Echocardiographic and electrocardiographic findings of cardiomyopathy in Duchenne and Becker-Kiener muscular dystrophies
Echocardiographic and electrocardiographic findings of cardiomyopathy in Duchenne and Becker-Kiener muscular dystrophies
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DOI：
10.1007/s00246-004-0689-2
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发表时间：
2005-01-01
影响因子：
1.6
通讯作者：
Dittrich, S
中科院分区：
文献类型：
--
作者：
Kirchmann, C;Kececioglu, D;Dittrich, S
我们在我们单中心机构的大型门诊组中评估了Duchenne肌营养不良症(DMD)和Becker-Kiener肌营养不良症(BMD)患者的心脏表现。分析包括修改患者记录(回顾)和当前的超声心动图、心电图和更完整的心电数据(前瞻性)。24%的患者左室短轴缩短率为25%(50年DMD，12.1+/-4.7岁：20BMD，17.1+/-8.5岁)。DMD和BMD分别为16.8+/-1.0和30.4+/-3.4(P<；0.05)。DMD患者动态心电图显示窦性心动过速占26%，昼夜节律丧失占31%，心率变异性降低占51%。出于这些原因，我们建议每年在DMD和BMD>；10年进行超声心动图检查。由于DMD患者心脏自主神经系统的障碍率较高，我们也推荐每年进行动态心电图检查。应该进行进一步的研究，以确定联合使用β-受体阻滞剂和血管紧张素转换酶抑制剂是否可以减少心脏自主神经失衡，从而改善DMD患者的预后。
We assessed the cardiac findings in Duchenne muscular dystrophy (DMD) and Becker-Kiener muscular dystrophy (BMD) patients in the large outpatient group of our single-center institution. The analysis included the revision of patient records (retrospectively) and current echocardiography, electrocardiogram (ECG), and Holier ECG data (prospectively). Reduced left ventricular fraction shortening (FS) < 25% was found in 24% of all patients (50 DMD, 12.1 +/- 4.7 years: 20 BMD, 17.1 +/- 8.5 years). Median age of onset of FS < 25% was 16.8 +/- 1.0 in DMD and 30.4 +/- 3.4 in BMD; (p < 0.05). Holter ECG in DMD demonstrated sinus tachycardia in 26% deprivation of circadian rhythm in 31%, and reduced heart rate variability in 51%. For these reasons, we recommend carrying out echocardiography annually in DMD and BMD > 10 years. Because the rate of disturbances in the cardiac autonomic nervous system is high in DMD, we also recommend Holter ECG annually. Further investigation should be conducted to determine if treatment with beta-blockers and ACE inhibitors in combination reduces cardiac autonomic nervous imbalance, thus improving patient outcome in DMD.