相关实验视频
Updated: Sep 10, 2025

06:33
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
7.8K
在轻链和跨氨酸粉症中的心声风险分层:元分析
David Koeckerling1, Rohin K Reddy2,3, Christian Eichhorn4
1Department of Cardiology, Angiology and Pulmonology, Heidelberg University Hospital, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany.
European heart journal open
|August 25, 2025
概括
心声学有效预测心脏粉症的死亡率. 左心室全球纵向应变和射出分数等关键指标为患者风险分层提供了重要的预后信息.
科学领域:
- 心脏病学
- 医学成像
- 基因组学
背景情况:
- 心脏粉症的回声心脏成像的预后价值在当前的国际指导方针中没有得到明确的定义.
- 心脏粉症,包括轻链 (AL) 和跨素 (ATTR) 类型,显著影响患者的结果.
研究的目的:
- 进行一项元分析,评估心声学测量结果与AL和ATTR粉样性病的临床结果之间的关联.
- 确定特定心声学参数在心脏粉症患者死亡率预测中的作用.
主要方法:
- 在MEDLINE,Embase,Cochrane图书馆和谷歌学者的系统文献搜索到2024年7月.
- 包括94项研究,包括16158名患者 (4788名AL,8241名ATTR,3129名混合),分析心声学变量 (LV-GLS,LVEF,TAPSE,IVSd,LVMi,E/ e) 与不良事件的关系.
- 随机效应模型用于所有原因死亡率的危险比率 (HR),数据由两位独立审查员提取.
主要成果:
- 左心室全球纵向应变 (LV-GLS) 的1% 降低与较高的全因死亡风险相关 (HR,1. 10).
- 增加左心室喷射分数 (LVEF) 和三环平面缩 (TAPSE) 显示出较低的全因死亡风险 (HRLVEF,0. 98;HRTAPSE,0. 94).
- 较高的E/ e'比率 (每1单位) 与所有原因死亡率的增加相关 (HR,1.02),而结构参数 (IVSd,LVMi) 与结果没有可靠的关联.
结论:
- 反映双心室变形和功能的心声参数与粉样性病的死亡率有着一致的联系.
- 结构性心声学参数与临床结果没有可靠的关联.
- 在心脏粉症患者中,回声心脏学具有显著的初始风险分层潜力.
相关概念视频
Heart Failure IV: Classification and Diagnostic Evaluation
32
Heart failure can be classified in various ways, with the most common classifications based on physical activity limitations, disease progression, severity, and treatment strategies.The Functional Classification of Heart Failure divides patients into four categories based on physical activity limitation due to symptom burden.Class I: Patients in this class have cardiac disease but no physical activity limitations. Ordinary activities like walking, climbing stairs, or routine tasks do not cause...
32
Acute Coronary Syndrome III: Diagnostic Studies
23
Diagnosing acute coronary syndrome or ACS begins with a thorough patient history. Notable symptoms include central, crushing chest pain radiating to the left arm, neck, jaw, or back, along with shortness of breath, sweating (diaphoresis), nausea, vomiting, dizziness, and palpitations.It is crucial to note any history of cardiac illnesses and assess risk factors, including age, gender, smoking, hypertension, diabetes, hyperlipidemia, and a sedentary lifestyle.During physical examination, vital...
23
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
57
The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
57
Cardiomyopathy III: Hypertrophic Cardiomyopathy
47
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...
47
Cardiomyopathy IV: Restrictive Cardiomyopathy
28
Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
28
Atherosclerosis II: Clinical Manifestations and Diagnostic Tests
38
Atherosclerosis is a progressive disorder that leads to the thickening and narrowing of arterial walls due to plaque buildup. This condition can cause various symptoms depending on the arteries affected:Coronary Artery Disease (CAD): This condition affects the coronary arteries and may lead to chest pain (angina), shortness of breath (dyspnea), heart attacks, and other heart disease symptoms.Cerebrovascular Disease: This affects blood flow to the brain, causing transient ischemic attacks (TIAs)...
38

