Objective: To explore the surgical strategy for Ebstein anomaly in children. Methods: From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm. The tricuspid valve regurgitation (TR) were mild in 26 cases, moderate in 46 cases and severe in 69 cases. Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction, 37 valve hoist), tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases. Surgical strategy were divided into biventricular heart function in 77 cases, one and a half ventricular heart function in 56 cases, and single ventricular heart function in 8 cases. Results: Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period. One case died in biventricular group. The complete atrioventricular block were occurred in 3 patients and pacemaker were applied. One hundred and forty cases discharged from hospital. There were mild TR in 118 cases, moderate in 14 cases and closed in 8 cases. One hundred and thirty-seven cases were followed up regularly in 18 to 172 months. Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases, moderate in 15 cases and severe in 1 case). Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases, moderate in 12 cases and severe in 3 cases). In the patients with severe TR (4 cases), 3 cases were reoperated by cone reconstruction. One case′s valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function, the oxygen saturation was increased. Two patients underwent tricuspid valve replacement, 1 died and the other′s mechanical valve was removed, and changed to single ventricular function repair. Conclusions Although tricuspid cone reconstruction can achieve good results, the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair. To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia, transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.

Objective To summarize 566 consecutive cases of Fontan operation in the treatments of complex congenital heart diseases and to analyze the risk factors of early surgical outcome.Methods We reviewed all 566 consecutive patients who underwent a Fontan procedure in Shanghai Children's Medical Center from January 2006 to May 2014.Preoperative records of staged surgeries,perioperative data and imaging results were obtained from medical records.Results A total of 566 patients(M/F =345/221) underwent a Fontan operation,with 166 cases of single stage Fontan operation and 400 cases of multi-staged operation.The median age at the time of the Fontan procedure was 5 years,and the median weight was 15.4 kg.In our series,there were 278 extracardiac conduits,110 intracardiac conduits,114 lateral tunnels,36 intracardiac/extracardiac conduits,and 27 direct cavopulmonary conduits.There were 36 cases of early Fontan failure,with overall early postoperative survival rate being 93.6％.The median ventilation duration was 8 h,with the median postoperative intensive care unit stay being four days,the average oxygen saturation being 0.90 at the end of ICU stay,the median chest drainage time being 10 days,and the median postoperative hospital stay being 19 days.Besides the CICU oxygen saturation,the rest postoperative results had no obvious differences in five surgical groups.Heterotaxy syndrome,earlier age at the time of the Fontan procedure,bilateral Glenn shunt,moderate AV valve regurgitation and severe and right ventricular-type morphology were risk factors of early Fontan failure,P ＜ 0.05.Conclusion In order to improve the early outcome of Fontan operation,closely follow-up should be taken directly after Glenn operation in high-risk patients.Doctors should also have an in-depth understanding of the of postFontan physiological and pathological features,and select the operation time and Fontan type most suited to the of the individualized characteristics.

Objective:To understand the morphologic and functional changes of ventricles between pre- and post- cone reconstruction(CR) surgery in children with Ebstein’s anomaly(EA).Methods:The clinical data of children with EA who underwent CR and cardiac magnetic resonance(CMR) in Shanghai Children’s Medical Center between July 2011 to April 2019 were collected and analyzed. Ventricular functions were assessed with the use of ejection fraction(EF), stroke volume index(SVI), cardiac output(CO), and cardiac index(CI). Ventricular morphologies were assessed with the use of end-diastolic ventricular volume(EDV), end-diastolic ventricular volume index(EDVI) and ventricular cine images. Paired student t tests and Wilcoxon rank sum tests were used for statistical analysis. Results:There were a total of 32 children with EA who underwent CR and CMR, with 13 males and 19 females, a median operative age of 2.9 years old(0.6-15.5 years old), and a mean follow-up time of(4.4±1.9) years. Seven patients had both preoperative and postoperative CMR, with a mean follow-up time of(3.3±1.4) years; Eleven patients had two or more postoperative CMR, with a mean interval time of(1.9±1.0) years. After the surgery, the median tricuspid-regurgitation grade decreased from 3 to 2, and the median New York Heart Association functional class improved from 2.5 to 1, the left ventricle(LV)-SVI, LV-EDV and LV-EDVI increased from 29.8 ml/m 2 to 43.2 ml/m 2( P=0.039), from 56.4 ml to 86.9 ml( P=0.004), from 50.5 ml/m 2 to 68.4 ml/m 2( P=0.022), respectively. And the long-term LV-EDV increased from 56.6 ml to 74.7 ml( P=0.002) when compared to that of early postoperative. There was no significant differences in right ventricle(RV)-EF, RV-SVI, RV-CO, RV-CI, RV-EDV and RV-EDVI between pre- and post- CR( P>0.05); but the long-term postoperative RV-CO and RV-EDV increased from 3.1 L/min to 4.1 L/min( P=0.008), from 67.5 ml to 96.5 ml( P<0.001), respectively, when compared with those of early postoperative. Conclusion:CR improves the function and morphology of both ventricles in children with EA. And although postoperative ventricles grow well, RV dysfunction persists.

Objective    To investigate the prognosis and risk factors of mild to moderate or moderate atrioventricular valve regurgitation (AVVR) after Fontan operation. Methods    A total of 34 patients with mild to moderate or moderate AVVR who accepted Fontan operation and atrioventricular valve (AVV) repair between 2004 and 2018 in our center were selected as an AVV repair group. The patients in the same period were matched as a control group by the ratio of 1 : 1-2. Finally 99 patients were included into this study, including 64 males and 35 females, with an average age of 63.4±36.3 months and weight of 17.3±6.7 kg. Grades of AVVR decreased more than 1 was defined as significant improvement. Endpoints of the study were death, Fontan takedown, AVV replacement. Risk factors including Fontan procedures, AVV repair procedures, cardiac anatomy were analyzed. Results    Patients were followed up for 1.5 (0.3-4.0) years. Overall mortality was 15.2%. Most (82.4%) of AVV repair group accepted single AVV repair procedure while partial annuloplasty was the most common (52.9%). With the extension of follow-up, the degree of AVVR in the whole group showed a gradually increasing trend (r=0.352, P=0.000). Mild to moderate AVVR improved spontaneously after Fontan operation, while moderate AVVR did not. AVV repair could improve the degree of AVVR after moderate regurgitation, without increasing the surgical mortality, and regurgitation significantly decreased in 8.8% patients. AVV repair was not effective for mild to moderate AVVR and would increase surgical mortality. Conclusion    AVV function shows a gradual downward trend after Fontan operation. AVV repair is effective for moderate AVVR, does not increase mortality, but the degree of improvement is limited. AVV repair is not effective for mild to moderate AVVR and increases surgical mortality.

Although genome-wide association studies have identified more than eighty genetic variants associated with non-small cell lung cancer (NSCLC) risk, biological mechanisms of these variants remain largely unknown. By integrating a large-scale genotype data of 15 581 lung adenocarcinoma (AD) cases, 8350 squamous cell carcinoma (SqCC) cases, and 27 355 controls, as well as multiple transcriptome and epigenomic databases, we conducted histology-specific meta-analyses and functional annotations of both reported and novel susceptibility variants. We identified 3064 credible risk variants for NSCLC, which were overrepresented in enhancer-like and promoter-like histone modification peaks as well as DNase I hypersensitive sites. Transcription factor enrichment analysis revealed that USF1 was AD-specific while CREB1 was SqCC-specific. Functional annotation and gene-based analysis implicated 894 target genes, including 274 specifics for AD and 123 for SqCC, which were overrepresented in somatic driver genes (ER = 1.95, P = 0.005). Pathway enrichment analysis and Gene-Set Enrichment Analysis revealed that AD genes were primarily involved in immune-related pathways, while SqCC genes were homologous recombination deficiency related. Our results illustrate the molecular basis of both well-studied and new susceptibility loci of NSCLC, providing not only novel insights into the genetic heterogeneity between AD and SqCC but also a set of plausible gene targets for post-GWAS functional experiments.
Adenocarcinoma of Lung/genetics* ; Carcinoma, Non-Small-Cell Lung/genetics* ; Carcinoma, Squamous Cell/genetics* ; Genetic Heterogeneity ; Genetic Predisposition to Disease ; Genome-Wide Association Study ; Humans ; Lung Neoplasms/genetics* ; Polymorphism, Single Nucleotide