk Factors for Mortality in the Arterial Switch Operation

Abstract： Objective To analyze risk factors for perioperative mortality in the arterial switch operation (ASO), in order to provide better operation and decrease the mortality rate. Methods We enrolled 208 ASO patients including 157 males and 51 females at Fu Wai Hospital between January 1, 2001 and December 31, 2007. The age ranged from 6 h to 17 years with the median age of 90 d and the weight ranged from 3 kg to 43 kg with the median weight of 5 kg. Among the patients, 127 had transposition of great artery (TGA) with ventricular septal defect (VSD), and 81 patients had TGA with intact ventricular septum (IVS) or with the diameter of VSD smaller than 5 mm. Coronary anatomy was normal (1LCX2R) in 151 patients and abnormal in the rest including 15 patients with single coronary artery, 6 with intramural and 36 with inverse coronary artery. Preoperative, perioperative and postoperative clinical data of all patients were collected to establish a database which was then analyzed by univariate analysis and multivariate logistic regression analysis to find out the risk factors formortality in ASO. Results There were 24 perioperative deaths (11.54%) in which 12 died of postoperative infection with multiple organ failure (MOF), 10 died of low cardiac output syndrome, 1 died of pulmonary hypertension, and 1 died of cerebral complications. Among them, 20 patients (18.30%) died in early years from 2001 to 2005, while only 4 (4.00%) died in the time period from 2006 to 2007, which was a significant decrease compared with the former period (Plt;0.05). The univariate analysis revealed that cardiopulmonary bypass (CPB) time was significantly longer in the death group than in the survival group（236±93 min vs. 198±50 min, P=0.002）, and occurrence of major coronary events (33.3% vs. 2.2%, P=0.000) and unusual coronary artery patterns（33.3% vs. 6.5%,P=0.000） were much more in the death group than in the survival group. Multivariate logistic regression analysis showed that early year of [CM(159mm]operation (OR=7.463, P=0.003), unusual coronary artery patterns (OR=6.303,P=0.005) and occurrence of majorcoronary events (OR=17.312, P=0.000) were independent predictors for perioperative mortality. Conclusion The ASO can be performed with low perioperative mortality in our hospital currently. Occurrence of major coronary events, unusual coronary artery patterns and year of surgery before 2006 are independent predictors for perioperative mortality.

体外膜式氧合在大动脉转位术后心室功能恢复与训练中的应用

摘要： 目的 评价体外膜式氧合（extracorporeal membrane oxygenation, ECMO）支持在婴幼儿大动脉错位（TGA）患者大动脉转位术（ASO）后心室功能恢复和适应性训练的临床结果及可行性。 方法 2005年1月至2008年8月，北京阜外心血管病医院7例TGA患者接受ASO后需要ECMO支持，其中男3例，女4例；年龄3周～14个月。ASO后心室不能适应新的血流动力学和/或合并心功能受损，采用静脉-动脉-ECMO辅助，占同期小儿先天性心脏病患者术后应用ECMO的36.84%（7/19）。插管途径为经胸右心房引流,升主动脉灌注；采用ECMO 系统为：Biomedicu(Medtronic)4例, Jostra 2例，Medos 1例；辅助流量20～100 ml/kg。 结果 7例患者平均转流时间174 h（64~266 h）,心室训练时间平均96 h。4例成功脱离ECMO，脱机率5714%（4/7）; 3例出院。死亡4例，其中3例不能脱离ECMO直接死亡，死亡原因为肾功能衰竭1例，出血1例，多器官功能衰竭1例；1例在脱离ECMO后6 d感染死亡。 结论 ECMO能为TGA患者ASO后心功能的恢复和左心室适应性训练提供有效的支持。