Fetal Ebstein Anomaly and Tricuspid Valve Dysplasia Registry
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Observation.
- Who it may be relevant to
- Registry conditions: Ebstein Anomaly, Tricuspid Valve Dysplasia. Basic parameters: No limits · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Canada
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Overview
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.
Interventions
- Other Observation
Patients will be followed by the registry for life-long outcomes.
Primary outcome measures
- Proportion of fetuses who survive to live-birth at term [Time frame: 37 0/7 to 40 0/7 weeks gestation]
- Proportion of live-born children who survive to 30 days [Time frame: 0-30 days]
- Freedom from death beyond the neonatal period [Time frame: 30 days-30 years]
Secondary outcome measures (10)
- Change in umbilical artery pulsatility index throughout gestation [Time frame: 20 0/7 to 40 0/7 weeks gestation]
- Proportion with hydrops throughout gestation [Time frame: 20 0/7 to 40 0/7 weeks]
- Average gestational age at birth [Time frame: At birth]
- Average birth weight [Time frame: At birth]
- Proportion who undergo neonatal cardiac surgery [Time frame: 0-30 days]
- Freedom from cardiac re-intervention [Time frame: 30 days-30 years]
- Freedom from cardiac arrhythmia [Time frame: 30 days-30 years]
- Infant neurodevelopment as assessed by Bayley Scales of Infant Development [Time frame: 12-15 months]
- Pediatric neurodevelopment as assessed by Adaptive Behavior Assessment System [Time frame: 3-18 years]
- Quality of life as assessed by Peds QL General and Cardiac Modules [Time frame: 2-30 years]
Eligibility criteria
Inclusion criteria
- 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
Exclusion criteria
- 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
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
Canada · 1 center
- The Hospital for Sick Children — Toronto
Identifiers
NCT: NCT05225311 · 1000076991