家族性st段抑郁综合征的心电图表现、心律失常和左心室受累

A. Christensen, C. Vissing, A. Pietersen, J. Tfelt‐Hansen, Thomas Hartvig Lindkær Jensen, S. Pehrson, F. Henriksen, N. Sandgaard, K. Iversen, H. Jensen, M. Olesen, H. Bundgaard
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引用次数: 5

摘要

背景:家族性st段抑制综合征是一种以持续、非缺血性st段偏离、心律失常和心力衰竭风险为特征的遗传性疾病。我们的目的是进一步表征ECG、心律失常和与这种新综合征相关的结构特征。方法:回顾性分析丹麦家族性st -抑郁综合征连续家庭的资料。分析心电图特征、心律失常的发生率和类型、收缩功能障碍的发生情况及中期预后。结果:40例患者(43%为男性;诊断时平均年龄49.1岁,来自14个明显不相关且患者≥2人的家庭。所有家族均存在常染色体显性遗传。心电图表型似乎是在青春期前形成的,st段偏离分别在V4/V5/II导联中持续且最明显。连续心电图分析显示,心电图表型的进展稳定到缓慢。运动加重了导联V5的st差,休息/应激差(平均值)最大为- 117 μV。在平均9.3±7.1年的随访期间,5例(13%)患者发生持续性室性心律失常或(流产)心源性猝死,10例(25%)发生房颤,2例(5%)发生其他室上性心律失常,10例(25%)被诊断为左心室射血分数≤50%。室性心律失常具有多态性,发病时(300-360 ms)伴有相对短时间耦合的室性早搏;未见QT延长。7例患者至少有一次导管消融;室上性心律失常5次,室性心律失常2次。男性出现的心律失常终点多于女性(P<0.01)。结论:经中期随访,家族性st段抑郁的心电图表型稳定到缓慢进展。临床上,上室性心律失常和室性心律失常都很常见;还有一定程度的左心室收缩功能障碍。家族性st段抑制是多形性室性心动过速的一种新的遗传原因。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Electrocardiographic Findings, Arrhythmias, and Left Ventricular Involvement in Familial ST-Depression Syndrome
Background: Familial ST-depression syndrome is an inherited disease characterized by persistent, nonischemic ST-deviations, and risk of arrhythmias and heart failure. We aimed at further characterizing the ECG, arrhythmias, and structural characteristics associated with this novel syndrome. Methods: Retrospective analysis of data from consecutive families with familial ST-depression Syndrome in Denmark. ECG features, prevalence and type of arrhythmias, occurrence of systolic dysfunction, and medium-term outcome were analyzed. Results: Forty affected individuals (43% men; mean age at diagnosis 49.1 years) from 14 apparently unrelated families with ≥2 affected members were included. Autosomal dominant inheritance was observed in all families. The ECG phenotype seemed to develop in prepuberty and the ST-deviations were persistent and most pronounced in leads V4/V5/II, respectively. Serial ECG analyses showed stable to slow progression of the ECG phenotype. Exercise accentuated the ST-deviations with a maximum difference between rest/stress (mean) of −117 μV in lead V5. During a mean follow-up of 9.3±7.1 years 5 (13%) patients developed sustained ventricular arrhythmias or (aborted) sudden cardiac death, 10 (25%) developed atrial fibrillation, 2 (5%) other supraventricular arrhythmias, and 10 (25%) were diagnosed with left ventricular ejection fraction ≤50%. The ventricular arrhythmias were polymorphic with relatively short-coupled premature ventricular contractions at onset (300–360 ms); no QT prolongation was observed. Seven patients had at least one catheter ablation; 5 for supraventricular arrhythmias and 2 for ventricular arrhythmias. Males experienced more arrhythmic end points than females (P<0.01). Conclusions: The familial ST-depression ECG phenotype is stable to slowly progressive after medium-term follow-up. Clinically, both supra- and ventricular arrhythmias are common; as are some degree of left ventricular systolic dysfunction. Familial ST-depression represent a novel inherited cause of polymorphic ventricular tachycardia.
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