PRKAG2突变心脏综合征由PRKAG2基因突变造成所编码的腺苷酸活化蛋白激酶(adenosine monophosphate-activated protein kinase,AMPK)的调节亚基γ2结构和功能发生异常所致,以心肌肥厚、心室预激和传导系统疾病为主要表现的一种罕见的常...PRKAG2突变心脏综合征由PRKAG2基因突变造成所编码的腺苷酸活化蛋白激酶(adenosine monophosphate-activated protein kinase,AMPK)的调节亚基γ2结构和功能发生异常所致,以心肌肥厚、心室预激和传导系统疾病为主要表现的一种罕见的常染色体显性遗传病。其临床表型多样,多为个案或家系报道,目前尚无大样本数据报道其发病率及预后。本文报道1例PRKAG2突变心脏综合征患者,并介绍PRKAG2相关诊疗进展。展开更多
Background To the effect of percutaneous coronary intervention （PCI） on plasma level of N-terminal pro-B-type natriuretic peptide （NT-proBNP） in patients with coronary heart disease （CHD） and normal left ventric...Background To the effect of percutaneous coronary intervention （PCI） on plasma level of N-terminal pro-B-type natriuretic peptide （NT-proBNP） in patients with coronary heart disease （CHD） and normal left ventricular function. Methods One hundred and five patients with CHD and normal ventricular function were enrolled. Blood samples for assessment of NT-proBNP and cTn-T were collected before and after PCI. Results The mean left ventricular ejection fraction was 60.3 ± 5.3%. After revascularization, the level of lgNT-proBNP was signifi-cantly reduced （2.40 ± 0.44 vs 2.23± 0.43, P 〈 0.001）. Subgroup analysis showed that the level of lgNT-proB-NP ws consistently decreased in different clinical classifications （stable angina： 45, unstable angina： 31 and acute myocardial infarction： 29） and target-vessel revascularization （left anterior descending artery： 30, left cir-cumflex artery： 26 and right coronary artery： 49）, and in 99 patients without elevation of post-procedural cTn- T, but it showed a trend of non-significant increase in 6 patients with elevated cTn-T. Conclusions Our study demonstrates that successful PCI reduces plasma NT-proBNP concentration in patients with CHD and normal ven-tricular function. This implicates that the impact of PCI should be considered in the interpretation of NT-proBNP change in clinical practice, and further studies are necessary to investigate the direct and/or indirect effect of myocardial ischemia on BNP/NT-proBNP.展开更多
文摘PRKAG2突变心脏综合征由PRKAG2基因突变造成所编码的腺苷酸活化蛋白激酶(adenosine monophosphate-activated protein kinase,AMPK)的调节亚基γ2结构和功能发生异常所致,以心肌肥厚、心室预激和传导系统疾病为主要表现的一种罕见的常染色体显性遗传病。其临床表型多样,多为个案或家系报道,目前尚无大样本数据报道其发病率及预后。本文报道1例PRKAG2突变心脏综合征患者,并介绍PRKAG2相关诊疗进展。
文摘Background To the effect of percutaneous coronary intervention （PCI） on plasma level of N-terminal pro-B-type natriuretic peptide （NT-proBNP） in patients with coronary heart disease （CHD） and normal left ventricular function. Methods One hundred and five patients with CHD and normal ventricular function were enrolled. Blood samples for assessment of NT-proBNP and cTn-T were collected before and after PCI. Results The mean left ventricular ejection fraction was 60.3 ± 5.3%. After revascularization, the level of lgNT-proBNP was signifi-cantly reduced （2.40 ± 0.44 vs 2.23± 0.43, P 〈 0.001）. Subgroup analysis showed that the level of lgNT-proB-NP ws consistently decreased in different clinical classifications （stable angina： 45, unstable angina： 31 and acute myocardial infarction： 29） and target-vessel revascularization （left anterior descending artery： 30, left cir-cumflex artery： 26 and right coronary artery： 49）, and in 99 patients without elevation of post-procedural cTn- T, but it showed a trend of non-significant increase in 6 patients with elevated cTn-T. Conclusions Our study demonstrates that successful PCI reduces plasma NT-proBNP concentration in patients with CHD and normal ven-tricular function. This implicates that the impact of PCI should be considered in the interpretation of NT-proBNP change in clinical practice, and further studies are necessary to investigate the direct and/or indirect effect of myocardial ischemia on BNP/NT-proBNP.
