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The Chinese Hypertrophic Cardiomyopathy Study(CHCS)

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China National Center for Cardiovascular Diseases

关键词

抽象

Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiac diseases, with a prevalence of ∼0.2%. Sudden cardiac death (SCD), heart failure and stroke are the major poor outcomes of HCM. Although about half of the patients were found to be caused by mutations mainly located in genes encoding sarcomere proteins, the causes in a significant proportion of patients with HCM are still unknown. Even in the patients with sarcomere mutations, the molecular pathways that eventually lead to cardiac hypertrophy are remained to be revealed. Furthermore, HCM presents with significant heterogeneity. SCD risk stratification and prevention by ICD are necessary. However, the strategy of SCD risk stratification recommended by the 2011 ACCF/AHA and 2014 ESC guidelines were based mainly on the evidence derived from American and European countries. The accuracy of these guidelines in Chines patients with HCM was not evaluated yet.

描述

In the present study, patients with HCM are recruited prospectively after informed consent was given. The investigator will collect the baseline clinical characteristics of the patients at enrollment, including comprehensive physical examination, laboratory testing of blood and urine, electrocardiography, 24-hr Holter, echocardiography, MRI and other examinations if necessary. The specimens retained include blood for all patients and myocardium for patients receive surgery of myocardial myectomy. Multi-omics screening, including genomics, epigenomics, transcriptomics, proteomics, metabolomics, will be performed to identified novel disease genes, signal pathway or processes, genetic risk factors and potential therapy targets of HCM. Furthermore, besides examining the accuracy of SCD risk prediction of the 2011 ACCF/AHA and 2014 ESC guidelines, we will identify novel risk factors associated with the clinical outcomes and construct predictive models suitable for Chinese patients with HCM.

日期

最后验证: 02/28/2018
首次提交: 01/25/2016
提交的预估入学人数: 02/24/2016
首次发布: 03/01/2016
上次提交的更新: 03/23/2018
最近更新发布: 03/26/2018
实际学习开始日期: 03/22/2011
预计主要完成日期: 12/30/2025
预计完成日期: 12/30/2028

状况或疾病

Cardiomyopathy, Hypertrophic

-

手臂组

干预/治疗
Hypertrophic cardiomyopathy
Individuals with an unexplained maximal left ventricle wall thickness ≥15 mm on echocardiography and/or cardiac magnetic resonance imaging or or ≥13 mm for individuals with family history of HCM, in the absence of other cardiac or systemic diseases capable of producing that magnitude of cardiac hypertrophy.

资格标准

有资格学习的性别All
取样方式Non-Probability Sample
接受健康志愿者
标准

Inclusion criteria: Patients with HCM diagnosed by observation of unexplained maximal left ventricle wall thickness ≥15 mm on echocardiography and/or cardiac magnetic resonance imaging or or ≥13 mm for individuals with family history of HCM.

Exclusion criteria: Individuals with other cardiac or systemic diseases capable of producing that magnitude of cardiac hypertrophy.

结果

主要结果指标

1. Cardiovascular deaths [an average of 2 years]

Including SCD and deaths due to heart failure and stroke.

次要成果指标

1. all-cause mortality [an average of 2 years.]

deaths due to all causes.

2. Heart failure [an average of 2 years]

Progress to level III or IV in New York Heart Association class.

3. Stroke [an average of 2 years]

including cerebral infarction and hemorrhage

4. Malignant arrhythmia [an average of 2 years]

The incidence rate of malignant arrhythmia

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