Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1.
Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1.
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DOI：
10.1161/jaha.119.015709
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发表时间：
2020-09
影响因子：
5.4
通讯作者：
Matumura T
中科院分区：
文献类型：
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作者：
Itoh H;Hisamatsu T;Tamura T;Segawa K;Takahashi T;Takada H;Kuru S;Wada C;Suzuki M;Suwazono S;Sasaki S;Okumura K;Horie M;Takahashi MP;Matumura T
1 型强直性肌营养不良涉及心脏传导障碍。心脏传导疾病可导致 1 型强直性肌营养不良患者出现致命性心律失常或猝死。本研究纳入了 506 名 1 型强直性肌营养不良患者（年龄≥15 岁；>50 个胞嘧啶-胸腺嘧啶-鸟嘌呤重复），并于 2006 年 1 月至 2016 年 8 月在 9 家日本神经肌肉疾病医院接受治疗。我们调查了遗传和临床背景，包括医疗保健、日常活动等。随访期间的生活、饮食摄入、心脏受累和呼吸受累。死亡原因或复合心脏事件（即室性心律失常、晚期房室传导阻滞和装置植入）的发生被评估为重要结果。在 87 个月的中位随访期间（第一季度至第三季度，37–138 个月），71 名患者死亡。在单变量分析中，起搏器植入（风险比 [HR]，4.35；95% CI，1.22-15.50）与猝死相关。相比之下，PQ 间期≥240 毫秒、QRS 时限≥120 毫秒、营养或呼吸衰竭与猝死的发生率无关。多变量分析显示，PQ 间期≥240 ms（HR，2.79；95% CI，1.9–7.19，P<0.05）或 QRS 持续时间≥120 ms（HR，9.41；95% CI，2.62-33.77，P < 0.01）是与 PQ 间期观察到的心脏事件发生率较高相关的独立因素<240 毫秒或 QRS 持续时间 <120 毫秒；这些心脏传导参数与猝死无关。心脏传导障碍是与心脏事件相关的独立标志。有必要对猝死发生的预测进行进一步研究。
Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1. This study enrolled 506 patients with myotonic dystrophy type 1 (aged ≥15 years; >50 cytosine‐thymine‐guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow‐up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow‐up period of 87 months (Q1–Q3, 37–138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22–15.50) were associated with sudden death. In contrast, PQ interval ≥240 ms, QRS duration ≥120 ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval ≥240 ms (HR, 2.79; 95% CI, 1.9–7.19, P<0.05) or QRS duration ≥120 ms (HR, 9.41; 95% CI, 2.62–33.77, P < 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval <240 ms or QRS duration <120 ms; these cardiac conduction parameters were not related to sudden death. Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.