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运动训练可改善由截断 titin 变体引起的扩张型心肌病患者的心血管功能。

Exercise training improves cardiovascular fitness in dilated cardiomyopathy caused by truncating titin variants.

机构信息

Department of Neurology, Rigshospitalet, Copenhagen, Denmark.

Department of Cardiology, Herlev Hospital, Herlev, Denmark.

出版信息

Heart. 2024 Nov 25;110(24):1416-1425. doi: 10.1136/heartjnl-2024-323995.

Abstract

BACKGROUND

Participation in regular exercise activities is recommended for patients with chronic heart failure. However, less is known about the effect of exercise in patients with genetic dilated cardiomyopathy (DCM). We sought to examine the effect of vigorousintensity training on physical capacity in patients with DCM caused by truncating titin variants (TTNtv).

TRIAL DESIGN

Non-randomised clinical pre-post trial of exercise training.

METHODS

Individuals with DCM-TTNtv were included from outpatient clinics for inherited cardiac diseases. The trial consisted of 8 weeks of usual care followed by 8 weeks of regular vigorous-intensity cycling exercise, enclosed by three test days. The primary outcome was change in peak oxygen uptake (VO). Secondary outcomes included change in blood volume, total haemoglobin mass, measures of systolic function and cardiac output/stroke volume during exercise.

RESULTS

Thirteen out of 14 included participants (43% women, age 48±11 years, body mass index: 30±6 kg/m) completed the trial. In the exercise training period, peak VO increased by +1.9 mL/kgmin (95% CI +0.9 to +2.9, p=0.002). Compared with usual care, exercise training improved peak VO by +2.9 mL/kg/min (95% CI +1.2 to +4.5, p=0.002), corresponding to a 10% increase. Adaptations to exercise training included an increase in resting cardiac output (+0.8 L/min, p=0.042), total blood volume (+713 mL, p<0.001), total haemoglobin mass (+73 g, p<0.001), and improved left ventricular (LV) systolic function (LV ejection fraction: +3.2% (p=0.053) and global longitudinal strain: -2.0% (p=0.044)). No exercise-related adverse events or change in plasma biomarkers of cardiac or skeletal muscle damage were observed.

CONCLUSIONS

Our study shows that vigorous intensity exercise training improved peak VO in patients with DCM-TTNtv. Exercise training was associated with improved LV systolic function and increased blood volume and oxygen carrying capacity. Future research should investigate the effect of long-term exercise in this group.

TRIAL REGISTRATION NUMBER

NCT05180188.

摘要

背景

建议慢性心力衰竭患者定期参加运动活动。然而,对于由截断的肌联蛋白变异(TTNtv)引起的扩张型心肌病(DCM)患者的运动效果知之甚少。我们旨在研究剧烈强度训练对 DCM-TTNtv 患者身体能力的影响。

试验设计

非随机临床前后试验的运动训练。

方法

从遗传性心脏病门诊纳入 DCM-TTNtv 患者。试验包括 8 周的常规护理,随后是 8 周的定期剧烈强度自行车运动,期间包括 3 个测试日。主要结局是峰值摄氧量(VO)的变化。次要结局包括血容量、总血红蛋白质量、运动时收缩功能和心输出量/每搏量的变化。

结果

14 名入选患者中有 13 名(43%女性,年龄 48±11 岁,体重指数:30±6kg/m)完成了试验。在运动训练期间,峰值 VO 增加了+1.9ml/kgmin(95%可信区间:0.9 至 2.9,p=0.002)。与常规护理相比,运动训练使峰值 VO 增加了+2.9ml/kg/min(95%可信区间:1.2 至 4.5,p=0.002),对应增加了 10%。运动训练的适应包括静息心输出量增加(+0.8L/min,p=0.042)、总血容量增加(+713ml,p<0.001)、总血红蛋白质量增加(+73g,p<0.001)和左心室(LV)收缩功能改善(LV 射血分数:+3.2%(p=0.053)和整体纵向应变:-2.0%(p=0.044))。未观察到与运动相关的不良事件或心脏或骨骼肌损伤的血浆生物标志物变化。

结论

我们的研究表明,剧烈强度运动训练可提高 DCM-TTNtv 患者的峰值 VO。运动训练与 LV 收缩功能改善以及血容量和携氧能力增加有关。未来的研究应调查该组患者长期运动的效果。

试验注册号

NCT05180188。

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