Risk Stratification in Children and Adolescents With Primary Cardiomyopathy
试验速览
- 阶段
- 不适用
- 发起方
- 入组人数
- 200
- 试验地点
- 2
- 主要终点
- major cardiovascular events
研究概览
简要总结
RIKADA is a prospective study performing systematic family screening including clinical and genetic testing in pediatric patients with primary cardiomyopathy and their first-degree relatives with the aim to facilitate risk stratification.
详细描述
RIKADA is a long-term prospective study performing in-depth phenotype and genotype characterization in children and adolescents with primary cardiomyopathy and their first-degree family members. Family screening contains complete cardiac work-up with medical history, physical examination, 12-lead-/Holter-electrocardiogram, cardiopulmonary exercise testing, echocardiography, cardiovascular magnetic resonance (CMR) and laboratory including genetic testing. The aim is to facilitate early identification of at-risk individuals and contribute to patient-specific follow-up and therapy regimes preventing progressive heart failure and arrhythmia in pediatric CMP.
研究设计
- 研究类型
- Observational
- 观察模型
- Family Based
- 时间视角
- Prospective
入排标准
- 年龄范围
- — 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Index patients:
- •Age ≤18 years
- •written informed consent of parents/legal guardians
- •diagnosis of primary cardiomypathy:
- •DCM: left ventricular (LV) systolic dysfunction and dilatation greater than two standard deviations (SD) above the mean of a normal population
- •HCM: LV hypertrophy and septal wall thickness above two SD
- •RCM: diastolic dysfunction and concordant atrial enlargement
- •LVNC: separation of the myocardium into a compacted (C) and a non- compacted (NC) layer with an NC/C ratio >2 in echocardiography and/or >2.3 in CMR
- •ARVC: according to the revised Task Force Criteria
- •First-degree family members (parents and siblings):
- •Age ≥3 years
- •written informed consent of parents/legal guardians and siblings ≥18 years
排除标准
- •unwillingness to give consent
- •myocardial inflammation / myocarditis
- •systemic disease with cardiac involvement (secondary cardiomyopathy)
- •structural congenital heart disease
结局指标
主要结局
major cardiovascular events
时间窗: from date of enrollment until the date of death, mechanical circulatory support or heart transplantation, assessed up to 8 years
death, need for mechanical circulatory support or heart transplantation
次要结局
未报告次要终点