Objective:To evaluate the effect of autologous hematopoietic stem cell transplantation (ASCT) on the treatment of relapsed/refractory multiple myeloma (RRMM) with chimeric antigen receptor T cell (CAR-T) therapy.Methods:A retrospective cohort study. The clinical data of 168 patients with RRMM who underwent CAR-T therapy at the Department of Hematology, Xuzhou Medical University Hospital from 3 January 2020 to 13 September 2022 were analyzed. Patients were classified into a transplantation group (TG; n=47) and non-transplantation group (NTG; n=121) based on whether or not they had undergone ASCT previously. The objective response rate (ORR), progression-free survival (PFS), overall survival (OS) and the levels of CD3, CD4, CD8, CD19, CD56 and natural killer (NK) cells before CAR-T infusion were analyzed by χ2 test, Kaplan-Meier method and independent sample t-test. Results:Among 168 patients with RRMM, 98 (58.3%) were male. The median age of onset was 57 (range 30-70) years. After CAR-T therapy, the ORR of patients was 89.3% (92/103) in the NTG and 72.9% (27/73) in the TG. The ORR of the NTG was better than that of the TG ( χ2=5.71, P=0.017). After 1 year of CAR-T therapy, the ORR of the NTG was 78.1% (75/96), and that of the TG was 59.4% (19/32). The ORR of the NTG was better than that of the TG ( χ2=4.32, P=0.038). The median OS and PFS in the NTG were significantly longer than those in the TG (OS, 30 vs. 20 months; PFS, 26 vs. 12 months; both P<0.05). The CD4 level before CAR-T infusion in the TG was significantly lower than that in the NTG (25.65±13.56 vs. 32.64±17.21; t=-2.15, P=0.034), and there were no significant differences in the counts of CD3, CD8, CD19, CD56, and NK cells between the TG and NTG (all P>0.05). Conclusion:Among patients suffering from RRMM who received CAR-T therapy, patients who did not receive ASCT had significantly better outcomes than those who had received ASCT previously, which may have been related to the CD4 level before receiving CAR-T therapy.

Objective    To sum up the experience of the primary modified single-patch (MSP) technique applied in our hospital for children with complete atrioventricular septal defect (CAVSD). Methods    The clinical data of 141 children who underwent primary MSP technique for CAVSD between June 2009 and December 2017 were retrospectively analyzed, including 62 males and 79 females with a median age of 6 (3, 11) months and a median weight of 5.8 (4.5, 7.0) kg. According to Rastelli classification, there were 116 patients in type A, 14 in type B and 11 in type C. Among them, 15 patients were diagnosed with Down’s syndrome. Cardiopulmonary bypass time, aortic cross clamp time, atrioventricular valve regurgitation and other clinical data were recorded during and after operation. Results    Postoperatively, 17 patients suffered from severe left atrioventricular valve regurgitation (LAVVR) and 6 patients severe right atrioventricular valve regurgitation (RAVVR). In the follow-up period, 5 patients suffered from severe LAVVR and 1 patient severe RAVVR. Left ventricular outflow tract obstruction (LVOTO) appeared in 1 patient during follow-up period and none at the end of follow-up. There were 5 early deaths and 2 late deaths. Twelve patients underwent reoperation with a median interval time of 268 (8, 1 270) days. Conclusion    MSP technique is a wise surgical strategy for CAVSD children with good outcomes, improved postoperative mortality and decreased atrioventricular valve regurgitation.

Objective    To analyze the effects of cardiovascular surgery on Williams syndrome (WS). Methods    The clinical data of 68 WS patients undergoing cardiovascular surgery in the Department of Cardiac Surgery, Guangdong Provincial People's Hospital from January 2010 to January 2020 were retrospectively analyzed. There were 48 males and 20 females with a median age of 2.8 years ranging from 3 months to 33 years. Except one patient undergoing the coarctation repair, the rest 67 patients underwent surgical interventions to correct supravalvular aortic stenosis (SAVS) and pulmonary artery stenosis with hypothermic cardiopulmonary bypass, concommitant with 3 patients of relief of left ventricular outflow tract obstruction, 2 patients of relief of right ventricular outflow tract obstruction, 2 patients of mitral valvuloplasty, 3 patients of ventricular septal defect repair and 1 patient of arterial catheter ligation. Results    Two (2.9%) patients died of sudden cardiac arrest on the next day after surgery. One (1.5%) patient died of cardiac insufficiency due to severe aortic arch stenosis 3 years after surgery. The effect of SAVS was satisfactory. Two (2.9%) patients progressed to moderate aortic valvular regurgitation during postoperative follow-up. A total of 5 (7.4%) patients were re-intervened after operation for arch stenosis or pulmonary stenosis. Conclusion    WS patients should be diagnosed early, followed up and  assessed for cardiovascular system diseases, and timely surgical treatment has a good clinical effect.

Objective:To investigate the correlation of red blood cell distribution width-to-platelet ratio (RPR) with clinical features and prognosis of patients with multiple myeloma (MM).Methods:The clinical data of 137 patients with MM who were admitted to the Affiliated Hospital of Xuzhou Medical University from April 2013 to July 2019 were collected. The receiver operating characteristic (ROC) curve was used to determine the best cut-off value of RPR. According to the best cut-off value of RPR, the patients were divided into high RPR group and low RPR group, and the differences in clinical characteristics and prognosis between the two groups were analyzed.Results:The best cut-off value of RPR was 0.10, and according to the best cut-off value, the patients were divided into high RPR group (RPR ≥ 0.10, 52 cases) and low RPR group (RPR < 0.10, 85 cases). There were statistical differences between the high RPR group and low RPR group in the proportion of patients between different stratification of Durie-Salmon (DS) staging ( χ2 = 17.110, P < 0.01), International Staging System (ISS) staging ( χ2 = 10.817, P = 0.001), red blood cell distribution width standard deviation(RDW-SD) ( χ2 = 26.937, P < 0.01), hemoglobin ( χ2 = 17.140, P < 0.01), lactate dehydrogenase (LDH) ( χ2 = 7.926, P = 0.005), erythrocyte sedimentation rate (ESR) ( χ2 = 9.513, P = 0.002), β 2-microglobulin (β 2-MG) ( χ2 = 7.726, P = 0.005), and bone marrow plasma cell ratio (BMPC) ( χ2 = 6.621, P = 0.010). The overall response rate (ORR) in the low RPR group was higher than that in the high RPR group [82.4% (70/85) vs. 71.2% (37/52)], but the difference was not statistically significant ( χ2 = 2.366, P = 0.124). The deep remission rate in the low RPR group was higher than that in the high RPR group [56.5% (48/85) vs. 19.2% (10/52)], and the difference was statistically significant ( χ2 = 18.327, P < 0.01). The results of multivariate analysis showed that the albumin, RPR and degree of remission were independent influencing factors for the overall survival (OS) of newly treated MM patients (all P < 0.05). Conclusion:MM patients with elevated peripheral blood RPR have shorter OS time, and RPR may be one of the indicators for evaluating the prognosis of MM.

Objective     To analyze the use of modified pericardial oblique sinus approach in surgical repair for total anomalous pulmonary venous connection in neonates. Methods     Between May 2005 and December 2015, 67 consecutive neonates with supracardiac or infracardiac type total anomalous pulmonary venous connection who underwent surgical repair in our institute were included in this study. The patients are divided into three groups according to the different approaches including a sulcus approach group (6 patients), a superior approach group (14 patients), and a modified pericardial oblique sinus approach group (47 patients). There were 53 males and 14 females at median age of 12.5 (7.0, 20.5) d.  Results     The time of cardiopulmonary bypass [88 (80.0, 107.0) min vs. 135 (121.0, 157.0) min, P<0.05] and aortic cross clamping of the modified pericardial oblique sinus approach group was significantly shorter than that of the sulcus approach group [45 (39.0, 53.0) min vs. 80 (73.0, 85.0) min, P<0.05]. Perioperative mortality (2.1% vs. 28.6%, P<0.05) was significantly lower in the modified pericardial oblique sinus approach group than that in the superior approach group. The long-term mortality (4.3% vs. 60.0%, P<0.05) was significantly lower in the modified pericardial oblique sinus approach group than that in the sulcus approach group or the superior approach group . The rate of pulmonary venous stenosis was significantly lower in the modified pericardial oblique sinus approach group than that in the sulcus approach group (2.1% vs. 50.0%, P<0.05) or superior approach group (2.1% vs. 35.7%, P<0.05).  Conclusions     In surgical correction of neonatal supracardiac and infracardiac total anomalous pulmonary venous, compared with the traditional surgical approach, the modified pericardial oblique sinus pathway can provide excellent surgical space and has a good surgical prognosis.

Objective To analyze the application of virtual reality technology and mixed reality techniques in our hospital before and during intraoperative evaluation of complicated congenital heart diseases .Methods Methods Retrospectively ana-lyze the clinical treatment, surgical decision-making, intraoperative and early prognosis of 11 children with complicated congen-ital heart disease assessed by virtual reality technology and mixed reality techniques.The time of operation was 34-121 min, CPB time was 26-101 min, the clamping time of aorta was 18-56 min.There was no operative death.Results All 11cases were assessed by virtual reality technology and mixed reality technology before surgery .Personalized surgical strategies were made based on the evaluation results.All patients had undergone operations successfully.Compared with traditional surgical methods, fewer surgical incisions and shorter operation time were required.And it improved the surgical results.Conclusion Virtual reality technology and mixed reality technology have a great advantage in preoperative and intraoperative evaluation of complex congenital heart diseases.They can optimize surgical strategies, shorten operation time, and reduce surgical trauma. They are worthy of further promotion and application in clinical practice.

Multiple myeloma (MM) is a kind of common hematologic neoplasms. The application of new drugs and autologous hemopoietic stem cell transplantation can significantly improve the response rate of MM. However, as it is impossible to eradicate the minimal residual disease, recurrence is unavoidable. Maintenance therapy has the potential to increase remission degree, thus lengthening the progression-free survival and overall survival time of MM patients.

Objective To evaluate the endothelium-dependent vasodilation in children after repair for coarctation of aota (CoA). Methods A group of 20 children having undergone CoA repair between January 2010 and October 2010 in Guangzhou Women and Children′s Medical Center were include in the study , including 12 infants aged less than 6 months and 8 ones aged more than 6 months. Another 20 healthy children were enrolled during the same period as controls. All the subjects underwent monitoring of resting blood pressure and flow-mediated dilation (FMD) of the brachial artery in 4-year follow-up. Results There were no resting hypertension in all subjects , but FMD in the CoA group was higher than in the control group and so it was with the early surgery group and non early surgery group. Conclusion The surgical repair for coarctation of aorta could not cure the vascular function impairment satisfactorily , neither is the early surgery effective in alleviating the injuries in vascular endothelia.

Objective To review the outcomes of atrioventricular valve replacement in patients with functional single ventricle and evaluate risk factors for mortality.Methods Retrospectively reviewed our experience with atrioventricular valve (AVV replacement) in patients with functional single ventricle from January 2008 to December 2014.Outcome included mortality and valve-related complications.Results Thirteen patients required AVV replacement.Prosthetic valve type was St.Jude bileaflet mechanical mitral valve.Valve size ranged from 27 to 31 mm,including 27 mm in 2,29 mm in 7,and 31 mm in 4 patients.Median age at valve replacement was 4.5 years(range:1.2 years to 18.0 years).Concomitant procedures included bidirectional Glenn shunt in 3,pulmonary artery banding in 1,and repair of pulmonary vein stenosis in 1 patient.There were four early deaths with a perioperative mortality of 30.8％.Complications after AAV replacement included complete atrioventricuiar block in 1,intracerebral hemorrhage in 1 patient due to valve-related anticoagulation.No patient had perivalvular leakage.There was one late death during a mean follow-up of 3 years (range:0.5-5.0 years).Five-year Kaplan-Meier survival was 61.5％.Fisher exact probability test showed that lower weight(＜ 10 kg) at operation and prosthetic size/weight ratio ＞ 2.0 were risk factors for overall mortality.Of the survival patients,functional status is NYHA class Ⅰ in 3,class Ⅱ in 5.No patient developed valve-related complications.Conclusion Atrioventricular valve replacement can be performed in patients with functional single ventricle with acceptable mortality.Lower weight and increased prosthetic size/weight ratio at operation were significantly associated with worse survival.