左心室缩是长QT综合征患者危及生命的节律失常事件的新型预测因子
Jing Yang1,2, Kun Li1, Fang Liu1
1Department of Cardiology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua Medicine, Tsinghua University, Beijing, 102218, China.
Orphanet journal of rare diseases
|December 23, 2025
概括
左心室高血压 (LVHT) 是长QT综合征 (LQTS) 患者危及生命的节律失常事件 (LAE) 的新型预测因素. 早期预防策略对于管理LQTS患者的LVHT至关重要,以避免心脏事件.
科学领域:
- 心脏病学 心脏病学
- 遗传学 是一个遗传学.
- 心脏电生理学 心脏电生理学
背景情况:
- 长QT综合征 (LQTS) 是一种遗传性心脏通道病变.
- 左心室超 (LVHT) 是LQTS患者罕见的发现.
- 结合LQTS和LVHT可能会增加心脏不良事件的风险.
研究的目的:
- 为了确定LQTS患者中LVHT的患病率.
- 在LQTS患者中分析LVHT和危及生命的心律失常事件 (LAE) 之间的关联.
主要方法:
- 研究了170名基因型确诊的LQTS患者的队列.
- 患者被分为具有LVHT (LQTS-LVHT) 和没有LVHT (LQTS-N-LVHT) 的LQTS组.
- 危及生命的心律失常事件 (LAE) 被定义为昏迷,心室低心率 (VT),ICD冲击或突然心脏死亡.
主要成果:
- 4.7%的LQTS患者 (8/170) 患有LVHT.
- 在霍尔特监测 (25.0%与2.7%) 上,LQTS-LVHT患者的心室动 (VF) 发病率较高.
- 在平均随访52.8个月后,LVHT是LAE的显著独立预测因子 (HR:3.02,P=0.015).
结论:
- 在LQTS患者中,LVHT是危及生命的节律失常事件的新型预测剂.
- 有效的预防性治疗对于LQTS-LVHT患者来说至关重要,以减轻心脏风险.
相关概念视频
ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias
416
Arrhythmia is a condition characterized by an irregular heart rhythm, with ECG changes that differ based on its origin and nature. The types of arrhythmias discussed below include atrial, junctional, and ventricular arrhythmias.Atrial ArrhythmiasPremature Atrial Complexes (PACs): PACs are early atrial beats caused by stress, caffeine, alcohol, electrolyte imbalances, hypoxia, hyperthyroidism, or certain medications (e.g., bronchodilators and decongestants). The ECG shows early P waves with an...
416
Dysrhythmias V: Evaluating Dysrhythmias
288
Dysrhythmias, also known as arrhythmias, are disturbances in the heart's rhythm that range from benign to life-threatening. A thorough evaluation is crucial for appropriate management and involves a comprehensive medical history, physical examination, and various diagnostic tests.Medical HistorySymptoms: Collect detailed information on palpitations, dizziness, syncope, chest pain, and fatigue. Note their onset, frequency, and triggers.Previous Cardiac Issues: Document any history of heart...
288
Dysrhythmias II: Classification of Tachyarrhythmias
457
Tachyarrhythmias are a type of dysrhythmia where the heart rate exceeds 100 beats per minute. Here are some common types of tachyarrhythmias:Sinus TachycardiaSinus tachycardia originates from increased impulses from the sinus node, leading to an elevated heart rate. It is often triggered by stress, fever, or exercise.Patients may experience palpitations, a sensation of a racing heart, dizziness, and chest discomfort.Causes and Risk Factors: Common causes include physical exertion, emotional...
457
Cardiomyopathy III: Hypertrophic Cardiomyopathy
360
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
360
Dysrhythmias III: Characteristics of Dysrhythmias
368
Dysrhythmias, also known as arrhythmias, are irregular heart rhythms that result from abnormal electrical activity in the heart, affecting its ability to circulate blood efficiently. Tachyarrhythmias, a subset of dysrhythmias, are characterized by abnormally fast heart rates exceeding 100 beats per minute. Here are some types of tachyarrhythmias with their distinct ECG features:Sinus Tachycardia:Sinus tachycardia presents a regular heart rhythm with an increased rate of 101-180 beats per...
368
Disturbances in Heart Rhythm
2.4K
Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
2.4K


