肥厚梗阻型心肌病猝死家族史患者心肌中Cx43表达变化

目的 通过观察猝死家族史肥厚型心肌病患者心肌中Cx43和TGFβ-1表达的改变，初步研究肥厚型心肌病猝死的可能机制。方法 分别选取猝死家族史肥厚型心肌病患者心肌组织（FHSD组,n=5）,非猝死家族史肥厚型心肌病患者心肌组织（NFHSD组,n=5）和非肥厚型心肌病患者心肌组织（NHCM组,n=5）作为研究样本（均获患者家属知情同意），应用Western blot和免疫组织化学方法检测心肌组织Cx43和TGFβ-1表达。结果 Western blot结果显示，FHSD组、NFHSD组心肌组织Cx43表达与NHCM组患者相比均显著性增高（P<0.01），NFHSD组Cx43表达明显高于FHSD组（P<0.05）；FHSD组、NFHSD组心肌组织TGF-β1表达与NHCM相比均显著性降低（P<0.01）；NFHSD组TGF-β1表达与FHSD组相比较无显著性差异。免疫组织化学结果与Western blot相同。结论 肥厚型心肌患者猝死的机制是否与心肌细胞Cx43表达在代偿期突然降低有关，尚需进一步研究。

Clinical significance of Cx43 expression of myocardium from patients with obstructive hypertrophic cardiomyopathy and family history of sudden death

AIM To preliminarily investigate the mechanisms underlying sudden death (SD) of hypertrophic cardiomyopathy (HCM) through analysis of the expression of Cx43 and TGFβ-1 in myocardium from patients with HCM and family history of sudden death (FHSD). METHODSSamples included five patients with HCM and FHSD, five patients with HCM and non-FHSD (NFHSD) and five patients with non-HCM (NHCM). Western blot and immunohistochemistry were used to detect the expression of Cx43 and TGFβ-1. RESULTSFor Western blot, expression of Cx43 in the FHSD and NFHSD group was much higher than in the NHCM group (P<0.01), and Cx43 in the NFHSD group was higher than in the FHSD group (P<0.05). However, expression of TGF-β1 in FHSD and NFHSD group was much lower than in the NHCM group (P<0.01), but there was no statistical significance between NFHSD and FHSD group. For immunohistochemistry, the findings were similar to Western blot. CONCLUSIONChanges of Cx43 in myocardium may be one of the causes leading to sudden death of HCM and TGF-β1 may mediate the changes of Cx43 in hypertrophic cardiomyopathy.