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AAOCA: Predictors of Ischemia
Study Design
CHSS Work Weekend
November 18-20th
Julie Brothers, M.D.
Medical Director, Coronary Anomaly Management Program
The Children’s Hospital of Philadelphia and
Perelman School of Medicine at the University of Pennsylvania
Marshall Jacobs, M.D.
Director of Pediatric Cardiac Surgery Outcomes Research
Johns Hopkins School of Medicine
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2. CURRENT REGISTRY STATUS

3. Participating Centers
39 Member Institutions

4. AAOCA Cumulative Enrollment & Number of Participating Institutions
We now have 39 institution that are enrolling into AAOCA. Do you want me to take out the red line for the enrolling institutions?
Enrollment numbers for 2016 are as of October 11, 2016,

5. Patient Characteristics
N (percent of current enrollment)
Current Enrollment
541
Alive
529 (97.8%)
Average age at diagnosis
10.3 +/- 6 years
Male gender
358 (66%)
Prospectively enrolled
419 (77.4%)
Surgical
300 (55.4%)
As of Nov
current numbers 541 enrolled
529 alive
12 dead One new deceased pt 9 years old elective repair LMCA went on ecmo
13 aborted sudden death
Males %
females 183
Prospective %
Retrospective 122
surgery
Yes %
No 241

6. Hypothesis/Goal
There will be differences in morphologic characteristics in patients with AAOCA who experience SCA or present with other evidence of myocardial ischemia
Sub-cohort analysis of patients who have had well-documented episodes of myocardial ischemia or significant arrhythmia
Goal to risk stratify patients based on a combination of clinical history and details of coronary morphology

7. Working Definition of Ischemia
Sudden cardiac arrest or death
Documented complex ventricular arrhythmia
Exercise test
J-point depression at least 2 mm or ST segment depression > 1 mm with flat/downsloping ST segment at 60 msec
Increasing ventricular arrhythmias with exercise
Lack of BP response
Imaging
Stress echo or nuclear scan with WMA or perfusion defect in correct coronary distribution territory

8. Summary of Ischemic Patients
Description
Number
Documented Ischemia 29
Coronary Anatomy
AAORCA 14
AAOLCA
Both 1
Male Gender 25
Surgical Patients 24
Aborted Sudden Death 12 4
8 (one from non-coronary sinus)
I don’t have numbers yet Julie but I know we have many more “true” ischemic patients enrolled.
A few more aborted sudden death patients
one new dead who died after OR.
I just screened one patient who’s father was Dx at age 35 with MI and had surgery, this patient also had surgery. Not sure if I can get any information on the fathers anomaly,
Definition of “ischemia”: 1 or more positive ischemia tests (among 210 patients tested) or presentation consistent with ischemia or lethal arrhythmia

9. Aborted Sudden Death Patients
Status
Coronary anomalous
Symptoms
Age at Diagnosis (yrs)
Age at time of Death (yrs)
Gender
Aborted SCD, died post-operatively on ECMO
AAOLCA
Arrythmia, MI, ECMO. To OR for unroofing 1 6 F
Aborted SCD, died in CCU
CPR V-fib AED x2 14 M
Alive post witness arrest
Arrest post cross country run, CPR and AED 12
N/A
AAOLCA (from non-coronary sinus)
V-Fib with exercse, AED
CPR x1min 16
Arrest with basketball, Vfib arrest, Defib x 2
CPR, AED for V fib 13
Arrest CPR 10
Alive s/p witnessed arrest
AAORCA
CPR AED applied and cardioverted 18
 N/A
arrest post cross country run, CPR and AED
syncope in car, CPR briefly 11
Arrest playing basketball, AED
Median age at diagnosis
13.8

10. 210 patients underwent ischemia testing, 181 negative
Among 181 non-ischemic patients, 76% had AAORCA
Among 29 ischemic patients, AAORCA = AAOLCA
Variables other than just anomalous coronary laterality are predictors of ischemia
These appear to be more prevalent with AAOLCA than AAORCA

11. ICL ECHO Review n=58 29 Ischemic patients 29 Non-Ischemic patients
Age Matched
29 Non-Ischemic patients
20 studies previously reviewed
38 to be reviewed
Made this slide up not sure if its helpful or not.
Shubhika Craig and Luc
Reviewer 1 =17
Reviewer 2 =18
Reviewer 3 =19
8 studies reviewed by all 3

12. Ischemic Group Studies Available N=58
Total N= patient
CT study report in Database
MRI study report in Database
# of CT-CD's available
# of MRI-CD's available
N=Patients with Both studies done
N= Patients with Both have CD's for review
Ischemic group 29 14 8 6 4
Non-Ischemic group 19 11 3 2 1
Total 58 33 12 25 9

13. Image Review: Echo
Among 201 echos received for patients with ischemic testing, 87 have had core lab (ICL) review and 114 have not been reviewed
*=died without echo
ICL Review
No ICL Review
Unavailable *
Ischemic Patients 8 20 1
Non-Ischemic 79 94
Total 87
114

14. CT/MRI 121 have other imaging modalities NOT reviewed
Issues with limited data sets sent to the CHSS DC for reading CTA/MRI

15. Questions/Issues
Do we want to/need to review all of the echos in the cohort?
Are the CT/MRI data necessary (I say yes)
Should we attempt to get the full dataset from each institution?
Is there anything else we should be considering?

16. Acknowledgments Thank you very much
The CHSS members and CHSS Data Center
Susan McIntyre, Clinical Research Nurse Coordinator, Data Center
All of the participating AAOCA patients and their families
The Registry has been generously funded by:
Michael H. Ludwig Memorial Foundation
Anthony’s Heroes for Hearts
Children’s Heart Foundation
Cardiac Center at the Children’s Hospital of Philadelphia
Children’s Mercy Hospital, Kansas City
Cardiovascular and Critical Care Research Center, Children’s Hospitals and Clinics of Minnesota
Cardiac Surgery Research and Teaching Fund, Hospital for Sick Children, Toronto