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.
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）.Twentynine patients were followedup,and in the followup period of 1.5 months to 2 years postsurgery, 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.
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 hemiFontan 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 secondstage 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 stagemodified LT Fontan operation, there were significantly more cases who had BSCPA operation preoperatively in MLT group than that in LT group. Twentytwo 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 months8 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.
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 (PaO2) significantly increased and alveolar-aterial oxygen gradient(AaDO2) markedly decreased after 1h in PLV group(Plt;005) and partial pressure of carbon dioxide in artery (PaCO2) also became small after 3h (Plt;005).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
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.
Objective To investigate surgical strategy for the treatment of muscular ventricular septal defect （MVSD） in infants with multiple ventricular septal defects（VSD）. Methods Clinical data of 46 infants with multiple VSD who underwent surgical repair in Shanghai Children’s Medical Center from January 2010 to April 2012 were retrospectively analyzed. There were 24 males and 22 females with their age of 8±6 months and body weight of 6.1±1.9 kg. All the patients received one-stage surgical repair，among whom MVSD of 10 patients was not found and repaired during the surgery. MVSD was repaired by surgical suture in 19 patients，hybrid repair under direct vision in 12 patients，and hybrid repair via the right ventricle in 5 patients. All the patients were regularly followed up after discharge by chest X-ray，ECG and color Doppler echocardiography to observe the closure of MVSD and the presence of residual shunt. Results All the 46 patients with multiple VSD survived their surgery without perioperative death. Three patients undergoing hybrid repair under direct vision received delayed sternal closure. One patient undergoing hybrid repair under direct vision had postoperative cardiac dysfunction. All the 46 patients were followed up for 1-17 months. Twenty-three patients had residual shunt of varying degrees during follow-up，and most of the MVSD of patients with residual shunt were ＜4 mm，who were receiving further follow-up and evaluation. Conclusion Appropriate surgical strategies can be applied according to specific surgical views during the operation for the treatment of MVSD in infants with multiple VSD，and satisfactory clinical outcomes can be achieved.
Objective To evaluate long-term outcomes of surgical repair for pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs）.?Methods?We retrospectively analyzed the clinical data of 29 patients with PA-VSD and MAPCAs who underwent surgical repair in Shanghai Children’s Medical Center from February 2001 to February 2010. There were 13 patients in the one-stage operation group and 16 patients in the staged operation group. There were 8 males and 5 females in the one-stage operation group with their age ranging from 8 to 168 （26.0±17.0）months. There were 9 males and 7 females in the staged operation group with their age ranging from 4 to 149 （26.5±15.8）months. Eight patients underwent their second operation with the time interval between the two operations ranging from 14 to 40 （28.3±11.7） months. For MAPCAs， 9 patients underwent unifocalization， 16 patients underwent ligation or occlusion via cardiac catheterization， and the other 4 patients left them open or unrepaired. We compared postoperative mortality， complications， long-term outcomes， and quality of life between the two groups. Quality of life of the 29 PA-VSD patients was measured according to Pediatric Quality of Life Inventory version 4.0 and compared with 100 children from a kindergarten as a control group.?Results?Postoperative mortality of one-stage operation group was significantly higher than that of staged operation group at 15.4% （2/13） vs. 0.0% with χ2=4.12 and P=0.04. There was no statistical difference in the rate of postoperative complications between the two groups at 75.0% （9/12） vs. 56.2% （9/16） with χ2=0.51 and P=0.47. Patients were followed up for 3 to 88 months， and the follow-up mortality of one-stage operation group was not significantly higher than that of staged operation group at 27.3% （3/11） vs. 6.2% （1/16） with χ2= 2.28 and P=0.13. There was no statistical difference in overall quality of life score between one-stage operation group and staged operation group （66.7±8.6 vs 70.2±13.0， t?=?-0.71， P=0.48） . The overall quality of life score of PA-VSD patients was significantly lower than that of control group （68.7±11.2 vs. 77.8±15.7， t?= 3.14， P=0.01）， mainly because the physical functioning score of PA-VSD patients was significantly lower than that of control group （57.7±11.7 vs. 83.0±19.6， t?=5.67， P=0.00）， but there was no statistical difference in emotional well-being score， social functioning score， and school functioning score between PA-VSD patients and the control group.?Conclusion It is the key to analyze different pulmonary artery conformation， choose individualized surgical procedure and properly manage MAPCAs so as to reduce postoperative mortality and complications of surgical repair for patients with PA-VSD and MAPCAs.
Abstract： Objective To investigating the variance of nuclear factorkappa B（NF-κB）,inflammatory factor and polymorphonuclear cells(PMNs) in lung， our study infer the role of PMNs infiltration and early activity of NF-κB in empirical study of lung injury in deep hypothermia and circulatory arrest. Our study also guess the possible mechanism of action in order to provide a more excellent program for lung protection. Methods Twelve immature pigs were randomly divided into two groups，there are six pigs in each group，one group was normothermic parallel circulation(control group)，the other was deep hypothermia and circulatory arrest(DHCA, experimental group)，we obtain lung tissue and venous blood from pigs to measure the variances of NF-κB by immunohistochemistry and inflammatory factor by enzymelinked immunosorbent assay(ELISA) at different time. Results The expression of NF-κB of the lung tissue specimen was negative before parallel circulation in both groups, there was no brown dyed cell nucleus and the variation was no statistically difference in two groups. The expression of NF-κB reached it‘s peak at half an hour of ischemia reperfusion, and most of the brown dyed cell nucleus were PMNs, then the expression of NF-κB decreased in the experimental group. The lung tissue specimens were all weakly negative at the time points after parallel circulation and there was no statistical difference among them. But the content of inflammatory factor increased gradually from half an hour of ischemia reperfusion to two hour of ischemia reperfusion， which reached their peak at two hour of ischemia reperfusion.There was significance variances at the content of tumor necrosis factor-α(TNF-α) at one hour of ischemia reperfusion， while at one and a half hour of ischemia reperfusion. There was significance variance at the content of interleukin-8 and interleukin-6 in the experimental group. While in the control group, there was statistically difference before and after parallel circulation, but there was no statistically difference among the time points after parallel circulation. Conclusion The early activity of NF-κB may have an important role in lung injury of DHCA，treatments aim directly at NF-κB may provide an important strategy for lung injury of DHCA.
Objective To evaluate the outcome of surgical repair of interrupted aortic arch (IAA) combined with anomalies. Methods We retrospectively analyzed the clinical data of 48 patients with IAA combined with anomalies undergoing one-stage biventricular repair in Shanghai Children's Medical Center from November 2006 to April 2016. There were 25 males and 23 females with a median age of 29 d (range, 8 to 91 d) and a mean weight of 3.80±0.67 kg. All patients underwent end-to-end anastomosis with patch augmentation, and relief of left ventricular outflow tract obstruction (LVOTO) was performed in 11 patients. Results In IAA children with anomalies, 39 (81.3%) suffered noncomplex lesions and 9 (18.8%) complex lesions. Mean follow-up was 72.1±19.7 months for 38 patients. There were 6 in-hospital deaths and 3 patients died during follow-up. The early survival rate was 87.5%, 5-year rate 83.3% and 10-year rate 81.3%. Reintervention was required in 10 patients, including 8 with subsequent LVOTO and 2 with anastomotic stenosis. Conclusion End-to-end anastomosis with patch augmentation is effective for IAA.
Objective To explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and Fontan procedure.