International Bicuspid Aortic Valve Consortium (BAVCon)
试验速览
- 阶段
- 不适用
- 状态
- 进行中（未招募）
- 入组人数
- 10,000
- 试验地点
- 12
- 主要终点
- Bicuspid aortic valve disease
研究概览
简要总结
Bicuspid aortic valve (BAV) disease is the most frequent congenital cardiac malformation, occurring in 0.5-1.2% of the US population. In young adults, it is generally a benign abnormality; but in older adults it is associated with thoracic aortic aneurysm or dissection in 20-30% of those with BAV. BAV is strongly associated with early development of aortic valve calcification or incompetence in >50% of BAV patients, and accounts for ~40% of the >30,000 aortic valve replacements (AVR) performed in the US each year. Yet, we know little of the etiology, cellular events and modifiers of progression of BAV to calcific aortic valve disease and we still do not understand the genetic cause(s) of BAV despite evidence for its high heritability.
The Specific Aims of this study are:
- To identify the genetic causes of bicuspid aortic valve disease and its associated thoracic aortic disease.
- To identify potential pathways to predict the clinical course of BAV disease and for treating human BAV disease.
To achieve these aims, we have created the International Bicuspid Aortic Valve Consortium (BAVCon), a consortium of institutions with cohorts of BAV patients and the expertise to fulfill the performance of these aims.
详细描述
The International Bicuspid Aortic Valve Consortium (BAVCon) Registry is the data collection arm of the International Bicuspid Aortic Valve Consortium, a 16 institution international consortium of investigators with interests in the genetics, molecular biology, imaging, surgery and natural history of bicuspid aortic valve disease.
The BAVCon Registry is a longitudinal cohort study, which is observational by design. The cohort will consist of patients with BAV. Consideration will be given to eventual inclusion of a disease-free control or comparison group. The study will compare genetic risk factors, cross-sectional and longitudinal data on risk factors related to the diagnosis, treatment and outcomes among groups of enrolled patients. As part of the natural course of clinical care, the patients and their physicians will determine the approach to treatment and the study will record the observed related outcomes. The study will not attempt to interfere with the outcomes through any type of planned intervention; therefore, there are no anticipated adverse events as a result of study participation.
Study Design
The Registry is designed to capture information on patients with bicuspid aortic valve disease. The BAVCon Registry population will be comprised of patients from sixteen BAVCon Clinical Centers. The participating BAVCon Clinical Centers are:
- Boston University School of Medicine, Boston (Dr. Simon Body, Consortium PI)
- GenTAC Consortium (Dr. Kim Eagle, PI)
- Hospital Vall d'Hebron, Barcelona, Spain (Dr. Arturo Evangelista, PI)
- Monaldi Hospital, Naples, Italy (Dr. Giuseppe Limongelli, PI)
- Mayo Clinic, Rochester (Dr. Hector Michelena, PI)
- Oxford University, Oxford, England (Dr. Malenka Bissell, PI)
- San Donato Hospital IRCCS, Milan, Italy (PI: Dr. Alessandro Frigiola)
- Tufts University, Boston (Dr. Gordon Huggins, PI)
- Université Laval, Quebec (Dr. Yohan Bossé, PI)
- University of Michigan, Ann Arbor (Dr. Bo Yang, PI)
- University of Salerno, Salerno, Italy (Dr. Eduardo Bossone, PI)
- University of Texas Medical School, Houston (Dr. Dianna Milewicz, PI) One center with specific expertise, but not enrolling patients, is University of Ottawa (Dr. Mona Nemer, PI).
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 8 Years 至 90 Years（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients diagnosed as having a bicuspid aortic valve
- •All ages ≥8 years
- •Able to provide fully informed consent
排除标准
- 未提供
结局指标
主要结局
Bicuspid aortic valve disease
时间窗: 10 years
The first specific aim is to identify the genetic causes of bicuspid aortic valve disease.
次要结局
- The development of thoracic aortic disease in patients with bicuspid aortic valve disease(10 years)