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非梗阻性肥厚型心肌病患者及心肌肌钙蛋白基因突变患者运动诱发的收缩功能障碍

Exercise-induced systolic dysfunction in patients with non-obstructive hypertrophic cardiomyopathy and mutations in the cardiac troponin genes.

作者信息

Sakata K, Ino H, Fujino N, Nagata M, Uchiyama K, Hayashi K, Konno T, Inoue M, Kato H, Sakamoto Y, Tsubokawa T, Yamagishi M

机构信息

Division of Cardiovascular Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan.

出版信息

Heart. 2008 Oct;94(10):1282-7. doi: 10.1136/hrt.2007.116970.

Abstract

OBJECTIVES

The aim of this study was to investigate left ventricular (LV) function reserve in hypertrophic cardiomyopathy (HCM) patients with and without cardiac troponin gene mutations before transition to the dilated phase.

METHODS

LV ejection fraction (EF) was continuously evaluated in 52 patients with non-obstructive HCM during supine ergometer exercise using radionuclide ventricular function monitoring with a cadmium telluride detector (VEST). On the basis of genetic analysis, patients were divided into two groups: 10 with cardiac troponin gene mutations (group A) and 42 without these gene mutations (group B).

RESULTS

Exercise duration, peak exercise load, and heart rate during exercise did not differ between the two groups. The differences from baseline to peak exercise ofthe LV end-diastolic volume decreased similarly in the twogroups. In contrast, the difference of the LV end-systolicvolume in group A increased significantly compared withgroup B (17.7% (SD 12.7%) vs 3.4% (SD 13.2%);p=0.0031). Consequently, the difference of LVEF ingroup A decreased significantly in contrast with group B(-14.1% (SD 11.1%) vs -1.2% (SD 11.7%); p=0.0025).Additionally, the changes in LVEF and stroke volumedecreased significantly more in group A than in group B(-22.2% (SD 18.6%) vs -1.1% (SD 17.8%); p=0.0017and -12.9% (SD 21.7%) vs 12.3% (SD 24.4%);p=0.0042, respectively).

CONCLUSIONS

These results suggest that HCM patientswith cardiac troponin gene mutations may displayexercise-induced LV systolic dysfunction more frequentlythan HCM patients without this abnormality

摘要

目的

本研究旨在调查肥厚型心肌病(HCM)患者在转变为扩张期之前,有无心肌肌钙蛋白基因突变时的左心室(LV)功能储备情况。

方法

采用碲化镉探测器(VEST)进行放射性核素心室功能监测,在52例非梗阻性HCM患者仰卧位测力计运动期间持续评估左心室射血分数(EF)。根据基因分析,将患者分为两组:10例有心肌肌钙蛋白基因突变(A组)和42例无这些基因突变(B组)。

结果

两组患者的运动持续时间、运动峰值负荷和运动时心率无差异。两组左心室舒张末期容积从基线到运动峰值的差异下降相似。相比之下,A组左心室收缩末期容积的差异与B组相比显著增加(17.7%(标准差12.7%)对3.4%(标准差13.2%);p=0.0031)。因此,与B组相比,A组左心室射血分数的差异显著降低(-14.1%(标准差11.1%)对-1.2%(标准差11.7%);p=0.0025)。此外,A组左心室射血分数和每搏输出量的变化比B组显著降低更多(-22.2%(标准差18.6%)对-1.1%(标准差17.8%);p=0.0017和-12.9%(标准差21.7%)对12.3%(标准差24.4%);p=0.0042)。

结论

这些结果表明,与无此异常的HCM患者相比,有心肌肌钙蛋白基因突变的HCM患者可能更频繁地出现运动诱发的左心室收缩功能障碍。

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