NCT05225311

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. Given the rarity of the disease, a retrospective arm was added to the original prospective study in May 2024.

Trial Health

77
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
1,500

participants targeted

Target at P75+ for all trials

Timeline
357mo left

Started Sep 2021

Longer than P75 for all trials

Geographic Reach
1 country

1 active site

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress14%
Sep 2021Sep 2055

Study Start

First participant enrolled

September 22, 2021

Completed
4 months until next milestone

First Submitted

Initial submission to the registry

January 6, 2022

Completed
29 days until next milestone

First Posted

Study publicly available on registry

February 4, 2022

Completed
28.6 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

September 1, 2050

Expected
5 years until next milestone

Study Completion

Last participant's last visit for all outcomes

September 1, 2055

Last Updated

May 31, 2025

Status Verified

May 1, 2025

Enrollment Period

29 years

First QC Date

January 6, 2022

Last Update Submit

May 27, 2025

Conditions

Ebstein AnomalyTricuspid Valve Dysplasia

Outcome Measures

Primary Outcomes (3)

  • 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 Outcomes (10)

  • 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

  • +5 more secondary outcomes

Study Arms (1)

Patients with Fetal Ebstein's Anomaly or Tricuspid Valve Dysplasia

Patients will be followed for life-long outcomes.

Other: Observation

Interventions

ObservationOTHER

Patients will be followed by the registry for life-long outcomes.

Patients with Fetal Ebstein's Anomaly or Tricuspid Valve Dysplasia

Eligibility Criteria

Sexall
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Fetuses diagnosed with EA/TVD of any severity with normal segmental anatomy (AV and VA concordance)

You may qualify if:

  • 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

You may not qualify if:

  • 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

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

The Hospital for Sick Children

Toronto, Ontario, M5G1X8, Canada

RECRUITING

MeSH Terms

Conditions

Ebstein Anomaly

Interventions

Observation

Condition Hierarchy (Ancestors)

Heart Defects, CongenitalCardiovascular AbnormalitiesCardiovascular DiseasesHeart DiseasesCongenital AbnormalitiesCongenital, Hereditary, and Neonatal Diseases and Abnormalities

Intervention Hierarchy (Ancestors)

MethodsInvestigative Techniques

Central Study Contacts

Lindsay Freud, MD

CONTACT

416-813-7500lindsay.freud@sickkids.ca

Diana Balmer-Minnes, BSc

CONTACT

416-813-7654diana.balmer-minnes@sickkids.ca

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Head of Fetal Cardiology

Study Record Dates

First Submitted

January 6, 2022

First Posted

February 4, 2022

Study Start

September 22, 2021

Primary Completion (Estimated)

September 1, 2050

Study Completion (Estimated)

September 1, 2055

Last Updated

May 31, 2025

Record last verified: 2025-05

Data Sharing

IPD Sharing
Will not share

Locations

CA(1)