在儿童期被诊断患有高性心肌病的患者的左心室缩功能障碍:来自SHaRe注册表的见解
在PubMed上查看摘要
概括
此摘要是机器生成的。被诊断为高性心肌病 (HCM) 的儿童比成年人更容易出现左心室缩功能障碍 (LVSD). 由于LVSD的预后不佳,需要谨慎监测,特别是在过渡到成人护理期间.
科学领域
- 心脏病学
- 儿童心脏病学
- 遗传学
背景情况
- 左心室静脉功能障碍 (LVSD) 是成年人高性心肌病 (HCM) 的罕见但严重并发症,与不良结果相关.
- 有限的数据存在于患有HCM的儿科患者的LVSD的患病率,预测因素和预后.
研究的目的
- 在儿童时期被诊断患有HCM的患者中调查LVSD的患病率,预测因素和预后.
- 将儿童诊断的HCM与成人诊断的HCM的发展和结果进行比较.
主要方法
- 来自国际多中心SHaRe (萨尔科默里克人类心肌病注册表) 数据的分析.
- 定义为左心室喷射率<50%. 通过死亡,心脏移植或左心室辅助装置的复合评估预后.
- 用于评估事件LVSD和预后的Cox比例危险模型.
主要成果
- 研究了1010名儿科HCM患者 (诊断时的中位数年龄为12. 7岁). 在5. 5年的中位随访期间,5. 5%的患者患有LVSD,9. 1%的患者患有LVSD.
- 在儿童HCM中,总体LVSD患病率为14. 7%,成人HCM患病率为8. 7%. 与成人群体相比,儿童群体的LVSD发病率较早 (平均年龄为32. 6岁).
- 儿科HCM中LVSD的预测因素包括诊断时的年龄较小,男性性别,致病性瘤变体,先前的隔膜缩小疗法和较低的初始射出分数. 40% 的儿科 LVSD 患者达到复合结局,女性的预后较差,排泄分数<5%.
结论
- 儿童时被诊断出HCM的患者比成年时被诊断出LVSD的患者有更高的生命期风险.
- 无论诊断时或发病时的年龄,HCM中的LVSD预后不佳.
- 在儿科HCM患者中,对LVSD进行密切监测至关重要,尤其是在他们转入成人护理期间.
相关概念视频
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...
Systolic Heart Failure and Compensatory MechanismsSystolic heart failure (also termed HFrEF, Heart Failure with Reduced Ejection Fraction) is the most prevalent type of heart filure. It results in a decreased volume of blood being pumped from the ventricle. The aortic arch and carotid sinuses have baroreceptors that detect reduced blood pressure, triggering the sympathetic nervous system (SNS) to release epinephrine and norepinephrine. Initially, this response aims to boost heart rate and...
Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
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...
Mitral regurgitation is characterized by the backward circulation of blood from the left ventricle to the left atrium during systole, a phase of the cardiac cycle when the heart contracts and pumps blood out of the chambers. This abnormal flow occurs primarily due to the dysfunction of the mitral valve or its supporting structures, which include the mitral leaflets, chordae tendineae, annulus, and papillary muscles.Etiology and Mechanisms:Primary Mitral Regurgitation: This type arises from...

