Transcatheter tricuspid valve replacement(TTVR)is characterized by minimal invasiveness,rapid recovery,and a significantly lower perioperative mortality rate compared to surgical procedures.It is the preferred treatment for patients with bioprosthetic valve failure following surgical tricuspid valve replacement.However,when the delivery system is relatively bulky,challenges can arise due to the reduced orifice area post-surgery and the constraints imposed by the valve frame.These factors may result in difficulties advancing the delivery system.Additionally,the tortuous right heart pathway and limited support provided by the guide wire further increase the complexity of the procedure.In the present case,the patient experienced bioprosthetic valve failure following surgical tricuspid valve replacement.During TTVR,the advancement of the delivery system across the tricuspid valve encountered difficulties.Our team promptly employed the parallel anchoring-supporting sheath and snared wire(PASS)technique,pioneered at our center.Utilizing a large supporting sheath in conjunction with a snare to secure the tip of a extra-stiff guide wire,we straightened the tortuous pathway,providing additional support to the extra-stiff guide wire.This maneuver successfully facilitated the advancement of the delivery system across the tricuspid valve,offering a practical and effective solution for overcoming intraoperative challenges associated with TTVR.

Background: Diabetes-induced cardiac fibrosis is one of the main mechanisms of diabetic cardiomyopathy. As a common histone methyltransferase, enhancer of zeste homolog 2 (EZH2) has been implicated in fibrosis progression in multiple organs. However, the mechanism of EZH2 in diabetic myocardial fibrosis has not been clarified. Methods: In the current study, rat and mouse diabetic model were established, the left ventricular function of rat and mouse were evaluated by echocardiography and the fibrosis of rat ventricle was evaluated by Masson staining. Primary rat ventricular fibroblasts were cultured and stimulated with high glucose (HG) in vitro. The expression of histone H3 lysine 27 (H3K27) trimethylation, EZH2, and myocardial fibrosis proteins were assayed. Results: In STZ-induced diabetic ventricular tissues and HG-induced primary ventricular fibroblasts in vitro, H3K27 trimethylation was increased and the phosphorylation of EZH2 was reduced. Inhibition of EZH2 with GSK126 suppressed the activation, differentiation, and migration of cardiac fibroblasts as well as the overexpression of the fibrotic proteins induced by HG. Mechanical study demonstrated that HG reduced phosphorylation of EZH2 on Thr311 by inactivating AMP-activated protein kinase (AMPK), which transcriptionally inhibited peroxisome proliferator-activated receptor γ (PPAR-γ) expression to promote the fibroblasts activation and differentiation. Conclusion Our data revealed an AMPK/EZH2/PPAR-γ signal pathway is involved in HG-induced cardiac fibrosis.

OBJECTIVES: To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography. METHODS: Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology. RESULTS: Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis). CONCLUSIONS TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period.
Female ; Humans ; Pregnancy ; Echocardiography ; Heart Defects, Congenital/surgery* ; Pulmonary Veins/surgery* ; Retrospective Studies ; Scimitar Syndrome/surgery* ; Infant, Newborn

【Objective】 To investigate the correlation of serum exosomal microRNA-301a (miR-301a) with cardiovascular and cerebrovascular events after peritoneal dialysis in diabetic nephropathy. 【Methods】 A total of 211 patients with diabetic nephropathy treated with peritoneal dialysis from June 2019 to June 2020 in the First Hospital Affiliated of Hebei North University were selected as study subjects. Serum exosomal miR-301a was detected by real-time fluorescence quantitative polymerase chain reaction. The patients were divided into high miR-301a group and low miR-301a group based on the median of miR-301a; the clinical data of the two groups were compared. The correlation of miR-301a with high-sensitivity C-reactive protein (hs-CRP) was analyzed by Spearman. Linear regression was applied to analyze the factors associated with the effect of miR-301a. The patients were followed up for two years. Kaplan-Meier and Log-Rank were conducted to compare the cumulative incidence of cardiovascular and cerebrovascular events between the two groups, and COX regression and restricted cubic spline were used to analyze the level-effect relationship between miR-301a and cardiovascular and cerebrovascular events after peritoneal dialysis. 【Results】 Thirty-seven cases (17.54%) of cardiovascular and cerebrovascular events occurred during follow-up. The hs-CRP level and dialysis duration were lower in low miR-301a group than in high miR-301a group (P<0.05). There was a positive correlation between miR-301a and hs-CRP (rs=0.237, P=0.001). Linear regression analysis showed that hs-CRP was independently associated with miR-301a (P<0.05). The cumulative cardiovascular and cerebrovascular event rate in low miR-301a group was 3.70% (4/108), which was lower than that in high miR-301a group [32.04% (33/103), P<0.001]. COX regression analysis showed that high serum albumin level was an independent protective factor for cardiovascular and cerebrovascular events after peritoneal dialysis in diabetic nephropathy,while high hs-CRP level and miR-301a >1.46 were independent risk factors for cardiovascular and cerebrovascular events after peritoneal dialysis in diabetic nephropathy. Restricted cubic spline fitting COX regression analysis showed a non-linear relationship between miR-301a and cardiovascular and cerebrovascular events after peritoneal dialysis in diabetic nephropathy (P<0.05). 【Conclusion】 hs-CRP is independently associated with miR-301a in diabetic nephropathy peritoneal dialysis patients. High miR-301a level suggests a high risk of cardiovascular and cerebrovascular events after peritoneal dialysis in diabetic nephropathy.

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/genetics* ; Child ; China/epidemiology* ; Cryptorchidism/genetics* ; Disorders of Sex Development/genetics* ; Female ; Genital Diseases, Male ; Genotype ; Humans ; Hypospadias/genetics* ; Male ; Membrane Proteins/genetics* ; Penis/abnormalities* ; Phenotype ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics*

Objective: To investigate the histopathologic, immunohistochemical, molecular genetic characteristics of CIC-rearranged sarcoma (CRS) with rhabdoid features. Methods: The clinical and pathologic data of two cases of CRS diagnosed between 2019 and 2021 at the Department of Pathology, Jiangsu Province Hospital were analyzed. Immunohistochemical study and fluorescence in situ hybridization (FISH) were performed. The relevant literature was reviewed. Results: Both patients were female, one was 58 years old, with tumor located in left thigh; the other was 43 years old, with tumor located in left pelvic cavity. Microscopically, both tumors were composed of small to medium-sized round, oval cells, arranged in nodules or sheets. The tumor cells showed irregular nuclear outline, coarse chromatin with prominent nucleoli and brisk mitotic activity. Both cases showed rhabdoid phenotype with myxoid stromal changes. Immunohistochemically, both cases were positive for CD99 and c-myc. High WT1 reactivity was seen in classic area, with low reactivity in rhabdoid area. There was no INI1 lost in both cases. Both were negative for NKX2.2 and NKX3.1. By FISH both cases demonstrated convincing break-apart signals of CIC gene. One patient died of disease after 1 month, and the other died of disease after 3 months. Conclusions: CRS is a small round cell undifferentiated sarcoma of the bone and soft tissue defined by molecular genetic characteristics, and may show atypical morphologic and immunophenotypic characteristics such as rhabdoid features. A correct understanding of its rare morphologic and immunophenotypic characteristics, combined with molecular pathologic detection, is conducive to correct diagnosis.
Female ; Humans ; Male ; Biomarkers, Tumor/analysis* ; In Situ Hybridization, Fluorescence ; Sarcoma/pathology* ; Sarcoma, Small Cell/pathology* ; Transcription Factors/genetics* ; Rhabdoid Tumor/pathology*

OBJECTIVE: To explore the regulation effect of myeloid leukemia No.1 Chinese herb medicine prescription combined with chemotherapy on Th17 cells in bone marrow fluid of AML patients, so as to provide guidance for improving AML treatment effect and patients' long-term survival. METHODS: Seventy patients with AML who were hospitalized in Department of Hematology, Wuwei People's Hospital from April 2017 to August 2019 were selected and enrolled in AML group, 25 healthy volunteers were selected and enrolled in control group; then according to therapeutic regimen, AML patients were divided into 2 groups: combined therapy group (myeloid leukemia NO.1 Chinese herb medicine prescription combined with chemotherapy) and non-combined therapy group (chemotherapy alone). Flow cytometry was used to detect the ratio of CD3 RESULTS: The ratio of CD3 CONCLUSION Th17 cells expression in bone marrow of newly diagnoses and relapsed AML patients significantly increase, and decrease significantly after treatment. Myeloid leukemia No.1 Chinese herb prescription combined with chemotherapy can significantly increase the CR rate and reduce the RL rate for AML.
Bone Marrow ; China ; Humans ; Leukemia, Myeloid, Acute/drug therapy* ; Medicine ; Prescriptions ; Th17 Cells ; Vascular Endothelial Growth Factor A

OBJECTIVE: To detect the level of vascular endothelial growth factor (VEGF) in bone marrow of patients with non-M3 acute leukemia (AL), and estimate its relationship with prognosis. METHODS: From January 2016 to December 2019, 114 patients with AL in department of Hematology, Wuwei People's Hospital were selected as study group, and 25 healthy volunteers were enrolled as control group. The concentration of VEGF in bone marrow was detected by ELISA. The patients were divided into high and low concentration group according to the level of VEGF. The overall survival (OS) and event-free survival (EFS) were compared among different groups. RESULTS: The level of VEGF in patients with AL was significantly higher than that in the control group. The median OS and EFS in the low concentration group was 34.5 and 32 months, respectively, while, in the high concentration group was 30 and 26 months, respectively. The differences between the two groups were statistically significant (P=0.010). There were significant differences in OS rate (P=0.035) and EFS rate (P=0.026) between low and high concentration group. Multivariate analysis showed that high VEGF concentration was an independent risk factor affecting OS (HR=2.619, 95%CI 1.070-6.406, P=0.035) and EFS (HR=2.221, 95%CI 1.074-4.552, P=0.031) in AL patients. CONCLUSION VEGF highly expresses in the bone marrow of patients with AL at initial diagnosis and relapse, and shows adverse effects on the prognosis.
Bone Marrow ; Disease-Free Survival ; Humans ; Leukemia, Myeloid, Acute ; Prognosis ; Vascular Endothelial Growth Factor A

Objective: To analyze the features of degenerating cystic thyroid nodules (DCTN) on conventional ultrasound and contrast-enhanced ultrasound (CEUS), and to explore the differentiation between DCTN and papillary thyroid carcinomas (PTC). Methods: A total of 46 DCTN (39 cases, including 12 males and 27 females, with an age range of 25 to 76 years) and 36 PTC (32 cases, including 8 males and 24 females, with an age range of 23 to 68 years) diagnosed via fine- needle aspiration (FNA) or surgery from February 2019 to January 2020 in the First Affiliated Hospital of Nanchang University were enrolled. The size, shape, margin, echogenicity, presence of shadowing, calcification and vascularity of DCTN and PTC were retrospectively evaluated, and 28 DCTN and 30 PTC underwent CEUS were separately analyzed and compared.The t test, χ² test or Fisher's exact test were implemented to compare the features of ultrasound among the two groups. The binary Logistic regression test was performed to determine whether the feature whose difference was statistically significant was an independent predictive risk factor. Results: A univariate analysis indicated that DCTN more frequently showed wider-than-tall shapes, marked hypoechogenicity, well-defined margin and no or dot-lined enhancement (wider-than-tall shapes: 36 vs. 17, χ²=8.511; well-defined margin: 30 vs. 15, χ²=4.523; marked hypoechogenicity: 27 vs. 9, χ²=9.310; no or dot-lined enhancement: 24 vs. 3, χ²=33.369; all P＜0.05). A multivariate analysis demonstrated that wider-than-tall shapes, well-defined margin and marked hypoechogenicity were independent predictors for DCTN (OR values were 5.204, 3.134 and 5.042, P values were 0.003, 0.031, and 0.003, respectively). Among 28 DCTN, 15 showed a decrease in mean maximum diameter (24.3±11.4 mm) with a mean time span of (18.6±10.5) months between the presence and absence of suspicious ultrasound features. Conclusions: Compared with PTC, DCTN shows the ultrasound characteristics of wider-than-tall shapes, well-defined margin, marked hypoechogenicity and no or dot-lined enhancement pattern. Ultrasound follow-up can help to identify spontaneous DCTN.
Adult ; Aged ; Carcinoma, Papillary/diagnostic imaging* ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Thyroid Cancer, Papillary/diagnostic imaging* ; Thyroid Neoplasms/diagnostic imaging* ; Thyroid Nodule/diagnostic imaging* ; Ultrasonography

OBJECTIVE: To evaluate the predictive value of pre-treatment serum uric acid (sUA) level for the prognosis of newly diagnosed multiple myeloma (NDMM) patients. METHODS: The NDMM patients admitted to our center from January 2014 to December 2018 were analyzed retrospectively, and 94 patients among them who were initially treated with bortezomib-based chemotherapy for at least 4 cycles were included in this study. Clinical characteristics, laboratory data and follow-up information were collected, and the predictive value of sUA on the overall survival (OS) of NDMM was evaluated by using receiver operating characteristic (ROC) curve based on the patient's pre-treatment sUA level and the survival status at the end of follow-up, and the correlation of the sUA level with patient's clinical, laboratory characteristics and overall survival (OS) was further analyzed. The univariate and multivariate Cox proportional-hazards model were used to identify the potential factors affecting OS. RESULTS: ROC analysis showed that the area under the curve for predicting OS in NDMM patients with sUA level was 0.702 (P<0.001), and the optimal cut-off value was 455.4 μmol/L. Compared to patients with low sUA (<455.4 μmol/L), patients with higher sUA (≥455.4 μmol/L) were more likely to have international staging system (ISS) stage III disease, beta2-microglobulin (β CONCLUSION Pre-treatment sUA level is a potential biomarker for the prognosis evaluation in NDMM patients, which deserves a further exploration and verification.
Bortezomib ; Humans ; Middle Aged ; Multiple Myeloma ; Prognosis ; Retrospective Studies ; Uric Acid