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.
Study Type
OBSERVATIONAL
Enrollment
1,500
Patients will be followed by the registry for life-long outcomes.
The Hospital for Sick Children
Toronto, Ontario, Canada
RECRUITINGProportion 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
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
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.