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重症法洛四联症根治术的围术期处理

目的探讨重症法洛四联症(TOF)围术期处理的方法与经验. 方法 1998年6月～2000年6月共纠治重症TOF 56例,行根治术43例,包括肺动脉闭锁7例,左右肺动脉发育较差26例,肺动脉瓣缺如2例,二期根治术8例. 结果残余室间隔缺损分流3例,残余肺动脉远端梗阻(压力阶差gt;30mmHg)11例;死亡2例(4.6%). 结论重症TOF因心脏畸形复杂、手术时间长、难度大和术后低心排血量发生率高,搞好围术期处理对提高重症TOF患者的术后生存率有重要意义.

Double-Switch术治疗矫正型大动脉转位

Progress on Diagnosis and Treatment of Congenital Tracheal Stenosis

Congenital tracheal stenosis (CTS) is a rare but potentially life-threatening disease which results in congnital airway lesion. CTS is often associated with cardiovascular anomalies and presented with a wide spectrum of symptoms. CTS has challenged pediatric surgeons for decades. Various classic approaches and new techniques, including computational fluid dynamics, tissue-engineering trachea, and 3D printing have been proposed for diagnosis and treatment of CTS. This review provides a snapshot of the main progress of diagnosis and treatment of CTS.

Application of Nikaidoh Operation to the Treatment of Transposition of Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis

Abstract: Objective To review the shortterm outcome of modified Nikaidoh operation, aortic translocation and biventricular outflow tract reconstruction as an alternative surgical procedure for the treatment of transposition of the great arteries with ventricular septal defect and pulmonary stenosis (TGA/VSD/PS). Methods Between January 2004 and December 2005, 8 consecutive patients had undergone Nikaidoh procedure for the treatment of TGA/VSD/PS at Shanghai Children’s Medical Center. All patients had ventriculoarterial discordance and atrioventricular concordance. Associated lesions included a straddling atrioventricular valve in one patient and hypoplastic left pulmonary artery. The median age at operation was 11.4±7.6months (4 to 29months). Weight of body was 8.0±1.9kg (5.2 to 11.0kg). No patient had previous palliative procedure. The surgical technique used was a modification of the Nikaidoh procedure. Results The median total cardiopulmonary bypass time was 176±50 minutes (range,112 to 250 minutes), and the median aortic crossclamp time was 101±27 minutes (range, 73 to 139minutes). The median length of stay in the intensive care unit was 12±9 days, with a median hospital stay of 19±12 days. There was 1 hospital death as a esult of severe left ventricle failure. There was no residual left ventricular outflow tract obstruction (LVOTO) and right ventricular outflow tract obstruction (RVOTO), but 3 patients with mild to moderate pulmonary regurgitation and 4 patients with moderate. At a median follow-up of 8.8 months (range, 3 to 18months), all patients were alive. All have the normal ventricular function. There were ejection fraction (EF) 0.64±0.02 and fractional shortening (FS) 0.33±0.02. None of the patients developed aortic insufficiency and progressed LVOTO or RVOTO. Conclusions Nikaidoh procedure is a valuable surgical option for TGA/VSD/PS in infant, especially in the presence of “inadequate anatomy” for a Rastelli repair. Big evidence and longer follow-up are required to fully assess the potential longterm benefits of this procedure compared with the Rastelli repair.

Patch Aortoplasty for Infant Coarctation of the Aorta with Hypoplastic Aortic Arch

Abstract: Objective To summarize the clinical experiences of resection with patch aortoplasty for infant coarctation of the aorta combined with aortic arch hypoplasia. Methods Between May 2007 and December 2009, 49 patients including 30 males and 19 females with coarctation with hypoplastic aortic arch underwent coarctation resection and patch aortoplasty in Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiaotong University. The age of the patients ranged from 23 days to 3 years and 1 month with thirtyfour patients under 6 months, ten between 6 months and 1 year old, and five more than 1 year old. The surgery under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion were performed in 31 cases and circulation arrest in 15 cases; under moderate hypothermia cardiopulmonary bypass in 3 cases. Pericardia patch was used in 31 cases, pulmonary autograft patch in 14 cases and xenograft pericardia patch in 4 cases. The associated intracardiac anomalies were repaired in the same stage. Results One case died from circulation failure during the perioperative period. The operative mortality was 204% (1/49). Low cardiac output syndrome and renal failure respectively occurred in 5 cases and 1 case who were cured afterwards by correspondent treatments. No residual obstruction was detected by echocardiography after the operation. Followup was carried out in fortyeight cases for a minimum of 4 months and a maximum of 3 years. Echocardiographic examination showed that the gradient through the aortic arch was more than 40 mm Hg and computed tomography showed recoarctation in 1 case who underwent reoperation eight months after the operation; the gradient was more than 20 mm Hg in 2 cases who were under continuous observation; all the rest cases had a fine aortic arch morphology and for these patients, the blood velocity at descending aortic arch was not obviously changed during the followup period compared with that right after operation, the computed tomography showed a normal aortic arch geometry. Left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before the operation without any aortic aneurysm detected. Conclusion Coarctation resection with patch aortoplasty is considered as an optimal surgical method for management of infant coarctation with hypoplastic aortic arch.

低温保存对山羊大动脉细胞形态的影响

目的 观察低温保存过程对山羊动脉细胞形态的影响. 方法 将12段山羊动脉按不同的降温速率分成4组,分别用透射电子显微镜和扫描电子显微镜观察冻存前后的动脉细胞形态. 结果 与正常血管组织相比,0.5 K/min组和0.1 K/min组虽细胞间连接紧密,但内皮细胞附着松散,出现大的空泡,有的平滑肌细胞肿胀,细胞质苍白多水,线粒体收缩;还有的虽平滑肌细胞轻微肿胀,但细胞器肿胀严重破坏. 5 K/min组和2 K/min组内皮细胞与基膜大量脱离,内皮细胞和平滑肌细胞破坏严重,出现核溶解及线粒体一些不能恢复的变化. 结论 经低温保存后的山羊主动脉与新鲜组织的区别明显,细胞组织形态被破坏,低温保存的降温速率越快,破坏越严重.

Longterm Followup of Left Ventricular Function and Aortic Valve Regurgitation after Rapid Twostage Arterial Switch Operation

Abstract： Objective To investigate the longterm complications and preventions of rapid twostage arterial switch operation through longterm follow-up. Methods We reviewed the clinical information of 21 patients of rapid twostage arterial switch operation from September 2002 to September 2007 in Shanghai Children’s Medical Center. Among them, there were 13 males and 8 females with an average age of 75 d (29-250 d) and an average weight of 5 kg (3.5-7.0 kg). The data of left ventricle training period and the data before and after the twostage arterial switch operation were analyzed, and the risk factors influencing the aortic valve regurgitation were analyzed by the logistic multivariable regression analysis. Results The late diameter of anastomosis of pulmonary and aortic artery were increased compared with those shortly after operation (0.96±0.30 cm vs. 0.81±0.28 cm, t=-1.183,P=0.262; 1.06±0.25 cm vs. 0.09±0.21 cm, t=-1.833,P=0.094), but there was no significant difference. The late velocity of blood flow across the anastomoses was not accelerated, which indicated no obstruction. The late heart function was better than that shortly after operation, while there was no significant difference between left ventricular ejection fraction(LVEF) during these two periods (62.88%±7.28% vs. 67.92%±7.83%,t=1.362,P=0.202). The late left ventricular end diastolic dimension(LVDd) was significantly different from that shortly after operation (2.16±0.30 cm vs.2.92±0.60 cm，t=-5.281，P=0.003). Compared with earlier period after operation, the thickness of left ventricular posterior wall thickness（LVPWT）was also increased (0.39±0.12 cm vs. 0.36±0.10 cm,t=0.700,P=0.500), but there was no significant difference. The postoperative aortic valve regurgitation was worsened in 4 patients (30.77%, 4/13), not changed in 7 patients and alleviated in 2 patients compared with that before operation. There was no severe regurgitations during the followup. The logistic regression analysis showed that the small preoperative diameter ratio of aortic valve to pulmonary valve and long follow-up time were two risk factors for the [CM(159mm]aggravation of aortic regurgitation. Conclusion There is a relatively high aortic regurgitation rate after rapid two stage arterial switch operation, but there is no later death or reoperation and the survival conditions are satisfactory. All patients must be followed up periodically to check the anastomosis of pulmonary and aortic arteries and the aortic valve.

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.

Application of Modified Lateral Tunnel Fontan Procedure on Complex Congenital Heart Disease in Children

Objective To summarize the treatment experiences of Modified lateral tunnel(LT) Fontan operation on complex congenital heart disease in children and investigate the advantages of this operation. Methods From March 1999 to August 2008, 86 patients with cynosis complex congenital heart disease underwent LT Fontan operation in our hospital. There were 47 male and 39 female aged 1.9-11.5 years with a mean age of 4.7 years and weighed 8.6-52.0 kg with a mean weight of 17.0 kg. There were 33 cases with asplenia syndrome, 17 cases with polysplenia syndrome, 11 cases with tricuspid atresia(TA), 11 cases with double outlet right ventricle(DORV) of atrioventricular discordance, 8 cases with complete transposition of great arteries(D-TGA) complicated with pulmonary stenosis, 5 cases with corrected transposition of great arteries(cTGA) and 1 case with Ebstein’s anomaly. Unilateral superior bidirectional superior cavopulmonary anastomosis(BSCPA), bilateral bidirectional superior cavopulmonary anastomosis and hemiFontan opertion were done before operatipon. The time between two operations was 0.7-7.8 years（3.6±2.9 years）. LT Fontan operation(LT-group, 47cases) and Modified LT Fontan operation(M-LT group, 39cases) were used in operation to drain blood from inferior vena cava to right pulmonary artery. Partly completed secondstage M-LT Fontan operation. Results There were 7 deaths in two groups(9%), 5 in LT group and 2 in M-LT group. There was no statistical significance（χ2=0.865，P=0.448）. In stagemodified LT Fontan operation, there were significantly more cases who had BSCPA operation preoperatively in MLT group than that in LT group. Twentytwo cases had low cardiac output syndrome after operation, 13 cases underwent peritoneal dialysis because of renal dysfunction, and theirurine volume recovered after 2-5 days’ dialysis. There were significantly more cases who had arrhythmia in LT group than that in M-LT group（χ2=8.763，P=0.003）. The time of chest drainage was longer in LT group than that in M-LT group（t=2.970，P=0.003）. The follow-up time was 3 months8 years. No death was found. In M-LT group 33（85%） cases were followed up and in LT group 39（83%）cases were followed up. No severe complication was found. Patients’ activity ability improved significantly. Conclusion The M-LT Fontan operation is an advanced operation to improve the success rate of operation and reduce postoperative complications.

同种带瓣大动脉重建右心室流出道

摘要目的 应用自行采集和冷冻保存的同种带瓣大动脉(VHC)完成先天性心脏病右心室流出道的重建，并观察其疗效及存在的问题。方法 用VHC材料治疗先天性心脏病95例，90例手术根治，5例行VHC右心室流出道与肺动脉连接姑息手术。结果 院内死亡13例；术后随访68例，死亡2例，均为感染。66例长期生存者中25例胸部X线片示VHC有钙化，多为主动脉材料，仅5例有轻中度压力阶差(35～60mmHg)。结论 VHC可广泛用于治疗复杂先天性心脏病。程序降温、超低温保存和两步化冻是VHC使用质量的保证。VHC的长期通畅比合成管道好，随植入时间延长钙化率增加，肺动脉VHC优于主动脉。有肺动脉高压者宜尽早手术。为了预防VHC植入后感染，应重视、改进其收集和保存的方法。