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Progress of Modified Fontan Operation

Though the 10 year survival rate of Fontan procedure has increased from 69% in 1990 to 85% in 2006. Recently researchers have strived to improve the longterm outcome of this procedure and have made great achievements. They found that among the ten items of standard, the limits associated with age, anomalous systemic or pulmonary venous drainage, sinus rhythm, ratio of pulmonary artery/aorta should be relaxed. New operative designs such as extracardiac lateral tunnel Fontan and new types of HemiFontan procedure using shortened extracorporeal circulation may help to decrease the postoperative complications. Standardized postoperative therapeutic plan after Fontan procedure may decrease the duration of chest drainage，shorten the inpatient days and also avoid longterm treatment. However, longterm followup after Fontan procedure showed that the incidence rate and risk of longterm postoperative complications were still high. This article was directed to review the researches about Fontan on operative indications, optimizing the operative strategies, modifying the postoperative monitoring techniques and longterm follow-up.

心上路径矫治新生儿期完全性肺静脉异位引流

目的总结新生儿期心上型完全性肺静脉异位引流（TAPVD）的手术治疗经验，推广心上法在TAPVD手术治疗中的应用。 方法回顾性分析2008年8月至2013年8月上海儿童医学中心收治新生儿期心上型梗阻型TAPVD患儿7例的临床资料，其中男5例、女2例，年龄8～28（18.3±7.7）d，体重3.1～4.5（3.57±0.47）kg。全部患儿在全身麻醉低温体外循环下行心上法矫治术。 结果7例患儿术后早期（1个月内）无死亡。随访3个月至4年，吻合口均保持通畅，无明显肺静脉回流梗阻，中期随访无心律失常及死亡发生。 结论心上法矫治新生儿期心上型TAPVD可获得较大吻合口，减少心房内操作，有效降低术后死亡率、心律失常及吻合口再狭窄率。

先天性心脏病外科治疗中国专家共识（四）：室间隔完整型肺动脉闭锁

Research advances in genetics and molecular biology of primary cardiac tumors

Primary cardiac tumors, which originate from the heart, are uncommon and can be classified as benign or malignant, with the majority being benign. Malignant primary cardiac tumors have a poor prognosis. Benign ones may also cause severe hemodynamic and electrophysiological consequences, but the prognosis is generally good if they are detected early and treated properly. In recent years, researches on the genetic and molecular causes of primary cardiac tumors have yielded some promising breakthroughs, with some of them already being translated into clinical practice. This article reviews research progress and its use in precise diagnosis and targeted therapy from the perspective of DNA, RNA, and protein changes, as well as prospects the promising research directions in the future.

Spontaneous Closure of Residual Ventricular Septal Defect after Surgical Repair in Infant

Abstract: Objective To investigate the prognosis of residual shunt after surgical repair in infants. Methods We selected 407consecutive infant patients (255 males and 152 females) with venticular septal defects(VSD) between January 2008 and June 2008 in Shanghai Children’s Medical Center, Shanghai Jiaotong University. Of the patients, 273 were less than 1 yearold, 88 were 12 yearsold, and 46 were 23 years old. All of whom underwent surgical repair of the ventricular septal defect with autologous pericardial defect treated by glutaraldehyde. Results Of the 407 patients, 29 had residual ventricular septal defects, there was no significant difference among age groups in the rate of residual shunts （χ2=0.054，P=0.973）.Twentynine patients were followedup,and in the followup period of 1.5 months to 2 years postsurgery, 26 patients had spontaneous closure, while the other 3 patients did not heal. None of the patients needed reoperation. There was no significant difference amongage groups or type of ventricular septal defect in the rate of residual shunts (χ2=1.035，P=0.596). Conclusions Postsurgical residual ventricular septal defects smaller than 0.4 cm will close spontaneously in most infants.

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.

Experimental Research of Partial Liquid Ventilation with Perfluorocarbon on Acute Lung Injury Induced by Cardiopulmonary Bypass

Objective To investigate the effect of partial liquid ventilation (PLV) with perfluorocarbon(PFC) and continuous pulmonary artery perfusion (CPP) on lung gas exchange and lung inflammatory reaction in acute lung injury(ALI) induced by cardiopulmonary bypass (CPB). Methods Eighteen of either sex piglets(weighting10.2±1.6kg) were randomly divided into three groups: Control group, CPP+CPB group (CPP group), PLV+CPP+CPB group (PLV group). Animals in control group received no treatment but conventional mechanical ventilation.In CPP group lung perfusion with oxygenated blood at 20-25ml/kg·min was given during aortic clamping. In PLV group PFC (FDC)12ml/kg was instilled into the trachea right after CPB stopping. The changes of gas exchange were mearsured before CPB and at 0h, 1h, 2h, 3h after CPB stopping. Histological sections were taken from right and left downsides of lung. Results Compared with control group, the partial pressure of oxygen in artery (PaO2) significantly increased and alveolar-aterial oxygen gradient(AaDO2) markedly decreased after 1h in PLV group(Plt;005) and partial pressure of carbon dioxide in artery (PaCO2) also became small after 3h (Plt;005).The change of gas exchange in CPP group was markedly improved. And role of lung protection of PLV was more better than that of CPP. Light microscopy: Express of intercellular adhesion molecule-1(ICAM-1) in the histopathological lesions of lung was bely positive in control group than that of PLV group and CPP group. Conclusion PLV and continuous pulmonary artery perfusion can improve the oxygenation of lung and inhibit inflammatory reaction of acute lung injury induced by CPB

Surgical Approach of Left Superior Vena Cava Distal Abnormalities in Infant and Young Children

Objective To evaluate the surgical approach of left superior vena cava（LSVC） distal abnormalities in infant and young children. Methods From April 1999 to December 2004, 19 cases of LSVC distal abnormalities were corrected by primary repair. There were 10 males and 9 females. Their age ranged from 2.7 months to 6.5 years and body weight from 3. 1 to 15.0 kg. Diseases complicated with LSVC included complex congenital cardiac disease 9 cases, ostium secundum atrial septal defect 4, partial abnormal pulmonary venous drainage （PAPVD） with atrial septal defect（ASI）） 2, tetralogy of Fallot（TOF） 3, and double outlet of right ventricle （DORV） 1 case. The ways for surgical treatment of distal abnormalities of LSVC were reconstruction of atrial septum, translocation of LSVC and reconstruction of atrial septum, right atrium and LSVC anastomosis, cavopulmonary anastomosis and repair of partially unroofed coronary sinus. Results One patient died and the diagnosis for the patient was LSVC with DORV and pulmonary hypertension （PH）. This patient died from crisis of PH , obstruction of blood flow in the left cavopulmonary anastomosis, severe low cardiac output,low arterial oxygen saturation and abnormal function of kidney. The mean pressure of right atrium was 9 to 18 mmHg. The percutaneous oxygen saturation （SpO2） was 0.98 1.00 for biventricular repair and 0.79-0. 88 for single ventricular repair and palliative repair. The echocardiography showed no obstruction of the blood flow in LSVC and pulmonary veins. The results of follow-up were satisfactory, from 3 months to 2 years. Conclusions Key for success of surgical approach of LSVC distal abnormalities is precise evaluation of different kinds of LSVC and different surgical approaches. Cavopulmonary anastomosis can not be used in the case of LSVC with PH.

Double-Switch术治疗矫正型大动脉转位

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.