Objective Norwood Stage Ⅰ is the standard procedure to cope with hypoplastic left heart syndrome (HLHS),which continues to be the most challenging congenital heart disease.The aim of this study is to retrospectively compare the classical perioperative management of Norwood Stage] with the modified strategy.Methods Between June 2010 and November 2017,totally 10 patients with HLHS underwent the standard Norwood Stage Ⅰ procedure.They are stratified to two tiers:Group A,from June 2010 to August 2014,there were 5 boys.Age at surgeries ranged from 29 to 75 days,and weight 2.57-3.50 kg with median of 3.13 kg.Group B,from August 2014 to November 2017,there were 4 boys and 1 girl.Age at surgeries ranged from 6 to 22 days,and weight from 2.0-3.1 kg.In Group A,all 5 cases underwent the standard Norwood Stage.procedure under deep hypothermic circulatory arrest,including 4 cases of modified Blalock-Taussig shunt (MBTS) and 1 case of RV-PA shunt.In Group B,all 5 cases adopted side graft technique and RV-PA shunt,aortic arch and ascending aorta were reconstructed with treated bovine pericardial patch.Group B used incubators to adjust systemic vascular resistance instead of vasodilators.Results Group A's early mortality is 40％;Group B's early mortality is 20％,1 case died of tamponade.Conclusion The standard Norwood Stage Ⅰ procedure is a complex procedure,which demands multidisplinary cooperation,to palliatively correct HLHS.To adjust and find a suitable perioperative managements can improve the results.Sharing experiences on perioperative managements of Norwood Stage Ⅰ between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period.

Objective:To investigate the timing of primary repair for tetralogy of Fallot based on pulmonary valve-sparing.Methods:A retrospective analysis of the perioperative data of children undergoing primary repair for tetralogy of Fallot in our hospital between June 2015 and May 2019 was performed. To determinate cutoff value of pulmonary valve-sparing by using receiver operating characteristic curve( ROC curve); the children were divided into two groups according to the age of cutoff value. The duration of postoperative mechanical ventilation, the length of hospital and ICU stay, emergency surgery rate, hospital mortality, 31 day readmission rate, valve-sparing rate and fast-track rate and other indicators were compared between the two groups. Results:A total of 105 children were enrolled according to the inclusion criteria, including 67 males and 38 females, with a body mass of 4.21 kg to 21.5 kg, the median body mass was 7.9 kg, and age 1.3 months to 99.1 months, the median age was 8.8 months. Cutoff value of age for pulmonary valve-sparing was between 5.5 months and 5.6 months(sensitivity 0.90, specificity 0.58, 95% CI: 0.588-0.792). Based on the age of 6 months, the children with tetralogy of Fallot were divided into two groups.There were no significant differences in duration of postoperative mechanical ventilation, length of ICU stay, emergency surgery rate, hospital mortality, 31 day readmission rate and fast-track rate in <6 months group compared with those in ≥6 months group( P>0.05). The rate of valve-sparing in <6 months group was higher than that in ≥6 months group(65.52% vs. 30.26%)( P<0.01), and the length of hospital stay was lower than that in ≥6 months group(11 days vs. 15 days)( P<0.01). The median follow-up was 14.5 months(0.3-54.9 months), and there was no reoperation intervention at the last follow-up. There were no significant difference in the follow-up period, pulmonary regurgitation and residual obstruction between the two groups( P>0.05). Conclusion:Therapeutic effect of elective early primary repair for tetralogy of Fallot in infants is not only satisfactory, but also improve the rate of pulmonary valve-sparing during operation. It is recommended that the age of elective primary repair for tetralogy of Fallot should be advanced to less than 6 months.

Objective:To discuss the effect of modified ultrafiltration combined with sequential infusion of blood products, such as platelets and cryoprecipitation, on perioperative coagulation function in neonates undergoing cardiovascular surgery under extracorporeal circulation.Methods:A retrospective analysis was performed on 83 neonates who underwent cardiovascular surgery from January 2017 to December 2019. They were divided into the control group (conventional ultrafiltration, 51 cases) and the treatment group (modified ultrafiltration + sequential infusion of blood products, 32 cases).Results:The age of treatment group was significantly younger than that of the control group ( P<0.05). The extracorporeal circulation time of the treatment group was significantly longer than that of the control group. Compared with the preoperative data, post-APTT, post-PT and post-INR were increased significantly in the control group, platelet count and FIB were decreased significantly ( P<0.01), while there was no significant difference in the treatment group. Compared with the treatment groups, post-APTT, post-PT and post-INR were significantly increased in the control group, and postoperative platelet count and FIB were significantly decreased ( P<0.05). The drainage of control group was significantly higher than that of the treatment group at 12 h and 24 h after surgery ( P<0.01). Conclusion:The application of modified ultrafiltration combined with sequential infusion of blood products can significantly improve perioperative coagulation and reduce mediastinal bleeding in neonatal after cardiovascular surgery.

Objective:To review and analyze the clinical experience of children with aortic valve stenosis and/or insufficiency treated with autologous pulmonary valve for aortic valve replacement procedure(Ross operation) with ePTFE artificial valve for right ventricular outflow tract reconstruction.Methods:From 2015 to 2020, 8 cases of aortic stenosis and/or aortic insufficiency treated by Ross operation in our center were collected, with an age of 0.5-13.2 years old. 4 cases of aortic stenosis were diagnosed preoperatively, 3 cases of aortic stenosis with aortic insufficiency, and 1 case of infective endocarditis involving the aortic valve. The operation was carried out in three steps: Harvest autologous pulmonary valve; the diseased aortic valve was resected and autologous pulmonary valve was transplanted to the aorta by aortic root transplantation; the right ventricular outflow tract was reconstructed by a handmade ePTFE artificial flap blood vessel.Results:In 6 cases, the right ventricular outflow tract was reconstructed by hand-sewn ePTFE trileaflets, and artificial univalve in 2 cases, no death occurred during operation; all patients were cured and discharged. The patients were followed up for 1 to 36 months, with mean of(12.63±12.19) months. There was no long-term death or valvular complications. During follow-up echocardiography indicated 1 case of moderate aortic regurgitation, 1 case of mild-moderate regurgitation, and moderate regurgitation was found in 2 patients with artificial single pulmonary valve. For the remaining patients, they were mild aortic regurgitation, and a trivial or mild pulmonary artery regurgitation with hand-sewn three-leaflets ePTFE artificial vessel; All patients were followed up at the last time with a peak pressure of(6.63±3.46) mmHg(1 mmHg=0.133 kPa) across the aortic valve. The left ventricular outflow tract and aortic annulus shrank slightly after surgery(the diameter of one patient with Ross-Konno operation increased), but the annulus diameter increased with age. There was no need for further intervention.Conclusion:The Ross operation is safe for the treatment of aortic valve disease, it has good hemodynamic effect, and the autologous pulmonary artery has growth potential, especially suitable for children and young patients. Hand-sewn ePTFE with trileaflet vessels for reconstruction of right ventricular outflow tract performs well in anti-regurgitation function in the short term or may be used as a replacement material for the homograft/heterograft vessel, but longer follow-up and more cases are needed.

Basic helix loop helix (bHLH) transcription factor plays an important role in biological processes. Bmsage is a class of bHLH transcription factor highly expressed in the silk gland of Bombyx mori, which is not only involved in the developmental regulation of the silk gland cells at the embryonic period, but also plays a crucial regulatory role during the synthesis of silk protein. However, currently, much of the property and structure of Bmsage is still remained unknown. To study the property, structure and biological role of Bmsage, we constructed several prokaryotic expression vectors of Bmsage fused with NusA, MBP, SUMO, Trx and His tags, respectively, then screened and determined the best soluble expression vector and condition of Bmsage in Escherichia coli combining with the induction temperature and IPTG concentration, and further purified the recombinant Bmsage by Ni-column affinity chromatography according to the established expression condition and characterized its secondary structure using circular dichroism spectra. The results showed that NusA and MBP could significantly enhance the soluble expression of Bmsage in E. coli, but it was difficult to separate Bmsage from these tags. SUMO could not only increase the soluble expression of Bmsage in E. coli to a certain degree, but also be effectively separated from Bmsage. Other tags did not effectively promote the soluble expression of Bmsage in E. coli. Circular dichroism spectra showed that the purified Bmsage had well-defined α-helix structure in solution, indicating that SUMO may promote the correct folding of Bmsage into native-like structure. These work not only establish a foundation for further study of the property, structure and function of Bmsage, but also provide a reference for the expression and purification of other similar proteins.

Objective    To summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum (PA/IVS) and to reflect on the strategies of right ventricular decompression. Methods    The clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively. There were 10 males and 2 females with a median age at the time of surgery was 5 d (range, 1-627 d). Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed. Results    One patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment. There were 2 (16.67%) patients received postoperative intervention. The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg. Mild pulmonary regurgitation was found in 7 patients, moderate in 2 patients, and massive in 1 patient. The median time of mechanical ventilation was 30.50 h (range, 6.00-270.50 h), and the average duration of ICU stay was 164.06±87.74 h. The average postoperative follow-up time was 354.82±331.37 d. At the last follow-up, the average Z score of tricuspid valves was 1.32±0.71, the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg (range, 21-146 mm Hg) and the average percutaneous oxygen saturation was 92.78%±3.73%. Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery, respectively, with the rate of reintervention-free of 81.8%. There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves (r=–0.506, P=0.201). Conclusion    For children with PA/IVS, the simple pursuit of adequate decompression during right ventricular decompression may lead to  severe pulmonary dysfunction, increase the risk of ineffective circular shunt, and induce refractory hypoxemia. The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.