1. CDC Site Visit at Emory CHD Surveillance Cooperative Agreement Clinical & Case Finding September 25, 2013 Wendy Book, MD

2. Sibley Heart Center William Mahle, MD Sibley Heart Center William Mahle, MD Organizational Schema Children’s Healthcare of Atlanta Brian Kogan, MD Children’s Healthcare of Atlanta Brian Kogan, MD Emory University Hospital Wendy Book, MD Anita Saraf, MD, PhD Emory University Hospital Wendy Book, MD Anita Saraf, MD, PhD Emory University Hospital Midtown William Mahle, MD Emory University Hospital Midtown William Mahle, MD Grady Memorial Hospital Allen Dollar, MD Neils Engberding, MD Grady Memorial Hospital Allen Dollar, MD Neils Engberding, MD Emory St. Joseph’s Hospital Mann Jokhadar, MD Emory St. Joseph’s Hospital Mann Jokhadar, MD Pediatric Cardiology Services Neill Videlefsky, MD Pediatric Cardiology Services Neill Videlefsky, MD Emory Bioinformatics Lisa Codgill Jeff Weaver Kenyon Register Andreas Kalogeropoulos Emory Bioinformatics Lisa Codgill Jeff Weaver Kenyon Register Andreas Kalogeropoulos Surveillance Database Metro Atlanta Congenital Defect Program Georgia Department of Health Population Database Emory University Rollins School of Public Health Carol Hogue, PhD, MPH Michael Kramer, PhD Cheryl Raskind-Hood, MS, MPH Emory University Rollins School of Public Health Carol Hogue, PhD, MPH Michael Kramer, PhD Cheryl Raskind-Hood, MS, MPH Principal Investigator Wendy Book, MPH Co-Investigators William Mahle, MD Brian Hogan, MD Carol Hogue, PhD, MPH Maan Jokhadar, PhD Michael Kramer, PhD, MPH Cheryl Raskind-Hood, MS, MPH Principal Investigator Wendy Book, MPH Co-Investigators William Mahle, MD Brian Hogan, MD Carol Hogue, PhD, MPH Maan Jokhadar, PhD Michael Kramer, PhD, MPH Cheryl Raskind-Hood, MS, MPH

3. Goals of RFA dd12-1207 1.Determine the prevalence of CHDs among a population- based sample of adolescents and adults in the U.S.; 2.Describe the health care utilization and direct medical care costs for adolescents and adults with CHDs; 3.Describe the distribution of disease severity, co-morbidities, and longer term outcomes of adolescents and adults with CHDs; 4.Describe any racial/ethnic or socioeconomic disparities in healthcare use or long-term outcomes; 5.Monitor the health outcomes in special populations such as pregnant women with CHDs; 6.Calculate the survival and life expectancy for persons with specific types of CHDs; 7.Describe the availability of specialty care and referral patterns that are currently in place; 8.Assist with public health planning.

4. “Because there is no population-based surveillance of CHD across the lifespan in the United States, no prevalence data are available on children, adolescents, and adults living with CHD.” Congenital Heart Public Health Consortium (2012)

5. Adult & Children Living with CHD: Prevalence Across the Lifespan 2000 (U.S.) Estimates 800,000 adults 600,000 children 2010 (extrapolation based on Canadian data) to U.S. Census data in 2010 2 million people (all ages) were possibly living with CHD in the United States, approximately: – 975,000 to 1.4 million children – 959,000 and 1.5 million adults. Note. Data from Congenital Heart Public Health Consortium, 2012.

6. What’s different about Georgia? Georgia > 3% U.S. population Atlanta metro area has grown significantly since 1960’s - now represents > 1% of the U.S. population However, same training structure remains – one major medical school in Atlanta, one major CTS training program, one major cardiology program NY & MA - multiple programs Note. Data from U.S. Census 2010

7. Metropolitan Atlanta Congenital Defects Program (MACDP) Established 1967 by CDC, Emory University and GMHI Covers a 5-county metropolitan Atlanta Population of about 2.9 million, with 51,600 annual births

8. Five-County Demographics by Age, Race, Ethnicity & CHD

9. Racial Distribution for CHD Consortium Sites Caucasian African- American Asian Georgia62.8%31.2%3.5% Massachusetts83.7%7.9%5.8% New York71.2%17.5%8.0% U.S.77.9%13.1%5.1%

10. Provisions of Outpatient Pediatric CHD Care Outpatient – CHOA outpatient, a.k.a. Sibley Heart Center, has 44 cardiologists at 19 offices – Pediatric Cardiology Services: 4 cardiologists – 4 other pediatric cardiologists Lower CHD capture – Medical College of Georgia (Augusta) 6 pediatric cardiologists

11. Provisions of Outpatient Pediatric CHD Care Augusta Atlanta Medical College of Georgia

12. Inpatient Pediatric Care in Georgia Children’s Healthcare of Atlanta (3 hospitals) – >85% of care in state, majority of all care in metro Atlanta No other children’s hospital in metro Atlanta One other provider of inpatient pediatric care – Medical College of Georgia (Augusta)

13. CHOA: Cardiac Surgical Volume by Age

14. How Many CHD Surgeries Conducted in Augusta? PediatricAdultTotal 2008220 20091027109 20101183121

15. Pediatric * Cardiac Surgical Volume by Site 2008-2010 * up to age 21 ** Medical College of Georgia *** Children’s Healthcare of Atlanta Site# (%) MCG ** 242 (8.6%) CHOA *** 2545 (91.4%)

16. Adult Congenital Heart Care in Georgia One Adult Congenital Heart Center in Georgia – Emory Healthcare (Emory HC) Emory HC network is largest provider of cardiac care in Georgia Model for transition – No patients >21 yrs. followed in the pediatric program

17. Other Providers of Adult CHD Care Emory Healthcare includes St. Joseph’s Grady Health and Grady Memorial Hospital (GMH) - more than 36,000 cardiology visits annually Numerous private practices – Referral pattern for Adult CHD care is to Emory

18. Emory Cardiac Surgical Volume (Adult) Hospital200820092010 Emory University Hospitals – Midtown10521003884 Emory University Hospital 790867759 Wellstar Kennestone Hospital 565527635 Athens Regional Hospital180300352 Total 258726972630

19. Overview of Emory Healthcare

21. FY 2008FY 2009FY 2010FY 2011FY 2012 FY2013 Annualized NEW PATIENT VISITS 199326276284290338 PROCEDURES4766826728429221,199 RETURN PATIENT VISITS 8641,0421,1821,5581,7101,875 GRAND TOTAL1,5392,0502,1302,6842,9223,412 EHC system sees 1.5 million visits per year Nearly 250,000 unique new patients each year EHC provides 170,000 cardiology visits per year Emory Adult Congenital Heart Center Visit Numbers EHC Visits

22. # patients Age Distribution, ACHD Clinic

23. Data Sources Metropolitan Atlanta Congenital Defects Program (MACDP) – Linked with National Death Index (NDI) Children’s Healthcare of Atlanta (CHOA) Sibley Heart Center (Sibley) Pediatric Cardiology Services (PCS) Emory Healthcare (EHC) Grady Health & Grady Memorial (GMH) CMS Medicaid Data Georgia Vital Records

24. Data Sources 2008-2010 Emory Healthcare – clinical electronic medical (eMR) records (current started 2009) and archived data available (2008) Grady Health – clinical electronic records (current started 2011) and archived electronic records (2008-2010 available) Sibley – Current and archived data available Pediatric Cardiology Services - electronic records CMS Medicaid (Children’s Medical Service – previously CCS - program provided care for CHD patients up until age 21 in a Medicaid expansion program. This data was not reported to CMS. Program ended 2009) MACDP with NDI – NDI linkage currently being revisited GA Vital Records (Death Certificates) – provided by GA Dept. of Public Health and maintained at CDC

25. Georgia Population Density Clinical Surveillance Counties Georgia Population Density & Counties

26. Pregnancy Plan Patient will be identified through billing codes for cardiac disease in pregnancy, cross-referenced with codes for CHD Existing pregnancy data can be de- identified and shared, but referral bias due to clinical research set at tertiary center Maternal mortality review (Georgia)

27. Surgeries, Procedures & Other Studies Procedures – “less invasive” repairs, typically done in the cath lab in cardiology Diagnostic Cath Imaging studies (MRI, CT, Echo) EKG Map to CPT/ICD9 codes, use STS grouping?

28. Uninsured & Medicaid Patient Numbers EHC Sibley/CHOA Adolescent 11-21 years Grady Health Overall 4% uninsured, 5% Medicaid overall ACHD 8% uninsured, 11% Medicaid <5% uninsured 47% Medicaid 53% Uninsured 14% Medicaid

29. Data: Progress to Date Data acquired from Sibley, CHOA, EHC data warehouse Archived EHC data received, being reviewed IRB protocol submitted, approval is pending final DUA approvals DUA approval required for Grady Health data Meeting held with Pediatric Cardiology Services, expect data in next month

30. 1.Second natural history study of congenital heart defects Circulation 1993 2.Wacker A. Outcomes of operated and unoperated adults with congenital cardiac disease lost to follow up for more than five years. Am J Cardiol 2005. Challenge #1: “Out of Care” In the “Natural History Study”, 40% of patients with AS/PS/VSD had not had a cardiac examination in over 10 years. 1 Of 10,500 adults with congenital heart disease in Germany, 8,028 were lost to follow up for more than 5 years. 2

31. Canadian Experience Reid. Prevalence and Correlates of Successful Transfer From Pediatric to Adult Health Care Among a Cohort of Young Adults With Complex Congenital Heart Defects Pediatrics 2004 Of adults with congenital heart disease transitioning to level 3 care, >25% had not had a single evaluation after their 18 th birthday.

32. Gaps in Care: HEART-ACHD Project 922 subjects from 12 ACHD centers. A >3 year lapse in care identified in 42% (25% w/ severe) 8% having lapses >10 years. Mean age at first lapse was 19.9 years Subjects were highly educated with 73% having more than high school education. Gurvitz M, Valente A, et al. Prevalence and predictors of gaps in care. J Am Coll Cardiol. 2013 May 28;61(21):2180-4

33. Challenge #2: Uninsured National Inpatient Hospital Database (1998-2004 & 2004-2010) 60% of persons between 2004-2010 had public insurance or were uninsured (O'Leary, Siddiqi, de Ferranti, Landzberg, & Opotowsky, 2013) ? accessibility of medical care to patients with public insurance or those who are uninsured ? increasing utilization of hospital care

34. Self-Care Trends in ACHD Between 40-60% of adults are not engaged in routine care up to 85% do not meet physical activity recommendations Dua, Cooper, Fox, & Graham Stuart, 2007 At least 20% of women do not attend pre- pregnancy counseling despite high rates of maternal and fetal complications Bowater et al., 2012 Drenthen et al., 2007 Gurvitz, M., Verstappen, A., Valente, A.M, Broberg, C., Cook, S., Stout, K., Kay, J., Ting, J., Kuehl, K., Earing, M., Webb, G., Opotowsky, A., Graham, D., Khairy, P., Landzberg, M., 2011

35. Challenge #3: ICD-9CM Codes for Case Definitions Misuse of codes 745.5 codes for ASD and for normal variant PFO (roughly 25-30% of population) In adults, PFO often picked up incidentally on echo for other indications 745.4 codes for VSD. Post-myocardial infarction VSD may also be under this code (1% of fatal MI) – 552K MI fatalities per year in U.S. – MI 208 per 100,000 in 2008

36. Blue Babies

37. The Problem: Where Are They Now?? I am 745.10

38. Transposition of Great Vessels: Mustard or Senning Palliation Welton M. Gersony, CHD

39. 1969: Balloon septostomy for dTGA 1973: “Mustard” procedure, atrial switch (no code) 1990: Hospitalization: Sick sinus syndrome (427.81), Pacemaker placed (33206) 1994: Hospitalization: Heart Failure (428.0) 1999: Pregnancy complicated by heart failure (648.5) 2003: Atrial flutter (427.32) 2009: Sudden Death, Death Certificate: (I46.2) This is What 745.10 Might Look Like in an Administrative Database

40. How Do We Group CHDs? 3 categories – Minor, Major, CHD with co-morbidities Healthcare Utilization – By Age – By Severity Suggestions: group by utilization & co- morbidities instead

41. Status of Project Administration: Accomplishments To-Date Collaboration Weekly meetings since Fall 2012 Team - Emory Congenital Heart Center & Rollins School of Public Health Project Staffing 1 Biostatistician (for Y3) 1.5 Project Manager (prev. 2.2 PMs) 3 MPH Graduate students – knowledgeable in CHD terminology, trained by in-house physicians Contracts & Validation IRB Approval - for Data Collection & Data Sharing DUA In Progress - Pediatric Cardiology Services DUA Completed – Grady Health, CHOA, & St. Joseph’s Hospital ResDAC- Submitted & currently under review LexisNexis Approval