1. CCHD Screening using Pulse Oximetry in the US: An Amazing Public Health Story
Lorraine Freed Garg, MD, MPH
Consultant in Public Health
October 14, 2016

2. Critical Congenital Heart Defects
Approximately 9 in 1,000 live births with congenital heart defects (CHD); 25% are critical congenital heart defects (CCHD)
Up to 25% of newborns with CCHD could be missed at the time of hospital discharge
Between 100 and 200 newborns are estimated to have died each year from missed CCHD
Significant morbidity due to delayed diagnosis
Mahle WT et al., Circulation 2009
Hokanson JS, Neonatology Today 2010

3. Detection of CCHD 60% Prenatal 20% Clinical 15.6% Pulse Ox 4.4%
Diagnostic Gap
Riede FT et al., Eur J Pediar 2010

4. CCHD Screening with Pulse Oximetry
Can detect mild hypoxemia without apparent cyanosis
Non-invasive and widely available
Meta-analysis: 13 eligible studies; 229,421 newborns
Sensitivity: 76.5% (95% CI 67.7 – 83.5)
Specificity: 99.9% (95% CI 99.7 – 99.9)
False-positive rate: 0.14% (95% CI 0.06 – 0.33)
Cost assessment
CDC Study in 7 NJ Birthing facilities
Mean estimated cost/ newborn screened: $14.19
$7.36 in labor costs
$6.83 in equipment and supply costs
Thangaratinam et al., Lancet 2012 ;
Peterson et al., Public Health Reports 2014

5. Timeline of CCHD Screening with Pulse Oximetry
SACHDNC voted to add to RUSP (2010)
Early literature
CHD Screening to added to RUSP (2011)
Tennessee Task Force against mandate (2005)
1995
1999
2003
2007
2011
AAP and AHA statement (2009)
Legislation in IN, NJ, MD
Large European Studies ( )

6. AAP CCHD Screening Map: 2011 State Actions on CCHD
3 states enacted legislation
Source: American Academy of Pediatrics State Government Affairs

7. AAP CCHD Screening Map: States’ Actions During 2011-2012 (As of December 17, 2012)
Source: American Academy of Pediatrics State Government Affairs

8. AAP CCHD Screening Map: States’ Actions During 2013 (As of September 16, 2013)

9. AAP CCHD Screening Map: States’ Actions During 2014 (As of October 20, 2014)
Source: American Academy of Pediatrics State Government Affairs

10. AAP CCHD Screening Map: States’ Actions During 2016 (As of August 9, 2016)

11. Multiple Stakeholders
Universal CCHD Screening
Family Advocates
Researchers
Legislators
Health Departments
State and Federal Government
Health Care Providers
Professional Organizations
Advocacy Organizations

12. Eve and Annamarie Mpls St Paul Magazine, July 2016
Twin Cities.com, July 2016

13. New Jersey’s Law
“The Commissioner of Health and Senior Services shall require each birthing facility licensed by the Department of Health and Senior Services to perform a pulse oximetry screening, a minimum of 24 hours after birth, on every newborn in its care.”
NJ first state to implement a mandate for pulse oximetry screening
Legislation signed into law June 2, 2011
Implementation date August 31, 2011
P.L. 2011, Chapter 74

14. Data Collection Goal Multi-Pronged Approach
Screening results on all births
Detailed information on failed screens
Multi-Pronged Approach
Screening information- all births
Vital Information System (VIP)
2011 – planning
implemented
Quarterly aggregate data –interim measure
Information on Failed Screens
Birth Defects Registry
Information about screening result and outcome
Relevant clinical information to evaluate contribution of screening to detection

15. Results of NJ Screening
August 31, 2011–December 31, 2014_______ Live births 338,124 Live births eligible to be screened* 328,591 Live births screened 327,447 Eligible live births screened 99.7%
*Excludes expirations, <24 hours, not medically appropriate at the end of the period

16. Evaluating the Unique Contribution of CCHD Screening: Failed Screens Registered to NJBDR 8/31/11 - 6/30/16
Total fails
N= 295
Pre-Identified Factors
Prenatal diagnosis of CCHD
Signs/symptoms at the time of the screen
Cardiac consult or echocardiogram planned prior to the screen
>=1 Pre-identified Factor
N= 162
No Pre-identified Factors
N= 133
CCHD
N= 25
CHD
N= 19
PFO/PDA Only
N= 37
Other significant medical conditions
N= 10

17. Importance of Training and Education
Provider Training
NJ Recommended Screening Algorithm
Quick Reference Guide
Birth Defects Registry Worksheet
NJ CCHD Screening Reference Guide
Grand Round Presentations
Online course for nurses
Parent Information (6 languages)

18. Many Innovative Resources at the Local and Regional Level

19. Screening Special Populations State-Led Activities
Out of Hospital births
WI, PA, MI, NJ mid-wife outreach and education
High Altitudes
CO, AZ, UT-adjustments to algorithm at high altitude
NICU
Multi-State Collaboration led by NJDOH and NJ AAP
5 States: California, Illinois, Minnesota, New Jersey, New York
Objectives:
Determine exclusion criteria for screening in NICU
Suspected prenatal CHD diagnosis
Echocardiogram before screening
Extreme prematurity
Burden of screening in the NICU at different times
Data forthcoming

20. National Level Involvement
Federal Agencies
HHS/HRSA
Addition of CCHD screening to the RUSP
6 Implementation Grants
NewSTEPS Technical Assistance Center
National CCHD Technical Assistance Webinars
Data repository
CDC
Research and economic evaluations
Support link between BDR and CCHD screening
Supporting states and health departments
Professional Organizations
AAP
AHA
March of Dimes

21. Challenges Remain
No current Federal funding for health departments (SHINE Legislation)
Special populations
Data collection not standardized
Not all states collecting data
Data reporting not uniform

22. What It’s All About

23. Acknowledgements New Jersey Department of Health Pulse Ox Team
NJ Chapter American Academy of Pediatrics
Centers for Disease Control and Prevention
New Jersey CCHD Screening Working Group
NJ’s Birthing Facilities
CCHD Screening Work Supported by:
Grant # H46MC24059 from the Genetic Services Branch
Division of Services for Children with Special Health Needs, Maternal and Child Health Bureau, Health Resources and Services Administration (HRSA)

25. BEGINNING OF EXTRA SLIDES

26. CCHD Screening Targets
Seven Primary Targets
Five Secondary Targets
Hypoplastic left heart syndrome
Coarctation of the aorta
Pulmonary atresia (intact ventricular septum)
Double outlet right ventricle
Tetralogy of Fallot
Ebstein anomaly
Total anomalous pulmonary venous return
Interrupted aortic arch
Transposition of the great arteries
Single ventricle
Tricuspid atresia
Truncus arteriosus

29. Detected Through CCHD Screening with Pulse Oximetry
Diagnostic evaluation attributable to POxS (n=133)
25 - CCHD
19 - CHD
10 - Other significant non-cardiac conditions
37 - PDA or PFO as only finding
22 - Identified during hospital quality control activities
20 – No documented reason for failed POxS