目的:分析扩大心肌切除术对消除肥厚型梗阻性心肌病(HOCM)二尖瓣反流的效果。方法:回顾2002-11至2017-07我院单一术者手术治疗的480例HOCM患者,患者均接受扩大心肌切除、离断二尖瓣装置与室间隔的异常连接及修剪乳头肌等操作,术后利用...目的:分析扩大心肌切除术对消除肥厚型梗阻性心肌病(HOCM)二尖瓣反流的效果。方法:回顾2002-11至2017-07我院单一术者手术治疗的480例HOCM患者,患者均接受扩大心肌切除、离断二尖瓣装置与室间隔的异常连接及修剪乳头肌等操作,术后利用超声心动图结果评估手术对左心室流出道(LVOT)梗阻及二尖瓣反流的改善效果。结果:480例患者中22例(4.6%)因二尖瓣器质性病变而行二尖瓣成形或置换,其余458例(95.4%)未行二尖瓣处理的患者中,1例(0.2%)术后第5天死于感染性休克,4例失访(0.9%),中位随访时间为6个月(3,12)。随访超声心动图结果显示:患者LVOT压差较术前显著减小[(12.8±11.6)mm Hg vs(89.1±30.6)mm Hg,1 mm Hg=0.133 k Pa,P<0.001];合并二尖瓣前向运动(SAM)的患者由术前451例(98.5%)下降至随访时42例(9.3%)(P<0.001);二尖瓣中量以上反流患者由术前的297例(64.8%),下降至随访期间的14例(3.1%)(P<0.001)。多因素回归分析显示:随访期间残余SAM现象的患者二尖瓣中量以上反流的发生率较高(OR=30.334,95%CI:5.619~163.739,P<0.001)。结论:扩大心肌切除术可消除绝大部分患者的LVOT梗阻及SAM相关二尖瓣反流。在不合并二尖瓣器质性病变时,HOCM患者极少需要同期处理二尖瓣。展开更多
Cardiomyopathies are defined as diseases of the myocardium with associated structural and functional abnormalities. Knowledge of these pathologies for a long period was not clear in clinical practice due to uncertaint...Cardiomyopathies are defined as diseases of the myocardium with associated structural and functional abnormalities. Knowledge of these pathologies for a long period was not clear in clinical practice due to uncertainties regarding definition,classification and clinical diagnosis. In recent decades,major advances have been made in the understanding of the molecular and genetic issues,pathophysiology,and clinical and radiological assessment of the diseases. Progress has been made also in management of several types of cardiomyopathy. Advances in the understanding of these diseases show that cardiomyopathies represent complex entities. Here,special attention is given to evolution of classification of cardiomyopathies,with the aim of assisting clinicians to look beyond schematic diagnostic labels in order to achieve more specific diagnosis. Knowledge of the genotype of cardiomyopathies has changed the pathophysiological understanding of their etiology and clinical course,and has become more important in clinical practice for diagnosis and prevention of cardiomyopathies. New approaches for clinical and prognostic assessment are provided based on contemporary molecular mechanisms of contribution in the pathogenesis of cardiomyopathies. The genotype-phe-notype complex approach for assessment improves the clinical evaluation and management strategies of these pathologies. The review covers also the important role of imaging methods,particularly echocardiography,and cardiac magnetic resonance imaging in the evaluation of different types of cardiomyopathies. In summary,this review provides complex presentation of current state of cardiomyopathies from genetics to management aspects for cardiovascular specialists.展开更多
Autoimmune mechanisms, including cellular and humoral immune, are likely to participate in the pathogenesis of at least a subgroup of idiopathic dilated cardiomyopathy (IDC), in which cellular immune-mediation plays a...Autoimmune mechanisms, including cellular and humoral immune, are likely to participate in the pathogenesis of at least a subgroup of idiopathic dilated cardiomyopathy (IDC), in which cellular immune-mediation plays a more important role. Cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is the major negative regulatory factor of cellular immunity. This study was conducted to investigate the association of CTLA-4 gene exon 1 A49→G polymorphism with susceptibility to IDC in Han Chinese and its influences on serum soluble CTLA-4 (sCTLA-4) and Th1/Th2 cytokine bias. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) techniques were used to analyze the dimorphism of CTLA-4 exon 1 in the unrelated Han ethnic population in Heilongjiang Province (including 48 IDC patients and 50 normal controls). Serum sCTLA-4, IFN-γ and IL-4 were evaluated by ELISA, with the ratio of IFN-γ/IL-4 as indicator for Th1/Th2 bias. Compared with controls, the frequencies of GG genotype (0.6042 and 0.3600, P=0.012) and the G allele (0.7396 and 0.5600, P=0.008) were significantly increased in IDC patients. Increased serum sCTLA-4 was found in the IDC group compared with the controls [(1.87±1.06) μg/L vs. (0.54±0.19) μg/L, P<0.05]. IFN-γ was much lower in IDC patients than that of the controls [(16.38±6.25) ng/L vs. (29.81±10.66) ng/L (P<0.05)], whereas no statistical difference of IL-4 was found between the two groups [(12.85±1.86) ng/L vs. (12.11±2.76) ng/L], so the ratio of IFN-γ/IL-4 declined significantly (1.63±0.50 vs. 3.01±0.89, P<0.05). Linear regression analysis manifested a significant interrelationship between the GG genotype, G allele frequencies and serum sCTLA-4, IFN-γ/IL-4 in the IDC group (r=0.57, P=0.021 and r=0.32, P=0.036). CTLA-4 gene A49→G substitution was associated with an increased IDC risk, which implicated that the CTLA-4 gene exon 1 may have a considerable role in autoimmune cardiac damage, possibly via a Thr→Ala change in signal peptide, which influences the protein synthesis and modification processes, with a result of functional alteration of sCTLA-4. The bias of Th1/Th2 paradigm was associated with the increased sCTLA-4 under certain background of immunogenetics.展开更多
文摘目的:分析扩大心肌切除术对消除肥厚型梗阻性心肌病(HOCM)二尖瓣反流的效果。方法:回顾2002-11至2017-07我院单一术者手术治疗的480例HOCM患者,患者均接受扩大心肌切除、离断二尖瓣装置与室间隔的异常连接及修剪乳头肌等操作,术后利用超声心动图结果评估手术对左心室流出道(LVOT)梗阻及二尖瓣反流的改善效果。结果:480例患者中22例(4.6%)因二尖瓣器质性病变而行二尖瓣成形或置换,其余458例(95.4%)未行二尖瓣处理的患者中,1例(0.2%)术后第5天死于感染性休克,4例失访(0.9%),中位随访时间为6个月(3,12)。随访超声心动图结果显示:患者LVOT压差较术前显著减小[(12.8±11.6)mm Hg vs(89.1±30.6)mm Hg,1 mm Hg=0.133 k Pa,P<0.001];合并二尖瓣前向运动(SAM)的患者由术前451例(98.5%)下降至随访时42例(9.3%)(P<0.001);二尖瓣中量以上反流患者由术前的297例(64.8%),下降至随访期间的14例(3.1%)(P<0.001)。多因素回归分析显示:随访期间残余SAM现象的患者二尖瓣中量以上反流的发生率较高(OR=30.334,95%CI:5.619~163.739,P<0.001)。结论:扩大心肌切除术可消除绝大部分患者的LVOT梗阻及SAM相关二尖瓣反流。在不合并二尖瓣器质性病变时,HOCM患者极少需要同期处理二尖瓣。
文摘Cardiomyopathies are defined as diseases of the myocardium with associated structural and functional abnormalities. Knowledge of these pathologies for a long period was not clear in clinical practice due to uncertainties regarding definition,classification and clinical diagnosis. In recent decades,major advances have been made in the understanding of the molecular and genetic issues,pathophysiology,and clinical and radiological assessment of the diseases. Progress has been made also in management of several types of cardiomyopathy. Advances in the understanding of these diseases show that cardiomyopathies represent complex entities. Here,special attention is given to evolution of classification of cardiomyopathies,with the aim of assisting clinicians to look beyond schematic diagnostic labels in order to achieve more specific diagnosis. Knowledge of the genotype of cardiomyopathies has changed the pathophysiological understanding of their etiology and clinical course,and has become more important in clinical practice for diagnosis and prevention of cardiomyopathies. New approaches for clinical and prognostic assessment are provided based on contemporary molecular mechanisms of contribution in the pathogenesis of cardiomyopathies. The genotype-phe-notype complex approach for assessment improves the clinical evaluation and management strategies of these pathologies. The review covers also the important role of imaging methods,particularly echocardiography,and cardiac magnetic resonance imaging in the evaluation of different types of cardiomyopathies. In summary,this review provides complex presentation of current state of cardiomyopathies from genetics to management aspects for cardiovascular specialists.
文摘Autoimmune mechanisms, including cellular and humoral immune, are likely to participate in the pathogenesis of at least a subgroup of idiopathic dilated cardiomyopathy (IDC), in which cellular immune-mediation plays a more important role. Cytotoxic T lymphocyte associated antigen-4 (CTLA-4) is the major negative regulatory factor of cellular immunity. This study was conducted to investigate the association of CTLA-4 gene exon 1 A49→G polymorphism with susceptibility to IDC in Han Chinese and its influences on serum soluble CTLA-4 (sCTLA-4) and Th1/Th2 cytokine bias. Polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) techniques were used to analyze the dimorphism of CTLA-4 exon 1 in the unrelated Han ethnic population in Heilongjiang Province (including 48 IDC patients and 50 normal controls). Serum sCTLA-4, IFN-γ and IL-4 were evaluated by ELISA, with the ratio of IFN-γ/IL-4 as indicator for Th1/Th2 bias. Compared with controls, the frequencies of GG genotype (0.6042 and 0.3600, P=0.012) and the G allele (0.7396 and 0.5600, P=0.008) were significantly increased in IDC patients. Increased serum sCTLA-4 was found in the IDC group compared with the controls [(1.87±1.06) μg/L vs. (0.54±0.19) μg/L, P<0.05]. IFN-γ was much lower in IDC patients than that of the controls [(16.38±6.25) ng/L vs. (29.81±10.66) ng/L (P<0.05)], whereas no statistical difference of IL-4 was found between the two groups [(12.85±1.86) ng/L vs. (12.11±2.76) ng/L], so the ratio of IFN-γ/IL-4 declined significantly (1.63±0.50 vs. 3.01±0.89, P<0.05). Linear regression analysis manifested a significant interrelationship between the GG genotype, G allele frequencies and serum sCTLA-4, IFN-γ/IL-4 in the IDC group (r=0.57, P=0.021 and r=0.32, P=0.036). CTLA-4 gene A49→G substitution was associated with an increased IDC risk, which implicated that the CTLA-4 gene exon 1 may have a considerable role in autoimmune cardiac damage, possibly via a Thr→Ala change in signal peptide, which influences the protein synthesis and modification processes, with a result of functional alteration of sCTLA-4. The bias of Th1/Th2 paradigm was associated with the increased sCTLA-4 under certain background of immunogenetics.
