Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1
Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1
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DOI：
10.1056/nejmoa062800
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发表时间：
2008-06-19
影响因子：
158.5
通讯作者：
Pascuzzi, Robert M.
中科院分区：
文献类型：
--
作者：
Groh, William J.;Groh, Miriam R.;Pascuzzi, Robert M.
背景：1型肌强直性营养不良症患者心脏传导异常可导致猝死。猝死风险的决定因素仍然不明确。方法对406例经基因证实的1型肌强直性营养不良成年患者进行心电图（ECG）预测猝死的应用价值评估。如果心电图至少具有以下特征之一，则认为患者存在严重异常：非窦性心律，PR间期240毫秒或更长，QRS持续时间120毫秒或更长，或二度或三度房室传导阻滞。结果入路心电图重度异常患者年龄大于非重度异常患者，骨骼肌损伤更严重，发生心力衰竭、左室收缩功能障碍、房性心动过速的可能性更大。在随访期间，这些患者更有可能接受起搏器或植入式心律转复除颤器。在平均5.7年的随访期间，81例患者死亡；27例猝死，32例死于进行性神经肌肉呼吸衰竭，5例死于心脏原因的非猝死，17例死于其他原因。在17例猝死患者中，有9例出现室性心动过速。严重心电图异常（相对危险度为3.30,95%可信区间[CI]为1.24 ~ 8.78）和临床诊断为房性心动过速（相对危险度为5.18,95% CI为2.28 ~ 11.77）是猝死的独立危险因素。结论成人1型强直性肌营养不良患者是心律失常和猝死的高危人群。心电图的严重异常和房性心动过速的诊断预示着猝死。
Background Sudden death can occur as a consequence of cardiac- conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise.Methods We assessed whether the electrocardiogram (ECG) was useful in predicting sudden death in 406 adult patients with genetically confirmed myotonic dystrophy type 1. A patient was characterized as having a severe abnormality if the ECG had at least one of the following features: rhythm other than sinus, PR interval of 240 msec or more, QRS duration of 120 msec or more, or second-degree or third-degree atrioventricular block.Results Patients with severe abnormalities according to the entry ECG were older than patients without severe abnormalities, had more severe skeletal-muscle impairment, and were more likely to have heart failure, left ventricular systolic dysfunction, or atrial tachyarrhythmia. Such patients were more likely to receive a pacemaker or an implantable cardioverter-defibrillator during the follow-up period. During a mean follow-up period of 5.7 years, 81 patients died; there were 27 sudden deaths, 32 deaths from progressive neuromuscular respiratory failure, 5 nonsudden deaths from cardiac causes, and 17 deaths from other causes. Among the 17 patients who died suddenly in whom postcollapse rhythm was evaluated, a ventricular tachyarrhythmia was observed in 9. A severe ECG abnormality (relative risk, 3.30; 95% confidence interval [CI], 1.24 to 8.78) and a clinical diagnosis of atrial tachyarrhythmia (relative risk, 5.18; 95% CI, 2.28 to 11.77) were independent risk factors for sudden death.Conclusions Patients with adult myotonic dystrophy type 1 are at high risk for arrhythmias and sudden death. A severe abnormality on the ECG and a diagnosis of an atrial tachyarrhythmia predict sudden death.