Fetal Ebstein Anomaly and Tricuspid Valve Dysplasia Registry

Recruiting

• Conditions: Ebstein Anomaly; Tricuspid Valve Dysplasia
• Interventions: Other: Observation

• Registration Number: NCT05225311
• Lead Sponsor: The Hospital for Sick Children

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2021-09-22
• Study First Submitted: 2022-01-06
• Study First Submitted QC: 2022-01-25
• Study First Posted: 2022-02-04
• Status Verified: 2024-01
• Last Update Submitted: 2024-01-26
• Last Update Posted: 2024-01-29
• Primary Completion: 2050-09
• Study Completion: 2055-09

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 1500

Inclusion Criteria

1. Mothers of fetuses (ages 14-54) diagnosed with EA/TVD of any severity with normal segmental anatomy (AV and VA concordance)

   1. Mothers may be enrolled at any gestational age, up to the day of pregnancy outcome (elective termination of pregnancy, demise, or live-birth)
   2. Singletons, twins or higher order multiples may be included

2. Consent obtained at a participating site

Exclusion Criteria

1. 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
2. Unable or unwilling to provide consent

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Patients with Fetal Ebstein's Anomaly or Tricuspid Valve Dysplasia	Observation	Patients will be followed for life-long outcomes.

Primary Outcome Measures

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

Secondary Outcome Measures

Name	Time	Method
Average gestational age at birth	At birth
Proportion who undergo neonatal cardiac surgery	0-30 days
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 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

Trial Locations

Locations (1)

The Hospital for Sick Children; 🇨🇦 Toronto, Ontario, Canada