1. Ebstein Anomaly Cohort: Update
Research Team
Joseph Dearani
Chris Knott-Craig
Christian Pizarro
Kimberly Holst
Jim Meza
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2. EA Cohort Update
Status Update
Brief Cohort Review
Next Steps
Timeline for Patient Enrollment
Planning ahead, facilitating site initiation

3. Since the last Work Weekend:
Sick Kids’ REB Approval!
Research Protocol
Data Collection Forms
Foundation of Echo CORE Lab

4. Cohort Overview: Research Questions
Classification System
Optimal Approach (timing, operation)
Neonate
Infant
Child
Early morbidity and mortality
Late morbidity, reoperation, mortality
Late functional status

5. Cohort
Inclusion Criteria: Ebstein anomaly dx at CHSS member institution
Neonate to 21 years old
Neonate ≤ 1 month
Pediatric > 1 month
Operative and medically managed patients
Exclude:
AV-VA discordance
HCM, PAPVC, AVSD, genetic syndromes
Retrospective (Look Back to 2010) to Prospective

6. Imaging Impacts all aspects of this cohort
Crucial to be accurate and uniform
Echocardiogram
Core Lab
Pre-op TTE, intra-op TEE, post-op TTE
2D and 3D when available
Fetal Echocardiograms when available
MRI
Need standard interpretation

7. 1. Classification System
Neonates (GOSE vs other)
Fetal?
Pediatric patients
Correlation of anatomy:
Echo
MRI
Surgery
Predictors of valve repairability

8. 1. Classification System, cont
To establish prognostic models based on classification system
RF for mortality prior to surgery (neonates)
Predictors of successful medical management
RF for mortality related to surgery
Models by age

9. 2. Optimal Management Neonates Current practice patterns
Timing of operative intervention
Patient anatomy
Physiology
Operative Approach
1V vs 2V
Risk factors
Transplant
Mortality

10. 2. Optimal Management, cont.
Pediatric patients
Current practice patterns
Timing of operative intervention
Physiology, symptoms
Patient anatomy
Operative Approach
1.5V vs 2V
Predictors of valve repairability
Optimal anti-arrhythmia procedures

11. 3. Early morbidity and mortality
Describe early outcomes
Identify operative and perioperative RF
Early morbidity
Early mortality
Determine modifiable risk factors to improve early outcome

12. 4. Late morbidity, reop, and mortality
Describe and determine predictors
Late arrhythmia
Right ventricular dysfunction
Exercise intolerance
Re-hospitalization
Reoperation
Risk factors
Risk profile and outcomes: Reoperation vs. primary operation
Transplant
Late survival

13. 5. Late functional status
Accurately describe late functional status
Exercise tests
Quality of life
Identify predictors of better/worse function
Operative
Medication
Arrhythmia management

14. Questions regarding Research Protocol and/or Research Questions?

15. Moving Forward: Needed Steps BEFORE enrollment:
Imaging Protocol
Refinement of Data Collection Forms
Database Construction
Training Materials
Imaging
Study Coordinators
Site Initiation Roll-Out
Training
IRB/REB

16. Imaging Protocol
Establishment of Echo CORE group of Echocardiographers
Welcome of additional cardiologists
Establish Protocols:
Image collection
Data Collection Forms
What images to review
Fetal Echocardiograms when available
Will require Sick Kids REB amendment

17. Data Collection Forms and Database Construction
Review and make updates if needed
Data Center Review
Feasible to include in the database
Database Construction
Research Coordinator Review
Feasible to send from individual institutions
Changes will be submitted to Sick Kids REB

18. Training Materials and Roll-Out
Imaging Team
CORE team
Institutional imaging teams
Study Coordinators
Study Enrollment and Protocol Training
Data Collection
IRB/REB Submission

19. Updated Timeline October 2016: Initial REB Approval Now: 1-3 months
Establish CORE Lab and needed protocols
Refine DCFs if needed
Develop Training Materials
REB Modifications: 2-3 months to review
Database Build: 1 month
Institutional Roll-Out: 1-2 months
Regulatory process: 2-3 months
Patient Enrollment: 6-9 months ..