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

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

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

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

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 (2009-2011)

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

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

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

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

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

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

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

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

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 2015 - 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

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

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

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)

Many Innovative Resources at the Local and Regional Level

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

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

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

What It’s All About http://www.state.nj.us/governor/news/news/552011/approved/20111109a.html

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)

BEGINNING OF EXTRA SLIDES

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

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