Fetal Ebstein Anomaly and Tricuspid Valve Dysplasia Registry
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 1,500
- 试验地点
- 1
- 主要终点
- Proportion of live-born children who survive to 30 days
研究概览
简要总结
Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) are rare congenital tricuspid valve malformations that carry among the highest mortality of all congenital heart disease diagnosed in utero. Despite the high mortality associated with severe EA/TVD in the fetus, it has only been studied retrospectively. By prospectively enrolling a cohort across multiple centers, many questions may be answered in the perinatal period and beyond. The registry will allow us to understand perinatal and postnatal decision-making in this complex group of patients across centers.
Given the rarity of the disease, a retrospective arm was added to the original prospective study in May 2024.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Mothers of fetuses (ages 14-54) diagnosed with EA/TVD of any severity with normal segmental anatomy (AV and VA concordance)
- •Mothers may be enrolled at any gestational age, up to the day of pregnancy outcome (elective termination of pregnancy, demise, or live-birth)
- •Singletons, twins or higher order multiples may be included
- •Consent obtained at a participating site
排除标准
- •Mothers of fetuses diagnosed with EA/TVD in the context of abnormal segmental anatomy (AV and/or VA discordance) or other lesions, such as congenitally corrected transposition of the great arteries or pulmonary atresia with intact ventricular septum
- •Unable or unwilling to provide consent
结局指标
主要结局
Proportion of live-born children who survive to 30 days
时间窗: 0-30 days
Proportion of fetuses who survive to live-birth at term
时间窗: 37 0/7 to 40 0/7 weeks gestation
Freedom from death beyond the neonatal period
时间窗: 30 days-30 years
次要结局
- Change in umbilical artery pulsatility index throughout gestation(20 0/7 to 40 0/7 weeks gestation)
- Average gestational age at birth(At birth)
- Proportion who undergo neonatal cardiac surgery(0-30 days)
- Proportion with hydrops throughout gestation(20 0/7 to 40 0/7 weeks)
- Average birth weight(At birth)
- Freedom from cardiac re-intervention(30 days-30 years)
- Pediatric neurodevelopment as assessed by Adaptive Behavior Assessment System(3-18 years)
- Infant neurodevelopment as assessed by Bayley Scales of Infant Development(12-15 months)
- Quality of life as assessed by Peds QL General and Cardiac Modules(2-30 years)
- Freedom from cardiac arrhythmia(30 days-30 years)
研究者
Lindsay Freud
Head of Fetal Cardiology
The Hospital for Sick Children