Fetal Ebstein Anomaly and Tricuspid Valve Dysplasia Registry

Study Overview
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Brief Summary
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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.

Official Title
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Fetal Ebstein Anomaly and Tricuspid Valve Dysplasia Registry

Conditions
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Ebstein Anomaly, Tricuspid Valve Dysplasia

Intervention / Treatment
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* Other: Observation


Participation Criteria
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Eligibility Criteria
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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
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients with Fetal Ebstein's Anomaly or Tricuspid Valve Dysplasia<br>Patients will be followed for life-long outcomes. | Other: Observation<br>* Patients will be followed by the registry for life-long outcomes.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Proportion of fetuses who survive to live-birth at term |  | 37 0/7 to 40 0/7 weeks gestation | 
| Proportion of live-born children who survive to 30 days |  | 0-30 days | 
| Freedom from death beyond the neonatal period |  | 30 days-30 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in umbilical artery pulsatility index throughout gestation |  | 20 0/7 to 40 0/7 weeks gestation | 
| Proportion with hydrops throughout gestation |  | 20 0/7 to 40 0/7 weeks | 
| Average gestational age at birth |  | At birth | 
| Average birth weight |  | At birth | 
| Proportion who undergo neonatal cardiac surgery |  | 0-30 days | 
| Freedom from cardiac re-intervention |  | 30 days-30 years | 
| Freedom from cardiac arrhythmia |  | 30 days-30 years | 
| Infant neurodevelopment as assessed by Bayley Scales of Infant Development |  | 12-15 months | 
| Pediatric neurodevelopment as assessed by Adaptive Behavior Assessment System |  | 3-18 years | 
| Quality of life as assessed by Peds QL General and Cardiac Modules |  | 2-30 years |