马润伟
云南省阜外心血管病医院 心外科二病区
Fallot-type absent pulmonary valve is a rare and complex congenital heart disease. Repair surgery for this condition during the neonatal period has a mortality rate of over 50%. We reported a neonate with Fallot-type absent pulmonary valve and occlusion of the left main bronchus. The patient's pulmonary artery had unusual anatomy of a type that has not previously been reported. This case report outlines a successful treatment strategy for patients with complex congenital heart disease and airway occlusion during the neonatal period and the effect of these unusual anatomical conditions on postoperative outcomes.
Journal of cardiothoracic surgery 2024
BACKGROUND AND AIMS:Smooth muscle and endothelial cell-enriched migration/differentiation-associated lncRNA (SENCR) has been reported to be associated with some cardiovascular diseases; however, its function and exact molecular mechanism in aortic dissection (AD) remain undefined. Thus, we investigated the effects of SENCR on AD and its potential mechanisms.METHODS AND RESULTS:SENCR expression in aortic media specimens from AD patients was detected by quantitative real-time PCR (qPCR). The roles of SENCR in vascular smooth muscle cell (VMSC) proliferation and migration as well as in the regulation of contractile phenotype genes were studied using CCK-8, wound healing, Transwell, qPCR and Western blot assays. Dual-luciferase reporter assays were performed to identify the regulatory correlation between SENCR, miR-206 and myocardin. Furthermore, mouse AD models were constructed with ApoE-/- mice, and the effect of upregulated SENCR on phenotypic switching in the AD model was detected using hematoxylin and eosin (H&E) staining and immunohistochemistry (IHC) assays. SENCR overexpression inhibited VSMC proliferation, migration and synthetic phenotype-related gene expression; decreased miR-206 expression; increased myocardin expression; and suppressed rupture of the aortic media in mice. SENCR knockdown had the opposite effects. Our results further suggested that miR-206 upregulation could reverse the inhibitory roles of SENCR upregulation and that myocardin upregulation could restore the function of SENCR upregulation in VSMCs. Dual-luciferase reporter assays confirmed that SENCR regulated miR-206, which directly targeted myocardin in VSMCs.CONCLUSION:SENCR overexpression suppressed VMSC proliferation and migration, maintained the contractile phenotype and suppressed aortic dilatation via the miR-206/myocardin axis.
Nutrition, metabolism, and cardiovascular diseases : NMCD 2022
Uncontrolled proliferation, migration and phenotypic switching of vascular smooth muscle cells (VSMCs) are important steps in the development and progression of aortic dissection (AD). The function and potential mechanism of miR-335-5p in the pathogenesis of AD are explored in this study. Specifically, the biological function of miR-335-5p is explored in vitro through CCK-8, Transwell, immunofluorescence, EdU, wound-healing, RT-qPCR and western blotting assays. In addition, an AD model induced by angiotensin II is used to investigate the function of miR-335-5p in vivo. A dual-luciferase assay is performed to verify the targeting relationship between miR-335-5p and specificity protein 1 (SP1). Experiments involving the loss of SP1 function are performed to demonstrate the function of SP1 in the miR-335-5p-mediated regulation of human aortic-VSMCs (HA-VSMCs). AD tissues and platelet-derived growth factor BB (PDGF-BB)-stimulated HA-VSMCs show significant downregulation of miR-335-5p expression and upregulated SP1 expression. Overexpression of miR-335-5p effectively suppresses cell proliferation, migration and synthetic phenotype markers and enhances contractile phenotype markers induced by PDGF-BB treatment. Additionally, SP1 is identified as a target gene downstream of miR-335-5p, and its expression is negatively correlated with miR-335-5p in AD. Upregulation of SP1 partially reverses the inhibitory effect of miR-335-5p on HA-VSMCs, whereas the downregulation of SP1 has the opposite effect. Furthermore, Ad-miR-335-5p clearly suppresses aorta dilatation and vascular media degeneration in the AD model. Our results suggest that miR-335-5p inhibits HA-VSMC proliferation, migration and phenotypic switching by negatively regulating SP1, and indicate that miR-335-5p may be a potential therapeutic target in AD.
Acta biochimica et biophysica Sinica 2022
Background: The present study aimed to explore the correlation between calcium-activated potassium channels, left atrial flow field mechanics, valvular atrial fibrillation (VAF), and thrombosis. The process of transforming mechanical signals into biological signals has been revealed, which offers new insights into the study of VAF. Methods: Computational fluid dynamics simulations use numeric analysis and algorithms to compute flow parameters, including turbulent shear stress (TSS) and wall pressure in the left atrium (LA). Real-time PCR and western blotting were used to detect the mRNA and protein expression of IKCa2.3/3.1, ATK1, and P300 in the left atrial tissue of 90 patients. Results: In the valvular disease group, the TSS and wall ressure in the LA increased, the wall pressure increased in turn in all disease groups, mainly near the mitral valve and the posterior portion of the LA, the increase in TSS was the most significant in each group near the mitral valve, and the middle and lower part of the back of the LA and the mRNA expression and protein expression levels of IKCa2.3/3.1, AKT1, and P300 increased (p < 0.05) (n = 15). The present study was preliminarily conducted to elucidate whether there might be a certain correlation between IKCa2.3 and LA hemodynamic changes. Conclusions: The TSS and wall pressure changes in the LA are correlated with the upregulation of mRNA and protein expression of IKCa2.3/3.1, AKT1, and P300.
Cells 2022
BACKGROUND:Congenital tricuspid valve malformations are known to occur, but tricuspid valve malformations associated with twins are rarely reported. We report this case from the point of view of a medical history, an auxiliary examination and a genetic pathogenesis to provide a reference for our peers.CASE PRESENTATION:We report a rare case of congenital heart disease in monozygotic twins of Hui nationality in Yunnan-Guizhou Plateau, they are normal conception. Twin 1 had Ebstein's anomaly, and received surgical treatment and recovered satisfactorily. Twin 2 had only partial tricuspid septal prolapse, and pulmonary hypertension occurred during follow-up.CONCLUSIONS:It is necessary to carry out individualized diagnosis and treatment for twins and follow-up observation by echocardiography for a long time. Choosing the right time for cardiac surgery is of great significance to the treatment of the disease.
Journal of cardiothoracic surgery 2022
Backgrounds:Pulmonary arterial hypertension (PAH) is characterized by progressive pulmonary vascular functional and structural changes, resulting in increased pulmonary vascular resistance and eventually right heart failure and death. Congenital Left-to-Right shunts (LTRS) is one type of congenital heart disease (CHD) and PAH associated with the congenital Left-to-Right shunt (PAH-LTRS) is a severe disease in children. However, changes in the lung microbiome and their potential impact on PAH-LTRS have not been not fully studied. We hypothesized that lung microbiota and their derived metabolites have been disturbed in children with PAH-LTRS, which might contribute to the progression and outcomes of PAH-LTRS.Methods:In this study, 68 age- and sex-matched children of three different groups (patients with PAH-LTRS cohort, patients with LTRS but have no pathologic features of PAH cohort, and healthy reference cohort) were enrolled in the current study. Bronchoalveolar lavage fluid samples from these participants were conducted for multi-omics analysis, including 16S rRNA sequencing and metabolomic profiling. Data progressing and integration analysis were performed to identify pulmonary microbial and metabolic characteristics of PAH-LTRS in children.Results:We found that microbial community density was not significantly altered in PAH-LTRS based on α-diversity analysis. Microbial composition analysis indicated phylum of Bacteroidetes was that less abundant while Lactobacillus, Alicycliphilus, and Parapusillimonas were significantly altered and might contribute to PAH in children with LTRS. Moreover, metabolome profiling data showed that metabolites involved in Purine metabolism, Glycerophospholipid metabolism, Galactose metabolism, and Pyrimidine metabolism were also significantly disturbed in the PAH-LTRS cohort. Correlation analysis between microbes and metabolites indicated that alterations in the microbial composition from the lung microbiota could eventually result in the disturbance in certain metabolites, and might finally contribute to the pathology of PAH-LTRS.Conclusion:Lung microbial density was not significantly altered in patients with PAH-LTRS. Composition analysis results showed that the relative microbiome abundance was different between groups. Metabolome profiling and correlation analysis with microbiota showed that metabolome also altered in children with PAH-LTRS. This study indicated that pulmonary microbes and metabolites disturbed in PAH-LTRS could be potentially effective biomarkers and provides valuable perspectives on clinical diagnosis, treatment, and prognosis of pediatric PAH-LTRS.
Frontiers in medicine 2022
