Although current pulse-oximetry technology cannot detect every cardiac defect, the rate of missed CCHD diagnoses is much higher when screening is based on physical examination alone.

Health-care providers and families also must understand limitations of pulse-oximetry monitoring to detect CCHD, that a negative screening result does not exclude the possibility of CCHD or other congenital heart disease, and that there is a potential for false-positive screen results.1


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Implementation of screening programs

According to the SACHDNC recommendations, it is the responsibility of individual states to develop and implement screening protocols to enhance the detection of CCHD.7 State departments of public health will lead comprehensive efforts to standardize screening practices, for instance, by disseminating screening protocols to all hospitals and birthing centers in their jurisdiction.

States will also perform ongoing data surveillance to monitor CCHD screening rates and the appropriateness of follow-up care in newborns with positive screens.

As of September 1, 2012, nine states — California, Connecticut, Indiana, Maryland, New Hampshire, New Jersey, Tennessee, Virginia, and West Virginia — had passed legislation mandating CCHD screening for newborns in their states.12 Another 18% of states had legislation introduced, and 10% had legislation pending.

Case Study: New Jersey

In 2011, the New Jersey Department of Health and Senior Services (NJDHSS) signed legislation requiring each birthing facility licensed by the NJDHSS to perform pulse-oximetry screening on every newborn in its care.11 Lorraine Freed Garg, MD, MPH, Medical Director of Newborn Screening and Genetic Services, NJDHSS, recently described New Jersey’s experience with CCHD screening.

The NJDHSS law was signed on June 2, 2011, and became effective on August 31, 2011. In preparation for launching the statewide screening program, the NJDHSS convened the Critical Congenital Heart Disease Screening Working Group to develop a recommended screening protocol (Figure 1). In addition, to train clinicians in CCHD screening, the NJDHSS distributed protocols, communicated with hospitals, identified pulse-oximetry contacts and each birthing facility, and conducted educational webinars.

The NJDHSS screening initiative includes surveillance to collect data related to CCHD screening, including the total number of births and the total number of screens. Discrepancies between total births and total screens in each quarterly reporting period should be explained as follows: infant death; transferred out of the birthing center at <24 hours of age; not medically appropriate; <24 hours of age at end of current reporting period; and born in prior reporting period. In addition, the NJDHSS Birth Defect Registry will compile data on all failed screens, including symptom history, upper-extremity (UE) and lower-extremity (LE) pulse-oximetry readings x 3, prenatal ultrasound and postnatal echocardiogram results, and final diagnosis explaining the failed screen.

In November 2011, the NJDHSS conducted a preliminary survey of 52 hospitals and 18 birthing facilities to assess initial efforts related to protocol implementation. Most facilities were utilizing the NJDHSS screening protocol and found no major barriers to successful implementation. Within the first 90 days of the CCHD screening program, more than 95% of infants underwent pulse-oximetry screening according to the NJDHSS protocol.11

The challenges were primarily in the areas of infrastructure and cost. For instance, while 83% of hospitals had the ability and expertise to conduct echocardiograms in newborns, 17% did not have this on-site capability.11 One hospital noted the burden of having to purchase an additional pulse oximeter as well as disposable wraps for reusable sensors, which were not accounted for in initial cost estimates.11 Hospitals also identified the need for educational materials and more intensive training to support clinicians across the spectrum of newborn care. While the mechanisms for data collection and surveillance were praised overall, some participants faced a steep learning curve related to data reporting.11 Any up-front errors may adversely affect the accuracy of surveillance data and should be addressed as part of ongoing quality-assurance efforts.

The NJDHSS attributed the successful implementation to the Critical Congenital Heart Disease Screening Working Group, dedicated hospital staff, and strong connections with birthing facilities. Follow-up surveys with participating hospitals and birthing centers should identify additional areas for improvement in CCHD screening.

Figure 1. New Jersey Department of Health and Senior Services

pulse-oximetry screening algorithm for CCHD

States including Tennessee, Massachusetts, Arizona, California, Iowa, Montana, and Washington have initiated pilot multicenter screening programs to determine the needs of local hospitals and birthing facilities. To date, 42% of states have not yet taken action to implement recommendations for CCHD screening.12

Future of CCHD screening

Pulse oximetry is a simple, safe, cost-effective tool for improving the early detection of CCHD in newborns. When added to the standard newborn physical examination, current pulse-oximetry protocols are associated with high sensitivity for CCHD, with low false-positive and false-negative rates.15-18

The FDA guidance document on the safety and effectiveness of pulse oximeters  will further clarify the technical standards of care in CCHD screening.6 When the FDA guidance document is finalized, any pulse oximeter considered for screening should meet the revised technical recommendations.6

Future advances will continue to improve the clinical utility of screening by further decreasing false-negative and false-positive rates. Manufacturers are making frequent improvements both in hardware and software in an effort to enhance the performance of pulse oximeters, and future guidelines from the SACHDNC are expected to reflect these new technological standards.

Moreover, findings from state surveillance programs may also reveal important gaps in pulse-oximetry screening for CCHD that can be addressed with revised protocols. Given the importance of feedback for continuous quality improvement, it is critical that caregivers participate and provide detailed information to surveillance programs.

Conclusions

Backed by evidence-based recommendations, endorsements from the HHS and national medical societies, and state-level legislation, pulse-oximetry screening for CCHD is poised to change the standard of care for infants born in the U.S.

Successful screening will require the standardization of protocols across hospitals and other care settings, as well as commitment from state public-health systems to support surveillance. Ongoing quality-assurance initiatives will continue to enhance the performance of pulse-oximetry screening across health-care settings. By participating in the implementation of screening programs in their institutions, health-care providers can contribute to the early detection of CCHD in affected newborns.

Gerard R. Martin, MD (CHAIR), is co-director of the Children’s National Heart Institute at the Children’s National Medical Center  and a Professor of Pediatrics at George Washington University, in Washington, DC.

Alex R. Kemper, MD, MPH, MS, practices in the Department of Pediatrics and Community and Family Medicine at

Duke University School of Medicine, in Durham, NC.

Elizabeth A. Bradshaw, MSN, RN, CPN, is coordinator for the Congenital Heart Disease Screening Program at the Children’s National Medical Center, in Washington, DC.

References

  1. Kemper AR, Mahle WT, Martin GR, et al. Strategies for implementing screening for critical congenital heart disease. Pediatrics. 2011;128:e1259-e1267.
  2. Hoffman JI. It is time for routine neonatal screening by pulse oximetry. Neonatology. 2011;99:1-9.
  3. Mahle WT, Newburger JW, Matherne GP, et al. Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the AHA and AAP. Pediatrics. 2009;124:823-836.
  4. Wren C, Reinhardt Z, Khawaja K. Twenty-year trends in diagnosis of life-threatening neonatal cardiovascular malformations. Arch Dis Child Fetal Neonatal Ed. 2008;93:F33-F35.
  5. O’Donnell CP, Kamlin CO, Davis PG, et al. Clinical assessment of infant colour at delivery. Arch Dis Child Fetal Neonatal Ed. 2007;92:F465-F467.
  6. U.S. Food and Drug Administration. Draft guidance for industry and FDA staff: pulse oximeters—premarket notification submissions [510(k)s].
  7. Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children. Recommendations and responses from the HHS Secretary.
  8. Mahle WT, Martin GR, Beekman RH, et al. Endorsement of Health and Human Services recommendation for pulse oximetry screening for critical congenital heart disease. Pediatrics. 2012;129:190-192.
  9. Maryland State Advisory Council on Hereditary and Congenital Disorders. Recommendations on implementation of screening for critical congenital heart disease in newborns: 2011 Legislative Report.
  10. Congenital Heart Disease Screening Program Toolkit. Children’s National Medical Center, Washington, D.C.
  11. Garg LF. Screening for critical congenital heart disease in newborns using pulse oximetry— New Jersey’s experience. March 22, 2012.
  12. Newborn Coalition. CCHD Screening Map.
  13. Das J, Aggarwal A, Aggarwal NK. Pulse oximeter accuracy and precision at five different sensor locations in infants and children with cyanotic heart disease. Indian J Anaesth. 2010;54:531-534.
  14. Phattraprayoon N, Sardesai S, Durand M, et al. Accuracy of pulse oximeter readings from sensor placement on newborn wrist and ankle. J Perinatol. 2012;32:276-280.
  15. Sedaghat-Yazdi F, Torres A Jr, Fortuna R, et al. Pulse oximeter accuracy and precision affected by sensor location in cyanotic children. Pediatr Crit Care Med. 2008;9:393-397.
  16. Thangaratinam S, Brown K, Zamora J, et al. Pulse oximetry screening for critical congenital heart defects in asymptomatic newborn babies: a systematic review and meta-analysis. Lancet. 2012;379:2459-2464.
  17. de-Wahl Granelli A, Wennergren M, Sandberg K, et al. Impact of pulse oximetry screening on the detection of duct dependent congenital heart disease: a Swedish prospective screening study in 39,821 newborns. BMJ. 2009;338:a3037.
  18. Ewer AK, Middleton LJ, Furmston AT, et al. Pulse oximetry screening for congenital heart defects in newborn infants (PulseOx): a test accuracy study. Lancet. 2011;378:785-794.
  19. Meberg A, Brügmann-Pieper S, Due R Jr, et al. First day of life pulse oximetry screening to detect congenital heart defects. J Pediatr. 2008;152:761-765.

All electronic documents accessed October 15, 2012.