婴儿先天性心脏病体外循环术后的治疗

Objective To study the treatment and outcome in infants with congenital heart disease after cardiopulmonary bypass. Methods The clinical data of 217 infants with congenital heart disease after cardiopulmonary bypass in Shengjing Hospital from Jan 2005 to Nov 2010 were retrospectively reviewed. Results Eleven infants died and the mortality was 5. 1% ( 11/217 ). Four infants died of severe low cardiac output syndrome,2 died of pulmonary hypertension crisis,2 died of low cardiac output syndrome complicated with acute renal insufficiency, 1 died of severe infection, 1 died of severe hypoxemia,and 1 died of chronic pericardial tamponade. The postoperation length of hospital stay was 7 ～ 52 d, 14 d in average. Conclusion With the improvement of surgical techniques,cardiac surgery with cardiopulmonary bypass on infants can result in good clinical effect. Active and effective treatment can reduce the perioperative mortality after cardiopulmonary bypass.     

完全性大动脉转位患儿外科术后早期并发症和近期随访

Objective To present the short-term outcomes of the patients with transposition of the great arteries after repair. Methods Between 2008 and 2009,71 patients with transposition of the great arteries (TGA) underwent corrective surgeries at this center. The 71 TGA patients were divided into TGA/IVS group whose ventricle septum was intact, and TGA/VSD group who complicated with ventricle septum defect. Their clinical data and postoperative short-term outcomes were analyzed and compared. Results The postoperative complications included 7 (9. 9% ) patients presented with low cardiac output syndrome, 12 ( 16. 9% ) with pulmonary stenosis, 21 ( 29. 6% ) with arrhythmia, 6 (8. 5%) with diaphragmatic paralysis, 6 (8. 5%) with airway abnormalities, and 13 ( 18. 3% ) with bacteria in sputum culture. Three patients (4. 2%) died after surgery. Fifty four out of the 68 patients were followed up for an average period of 12. 0 ± 7. 4 months. Four (7. 4%) patients died during follow-up. Three patients (5. 6%) underwent interventional therapies for pulmonary stenosis. The survival rate of 57 patients was 87. 7% at the end of the first postoperative year. No differences of the postoperative complications and survival rate were noted between the TGA/IVS and TGA/VSD group.Conclusions The postoperative short-term outcomes of the patients with transposition of the great arteries are good.     

功能性单心室的外科治疗及近期疗效

目的 总结功能性单心室的外科治疗经验.方法 回顾性分析我科2003年12月至2010年7月59例功能性单心室患儿,对其手术时机,手术方式及手术效果进行分析,并用Logistic回归分析Glenn手术的高危因素.结果 死亡8例(病死率13.56%),其中4例为Glenn术后死于低心排和多器官功能衰竭,2例为Banding术后死于低心排,1例为TCPC术后死于多器官功能衰竭,1例为B-T分流术后死于低心排.Glenn术后的主要并发症为上腔静脉梗阻综合症和低心排,Fontan术后的主要并发症是房性心律失常和胸腔积液.Logistic回归分析得出房室瓣反流是Glenn术后患儿死亡的高危因素.结论 功能性单心室心内畸形复杂,需根据肺血流的情况及早进行姑息手术,选择合适的术式.改良Fontan是进行生理矫正的理想术式,保留肺动脉前向血流的Glenn手术是保证患儿顺利进行Fontan术的有效方法,但需根据肺动脉狭窄的程度进行处理,避免上腔静脉综合症的发生.

Abstract：

Objective To retrospectively analyze the outcome of surgical treatment of patients with functional single ventricle. Methods Betveen December 2003 and July 2010, 59 patients with functional single ventricle underwent corrective surgeries, and were followed up at this center. Their median age was 3. 0 months old at pulmonary artery band surgery (PAB), 13. 5 months at bidirectional Glenn procedure (BDG), and 11 years old at Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, age at surgery, and type of the surgical procedure were retrospectively analyzed. Logistic regression analysis was also performed to determine the risk factors associated with Glenn. Results Duration of mechanical ventilation was 30. 4 ± 19. 7 hours. Length of stay in ICU was 4. 3 ± 1.6 days. Length of hospital stay was 34. 8 ± 15. 9 d.SpC2 increased from 75 ± 6. 97% to 88 ± 6. 28% after operation. Exercise tolerance was also improved remarkably. Eight deaths were noted after surgery (13. 56%). The leading complications after Glenn surgery were superior vena cava syndrome and low cardiac output syndrome. Complications after Fontan surgery were atrial tachycardia and pleural effusions. Logistic regression analysis suggested atrioventricular valve regurgitation was an important risk factor for the death of the patients had Glenn surgery. Conclusions The clinical outcome of surgical treatment of patients with functional single ventricle is satisfied. The surgical strategy should be made according to anatomic abnormality of the functional single ventricle and pulmonary circulation. Modified Fontan operations can become an alternative therapy for patients who are not suitable for double ventricle correction. Glenn with antegrade pulmonary blood flow may be a temporary palliation prior to Fontan surgery, but the pulmonary blood flow should be controlled to avoid the occurrence of superior vena cava syndrome.     

室间隔缺损术后反应性肺高压危险因素及疗效分析

目的 探求室间隔缺损、肺高压患儿术后反应性肺高压的发生率、危险因素.评估术后反应性肺高压的治疗效果.方法 对1991年至2006期间术前诊断室间隔缺损、肺高压在我科接受室间隔缺损修补术患儿的客观病史资料进行回顾性研究.搜集患儿围术期客观指标进行统计和分析,评估术后早期疗效.探求术后反应性肺高压的危险因素.结果 16年间共2 141例室间隔缺损合并肺高压的患儿纳入本课题.术后住院早期死亡20例,住院晚期死亡3例,术后病情平稳出院2 118例.术后反应性肺高压发生率为6.1%,术后肺高压危象的发生率为2.1%、死亡率为2.3%.术中体外循环时间≥90min(P＜0.01)、主动脉阻断时间≥75min(P＜0.01)、术后残余分流(P＜0.01)是发生术后反应性肺高压的相关危险因素.结论 室间隔缺损、肺高压的患儿术后反应性肺高压的独立危险因素有:术前年龄、Pp/Ps、存在充血性心力衰竭、术后二尖瓣反流中度以上.术后反应性肺高压的患儿更容易依赖儿茶酚胺类药物,左房途径输入儿茶酚胺类药物能增加其强心效果.

Abstract：

Objective To study the risk factors and management for reactive pulmonary hypertension (RPH) after corrective surgery of ventricular septal defect (VSD) in children. Methods From 1991 to 2006, 2141 patients who underwent corrective surgery for VSD were recruited in this study.The patients' clinical data, including patient's gender, age, diagnosis, the ratio of pulmonary pressure to systolic pressure (Pp/Ps), the time of cardiopulmonary bypass (CPB), postoperative RPH, pulmonary hypertension crisis (PHC) and other complications, and the treatment of RPH, were retrospectively analyzed. The risk factors of postoperative RPH were statistically analyzed. The outcomes of RPH treatment were also evaluated. Results Twenty patients died during the early in-hospital period, 3 patients died during the late in-hospital period, and the others were recovered after surgery. The mobility of postoperative RPH and PHC were 6. 12% and 2. 06%, respectively. The overall mortality of the patients was 2. 27%. The patients had CPB≥90 min, aortic clamp time≥75 min, postoperative catecholamines treatment, and residual intracardiac shunting after surgery were more likely to develop postoperative RPH. Conclusions The independent risk factors of postoperative RPH include patients'Pp/Ps ratio, age, congestive heart failure before surgery, moderate mitral regurgitation after surgery.Catecholamines treatment could effectively improve right ventricular function in the patients with RPH.     

与先天性心脏病相关的NUSS术

目的 探讨同期治疗合并先天性心脏病(先心)的漏斗胸及先心术后漏斗胸应用NUSS术的方法及可行性.方法 统计我院2006年7月至2010年6月与先心相关的漏斗胸15例,A组8例合并先心的漏斗胸患儿在同期行心脏手术和NUSS术,其中男5例,女3例,年龄4岁～13岁4个月(平均6岁1个月).其中4例行室间隔缺损经胸伞封术,2例行房间隔缺损经胸伞封术,1例在体外循环下行室间隔缺损修补术,1例在体外循环下行右室双腔矫治及室间隔缺损修补术.B组7例均为先心术后的漏斗胸行NUSS术,其中男5例,女2例,先心手术年龄6个月～3岁10个月(平均1岁9个月),NUSS术年龄4岁7个月～8岁(平均6岁2个月),6例为体外循环下室间隔缺损修补术后,1例为体外循环下法乐四联症矫治术后.术后常规放置有心包纵隔和/或胸腔引流管.结果 所有患儿手术顺利,A组术后5～14 h拔除气管插管,平均(8.8±2.6)h.B组术后4～8 h拔除气管插管,平均(5.9±1.2)h,48～72 h拔除心包纵隔或胸腔引流管.无手术死亡、大出血及胸腔脏器损伤等并发症.术后检查先心矫治效果良好,肺复张良好.术后两组各出现1例切口延期愈合,经治疗后,均顺利出院.随访6个月～4年效果良好.结论 NUSS术用于治疗先心术后漏斗胸以及同期治疗合并先心的漏斗胸安全可行,可有效降低或避免二次手术的难度及风险.

Abstract：

Objective To investigate the clinical outcomes of combining corrective surgery for congenital heart disease (CHD) and NUSS surgery for pectus excavatum (PE) as one-staged operation. Methods From July 2006 to June 2010, 15 children with CHD associated with PE were recruited in this study, and divided into two groups: group A underwent one-staged and group B with twostaged operation. Group A had 8 patients including 5 males and 3 females, aging from 4 to 13.4 years (mean, 6 years and 1 month). Among the 8 patients, 6 with ventricular septal defect (VSD) and 2with atrial septal defect (ASD) underwent interventional or open repair. After the corrective surgery for their CHD, the 8 patients underwent NUSS procedure for pectus excavatum. The group B had 7patients, including 5 males and 2 females. Among them, 6 had VSD and 1 had tetralogy of fallot (TOF). At the first stage, the patients underwent corrective surgery to repair VSD and TOF under CPB. At the second stage, the patients were performed NUSS surgery to correct PE. Their ages at surgery were 4 years and 7 months to 8 years old (mean, 6 years and 2 months). Results All operations were finished successfully. The endotracheal tube was removed 5 to 14 hours after surgery on Group A patients (mean, 8.75 ± 2.59 h), and 4 to 8 hours on Group B patients (mean, 5.86 ±1. 24 h). The drainage tubes of pericardium, mediastinal or chest were removed 48 to 72 h later after surgery. The patients were followed up for 6 months to 4 years. No surgery-related death, hemorrhage, thoracic organ dysfunction and other severe complications were noted. Delayed wound healing was observed on 1 patient in each group. Others recovered from surgery and were discharged from the hospital. Conclusions It is safe to combine corrective surgery for CHD and NUSS procedure for PE as one stage operation.     

小儿感染性心内膜炎治疗的现状

Objective During recent years several changes have occurred in the clinical characteristics of infective endocarditis (IE) which has made a new challenge in the management of this disease. This study aimed to understand current practice pattern in the management of pediatric IE in China. Methods This retrospective, multicenter study was conducted in 13 hospitals. Clinical data of 268 patients diagnosed as IE according to the new IE criteria (trial) between 2000 and 2006 were analysed, focusing particularly on management and outcome of patients. The mean age of patients was 8.94 years (18 d-18 years). Results Except for one patient who died after admission without treatment, 56 antimicrobial agents were used in the management of this disease in the 267 patients, including cephalosporin group (15), penicillin group (8), beta-lactamase inhibitor combination (8), aminoglycosides (4), glycopeptide agents (3) etc. The most commonly used antibiotics were as follows: penicillin G (125 cases/times), cefotaxime (113), vancomycin (78), ceftriaxone (73), ampicillin (66), cefuroxime (56), piracillin (48), amikacin (39) etc. For management of this disease, only one antibiotic agent was used in 33 (12.3%) patients, two antibiotic agents in 83 (31.1%) patients, 3 antibiotic agents in 44 (16.5 %) patients, 4 antibiotic agents in 57(21.3%) patients, 5 antibiotic agents in 25(9.4%) patients, 6 or more antibiotic agents in 25 (9.4%) patients. The most commonly used antibiotic agents in patients with streptococci detected in blood culture were penicillin G, cephalosporins, vancomycin, beta-lactamase inhibitor combination, and aminoglycoside, in patients with staphylococcus detected in blood culture were cephalosporins, oxicillin, vancomycin, aminoglycoside, and quinolones. Duration of antibiotic treatment was from 1 day to 98 days, less than 2 weeks in 19 (7%) patients, 2 weeks to less than 4 weeks in 74 (27.7%) patients, 4-6 weeks in 122 (45.7%) patients, more than 6 weeks in 52 (19.4%) patients; 123 patients simultaneously underwent surgical management (for removal of vegetations and intracardiac defects or residual shunt repair 105, and valve repair 8, valve replacement 6, intracardiac defect repair 4) .Of the 268 patients, 186 patients were cured, 4 patients were referred to surgery, 18 patients died and 60 patients refused medical advice. In antibiotics and surgical treatment group (123 patients), 111 patients were cured, 4 patients were referred to surgery, 5 patients died, 3 patients refused medical advice, in antibiotics treatment group (145 patients), 75 patients were cured, 13 patients died, 57 patients refused medical advice. The outcomes were significantly different between the two groups (χ2=61.7, P=0.000). The results of multivariate logistic regression analysis showed that Staphylococcus aureus as a pathogen (χ2=4.40,P=0.036,OR=9.78,95% CI 1.16-82.26), children with repaired congenital heart disease (χ2=9.4,P=0.002,OR=9.8,95% CI 2.28-42.16), and complicated with heart failure (χ2=10.36,P=0.001, OR=0.075,95% CI 0.16-0.36) were risk factors related to death. Conclusion This study revealed the current status in the management of pediatric IE in China. Wide range antibiotic agents and diverse regimens are used to manage children with IE. For improving management of pediatric IE, there is an urgent need for guidelines or recommendations or consensus for management of pediatric IE stipulated by multidiscipline specialists, and randomized controlled clinical trials are required to provide evidences.     

Factors controlling fetal echocardiography determine the diagnostic accuracy of isolated ventricular septal defect

Background:Fetal echocardiography (FECG) is a key screening tool for prenatal cardiac abnormalities.Herein,we examined the ultrasonic factors determining prenatal ultrasonic diagnosis of isolated ventricular septal defect (IVSD).Methods:The diagnostic role of ultrasonic factors was investigated in patients in middle or late pregnancy,diagnosed with IVSD by FECG and confirmed using postnatal echocardiography.Results:One hundred and six patients with IVSD were enrolled;the majority had perimembranous VSD.The combined imaging mode of 2 dimentionalechocardiography (2DE) and color doppler flow imaging (CDFI) showed the highest rate (56.6％) of IVSD detection,while CDFI was more efficient than 2DE (32.1％ vs.11.3％).The single-view mode was more efficient than multiple-view mode (75.5％ vs.24.5％).The highest efficient mode to detect IVSD was achieved using combined imaging mode on the single view of the left ventricular outflow tract view (LVOTV) (28.3％).FECG correctly classified 71.7％ of fetal IVSD.There was a significant difference of accuracy rate in classifying IVSD among the three different imaging modes (x2=7.141,P＜0.05).The single imaging mode of CDFI and the mode of CDFI combined with 2DE correctly classified 75.9％ and 75.0％ of fetal IVSD,respectively.LVOTV was the most accurate view of fetal IVSD classification (85.2％;x2=15.782,P＜0.05).There was no difference in accuracies of IVSD classification among multiple-view modes (x2=2.343,P＞0.05) or between single-view mode and multiple-view mode （x2=0.32,P＞0.05).Conclusion:Single LVOTV in CDFI or CDFI combined with 2DE of FECG were the most effective diagnostic modes for fetal IVSD diagnosis.     

应用儿童终末期肝病模型评分预测Kasai术后联合激素治疗胆道闭锁的短期预后

Objective To assess the effects of steroid therapy on the biliary atresia patients after Kasai portoenterostomy using pediatric of end-stage liver disease (PELD) scoring system. Methods Eighty patients with biliary atresia were enrolled in this study. The patients were randomly grouped into Kasai surgery group and combined therapy group. The patients of Kasai surgery group were performed Kasai portoenterostomy. The patients in combined therapy group underwent Kasai portoenterostomy and steroid therapy after surgery. Each patient's PELD score was calculated. Biochemical parameters,mortality,and PELD scores of two groups were compared. Results In the patients with a PELD less than 16, the levels of total bilirubin (TBIL), prothrombin time-international normalized ratio (INR) and PELD score of combined therapy patients were 23. 3 ± 1. 1 mg/L,2. 31 ± 0. 24,and 10. 6 ± 2.3, respectively; which were significantly lower than these parameters of the Kasai surgery group patients (TBIL,28. 9 ± 2. 1 mg/dL; INR,2. 63 ± 0. 18; PELD, 13. 2 ± 2.7). The combined therapy patients' albumin was also significantly higher than that of Kasai surgery group patients (P＜0. 05). The same results were also seen among the patients with a PELD between 16 and 28. Among the patients with a PELD higher than 28,only the difference of TBIL between the Kasai surgery group and combined therapy group was found. The mortality of the combined therapy patients was significantly lower than that of Kasai surgery group patients among the patients whose PELD ＜28. However, in the patients with a PELD≥28,no difference of the mortality was found between the two groups. Conclusions In the BA patients with PELD less than 27, Kasai portoenterostomy plus postoperative steroid therapy can improve the outcomes and prognosis.     

婴儿先天性心脏病体外循环术后的治疗

目的 探讨婴儿先天性心脏病体外循环术后的治疗及转归.方法 对我院2005年1月至2010年11月经体外循环治疗的217例婴儿的临床资料进行回顾性分析.结果 全组术后死亡11例,住院病死率为5.1%(11/217).其中因重症低心排出量综合征死亡4例,肺动脉高压危象死亡2例,低心排出量综合征合并急性肾功能不全死亡2例,重症感染死亡1例,严重低氧血症死亡1例,慢性心包填塞导致心跳骤停死亡1例.术后住院时间7～52 d,平均14 d.结论 随着外科治疗技术的提高,婴儿体外循环下心脏手术治疗可以取得良好的治疗效果.体外循环术后采取积极有效的治疗是降低围手术期病死率的关键.

Abstract：

Objective To study the treatment and outcome in infants with congenital heart disease after cardiopulmonary bypass. Methods The clinical data of 217 infants with congenital heart disease after cardiopulmonary bypass in Shengjing Hospital from Jan 2005 to Nov 2010 were retrospectively reviewed. Results Eleven infants died and the mortality was 5. 1% ( 11/217 ). Four infants died of severe low cardiac output syndrome,2 died of pulmonary hypertension crisis,2 died of low cardiac output syndrome complicated with acute renal insufficiency, 1 died of severe infection, 1 died of severe hypoxemia,and 1 died of chronic pericardial tamponade. The postoperation length of hospital stay was 7 ～ 52 d, 14 d in average. Conclusion With the improvement of surgical techniques,cardiac surgery with cardiopulmonary bypass on infants can result in good clinical effect. Active and effective treatment can reduce the perioperative mortality after cardiopulmonary bypass.     

小儿肥厚型梗阻性心肌病的非药物治疗

目的 总结儿童肥厚型梗阻性心肌病(HOCM)非药物治疗的效果.方法 2008年11月至2010年6月,4例在本院住院的药物治疗无效的HOCM患儿,术前跨左室流出道压差(LVOTG)分别为60、147、58、114 mm Hg(1 mm Hg=0.133 kPa).例1、2采用经皮腔内室间隔心肌消融术(PTSMA)治疗,例3、4采用室间隔心肌切除术(SM)治疗.术后随访时间1～18(9.3±8.1)个月.结果 4例患儿症状均明显减轻;除例2外,其余3例心功能均明显改善.超声心动图显示4例患儿术后即刻左室流出道梗阻(LVOTO)均明显缓解;随访中,除例2的LVOTG逐渐反弹外,其余3例均持续缓解.所有病例均未发生严重并发症.例1行PTSMA术后出现一过性完全性右束支传导阻滞(CRBBB),1个月后转为心室内阻滞;例3和例4行SM术后出现完全性左束支传导阻滞(CLBBB),其中例4术后1个月后变为心室内阻滞,例3的CLBBB持续存在.结论 初步经验表明,对于药物治疗无效、有明显症状的HOCM儿童,应用SM和PTSMA治疗安全、有效,近期疗效理想,远期疗效及并发症需要进一步随访研究.

Abstract：

Objective To retrospectively summarize the effect of non-medical therapies for pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM).Methods From Nov.2008 to Jun.2010,4 children with drug-refractory HOCM were admitted to our hospital.Their ages were 14, 7, 9 and 6 years old, respectively.Their body weights were 38, 17, 21.5 and 17 kg, respectively.Before operation, the pressure gradients over left ventricular outflow tract (LVOTG) were 60, 147, 58 and 114 mm Hg ( 1 mm Hg =0.133 kPa), respectively.And mitral regurgitation (MR) areas were 2.2, 7.3 cm2 and 2.9cm2, respectively, except that it was trivial in one case. Percutaneous transluminal septal myocardial ablation (PTSMA) was performed in case I and 2.Septal myectomy (SM) was performed in case 3 and 4.Follow-up was first performed right after operation or before discharge, then 1 month, 3 months, 6 months,and 12 months after operation, and then once a year.The follow-up period was 1-18 (9.3 ± 8.1 ) months.Results All patients experienced relieved symptoms.Three of them had their NYHA functional class improved except case 2.Echocardiography revealed that LVOTGs right after operations were 38, 79, 20 and 0 mm Hg, respectively, suggesting significant improvement of left ventricular outflow tract obstruction (LVOTO) in all patients.During follow-up, case 2 suffered from recurrence of LVOTO, while the other 3cases showed sustained relief.In the last follow-up, the LVOTGs of the four patients were 19, 168, 16 and 0 mm Hg, respectively.Echocardiography also revealed that MRs of all patients were significantly reduced,even in case 2 whose LVOTG rebounded, with no recurrence during follow-up.Severe complications were absent, such as ventricular septum perforation, cardiac tamponade, ventricular tachycardia or ventricular fibrillation.No one suffered from complete heart block. Transient complete right bundle branch block (CRBBB) was observed in case 1 after PTSMA and converted to intraventricular block after 1 month.Complete left bundle branch block (CLBBB) was present in both case 3 and 4, who received SM.In case 4, it converted to intraventricular block after 1 month while in case 3 CLBBB persisted.Conclusions The initial experience showed that PTSMA and SM were safe and effective for drug-refractory symptomatic HOCM children, with satisfactory short-term results.Further studies are needed to evaluate the long-term results and complications.     

Closure of ventricular septal defect through the pulmonary artery

Summary A transpulmonary arterial approach to the closure of a high ventricular septal defect (VSD) has been used, between 1978 and 1982, in eight patients. The reasons were ease of access and the wish to overcome the problems associated with right ventriculotomy. The patients' ages ranged from three weeks to 15 months, their weight from 2.9 kg to 9 kg. The approach was used both when the VSD was an isolated anomaly and when there were major associated defects. It is in this latter group, four with aortic arch anomalies, two with additional double outlet right ventricle (DORV), that avoidance of ventriculotomy was most helpful. It was especially important in the two patients with DORV and a perimembranous, outlet subpulmonary VSD, where it was possible to close off the left ventricular outflow tract and pulmonary valve using a patch, without opening the right ventricle, which was subsequently to become the systemic ventricle. This technique obviates the need for ventriculotomy in the closure of some perimembranous outlet and doubly committed subarterial VSDs, and is the approach of choice for the closure of a perimembranous, outlet, subpulmonary VSD in DORV.     

婴儿法洛四联症术后并发症处理及一年随访

目的 总结婴儿法洛四联症(TOF)术后早期并发症的处理及术后1年的随访结果 .方法 收集2004年1月至2007年3月安徽省立儿童医院婴儿TOF术后35例,年龄4～12个月.全组均在全身麻醉、低温体外循环下进行手术,其中一期根治30例,姑息术5例.结果 10例出现术后早期并发症,合并低心排血量综合征6例,严重心律失常2例,灌注肺2例.随访1年,发现右室流出道严重残余梗阻2例,残余漏1例.围手术期死亡2例,死因均为低心排血量综合征,术后4个月死亡1例,死因为单肺灌注,病死率8.6%.31例患儿心功能评级为纽约心脏协会(NYHA)分级Ⅰ或Ⅱ级.结论 充分的术前准备、合理的手术方法 和心肌保护、及早发现和正确处理并发症、对少尿患儿尽早行腹膜透析,可有效地降低婴儿TOF围手术期的病死率.对于有症状的TOF病例,应在婴儿期行根治手术,能收到满意的效果.     

Arterial Switch

A relatively large spectra of anatomic variations are found within the unifying features of discordant ventriculoarterial connections. Variants that lend themselves to anatomic repair by the arterial switch operation are discussed, these include transposition of the great arteries with intact ventricular septum (TGA IVS), TGA associated with a ventricular septal defect (TGA VSD), double-outlet right ventricle with subpulmonary VSD (DORV VSD), and TGA or DORV with VSD associated with coarctation. Double discordance with VSD, which is currently treated by double switch or Rastelli and atrial switch and which probably represents, in our department, the only remaining indication for atrial switch, is not discussed. Also, we exclude TGA associated with pulmonary stenosis, which is treated by Rastelli or REV operation.     

Anatomic evidence of spontaneous intrauterine closure of a ventricular septal defect

Summary An infant, born to a mother on carbamazepine monotherapy, had a complex cardiac anomaly, consisting of double-outlet right ventricle (DORV), right-sided aorta, pulmonary artery hypoplasia, left ventricular endocardial fibroelastosis, and anatomic evidence of a spontaneously closed muscular ventricular septal defect (VSD). The last finding is one which, to the best of our knowledge, has never been illustrated before.     

婴儿先天性心脏病1387例外科治疗结果分析

目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.     

单源化手术治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管

目的 探讨肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的手术方式,评价其临床疗效.方法 2008年7月至2010年8月本院采用单源化手术治疗肺动脉闭锁（PA）合并室间隔缺损（VSD）及重要主肺动脉侧支血管（MAPCAs）患儿8例.男3例,女5例,年龄4～72个月,中位年龄24个月,体重7.5～15.5 kg,平均体重（11.2±2.4）kg.7例采取分期于术治疗,1例采取一期根治手术.结果 全组患儿均存活.随诊6～20个月,1例一期根治手术患儿痊愈;7例分期手术患儿,临床紫绀症状均好转.其中3例肺动脉明显发育,进一步行二期根治手术治愈.结论 单源化手术是治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的有效方法,分期手术可取得良好的临床疗效.     

手术治疗婴幼儿法洛四联症83例

目的 总结婴幼儿法洛四联症（TOF）的外科手术情况,初步探讨手术时机、术式及与近期疗效的关系,以提高手术矫治效果.方法 2003年8月至2008年5月本院共手术矫治年龄≤3岁的TOF患儿83例（86次）.年龄2～36个月,体重3.5～13 kg.17例有反复缺氧发作,1例术前需呼吸机支持.患儿术前均行心脏彩超检查,1例行心血管造影.75例采取一期根治手术,68例经右房修补室间隔缺损,62例跨肺动脉瓣扩大右室流出道.根据Mcgoon指数以及左心室发育情况,8例采取姑息治疗（中央性体肺分流）,其中3例于11～15个月后行二期根治手术.结果 患儿McGoon指数≥1.28时预后良好.全组存活79例,根治、姑息治疗后各死亡2例,病死率4.7%（4/86）;二期根治患儿中无一例死亡.术后随访3～62个月,无死亡病例.结论 TOF患儿婴幼儿期采取一期根治可获得满意的近期效果,少数重症患儿仍需分期矫治.     

Double-Outlet Right Ventricle Without Interventricular Communication: An Unusual and Challenging Problem

A ventriculoseptal defect (VSD) usually is present in patients with a double-outlet right ventricle. This report describes a case of double-outlet right ventricle with a completely shrouded inlet VSD and no interventricular shunting. This was associated with a severely regurgitant dysplastic mitral valve. The anatomy and clinical management of the patient is discussed together with an overview of the existing literature.     

法洛四联症右心室流出道心肌的超微结构及力学特性

目的 研究法洛四联症（ＴＯＦ）右心室流出道心肌细胞的超微结构变化及心肌组织的生物力学性能。方法 对２４例ＴＯＦ根治术手术前后的右心室及肺动脉的压力变化进行对比研究。对其中７例ＴＯＦ与对照组７例先心病小室缺、小房缺应用透射电镜进行右心室流出道心肌的超微研究。对４例ＴＯＦ与２例非先心病死亡右心室心肌进行多样本量的对比，采用计算机控制的小力学量软组织力学测试症台进行右心室心肌力学的研究。结果 ＴＯＦ右心