Endorsement of Health and Human Services Recommendation for Pulse Oximetry Screening for Critical Congenital Heart Disease

• pulse oximetry
• critical congenital heart disease
• recommended uniform screening panel
• screening
• newborn infants

Delayed diagnosis of critical congenital heart disease (CCHD) can result in death or injury to infants. The current approach to detect CCHD in the United States relies on prenatal ultrasound examinations and the physical examination findings in the newborn nursery. Unfortunately, this approach fails to identify a significant number of cases of CCHD, which may lead to late diagnosis with significant morbidity, permanent injury of vital organs, and in some cases, death. A number of studies have revealed that adding pulse oximetry to the assessment of the newborn can enhance detection of CCHD. Pulse oximetry is a readily available, noninvasive, and painless technology that can be incorporated into the routine assessment of the newborn.

On September 21, 2011, the Secretary of Health and Human Services (HHS), Kathleen Sebelius, recommended that screening for CCHD be added to the recommended uniform screening panel (RUSP). This recommendation was based in large part on the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children’s recommendations and a 2-day comprehensive evidence review of screening strategies by national and international stakeholders. The American Academy of Pediatrics (AAP) strongly supports the decision of the Secretary of HHS to add screening with pulse oximetry to the RUSP. The AAP has been a vigorous advocate of early detection of CCHD to prevent childhood deaths or injury that might occur as a result of late detection.

There are a number of important issues that relate to the implementation of pulse oximetry into the routine care of the newborn. A detailed description of these issues has been published by the AAP.¹ This publication provides a detailed screening algorithm developed by the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children. It also provides detailed recommendations regarding necessary equipment, personnel and training, and appropriate management of a positive screening result. The highlights of screening implementation are as follows:

• The screening is targeted toward healthy newborn infants in the newborn nursery.

• Screening should be performed with motion-tolerant pulse oximeters. It is appropriate to use either disposable or reusable pulse oximetry probes.

• Screening should not be undertaken until 24 hours of life or as late as possible if early discharge is planned to reduce the number of false-positive results. Separate consideration for home births is necessary.

• Oxygen saturations should be obtained in the right hand and one foot. Threshold for a positive screening result is detailed in the publication and relates to both the absolute reading by the pulse oximeter as well as the difference between the 2 extremities. Screening that has a pulse oximetry reading of ≥95% in either extremity with a ≤3% absolute difference between the upper and lower extremity would be considered a pass, and the screening would end. It is recommended that repeated measurements be performed in those cases in which the initial screening result was positive, again in an effort to reduce false-positive results. Infants with saturations <90% should receive immediate evaluation. It is important to note that the oxygen saturation thresholds for a positive screening result may vary at high altitude. Appropriate studies need to be performed at high altitude to establish reliable thresholds.

• In the event of a positive screening result, CCHD needs to be excluded with a diagnostic echocardiogram. Infectious and pulmonary causes of hypoxemia should also be excluded.

The AAP can play an important role in assuring the safe and effective implementation of screening for CCHD. This includes preparing members to implement screening; engaging pediatricians to participate in quality-improvement activities to ensure that newborn infants are appropriately screened with follow-up echocardiograms and specialty care, as required; partnering with public health agencies for surveillance of CCHD; and advocating for appropriate payment for all screening-related activities. In addition to promoting the implementation of pulse oximetry based screening programs, the AAP supports widespread surveillance for CCHD. Such surveillance will yield several benefits, including documentation of any improvement in detection as well as identification of areas of weakness for this comprehensive screening strategy.

Although the Secretary of HHS has recommended that screening for CCHD be added to the RUSP, it will be up to states to determine how they wish to incorporate this into their own screening programs. This may be accomplished by legislation, regulation, or adoption as a standard of practice. The majority of disorders identified on the RUSP are adopted by most states, but there is variability among the states. A number of states have already incorporated routine screening for CCHD into the statewide panel. These states can serve as a paradigm for subsequent implementation by other states.* It will be critical that AAP chapters work with state health departments to implement the new screening strategy for CCHD in a manner that is adequately financed, cost-effective, and practical; that ensures screening and for echocardiographic/pediatric cardiac evaluation and therapeutic intervention as indicated with provision for follow-up for all newborn infants within the framework of the medical home; and that ensures that related payment issues for technical and professional services are addressed. It is hoped that through these collaborative efforts, the burden of delayed diagnosis of congenital heart disease will be reduced and the health of children will be enhanced for years to come.

• Copyright © 2012 by the American Academy of Pediatrics