The AAP has updated newborn critical congenital heart disease (CCHD) screening recommendations to address key developments since screening using pulse oximetry was added to the U.S. Recommended Uniform Screening Panel (RUSP) in 2011.
The updates, outlined in a new clinical report, include endorsement of a simplified screening algorithm; a call for states to adopt a common uniform dataset to aid in surveillance; and recommendations to improve education for health care providers and others on limitations of screening, identification of noncardiac conditions and protocol adherence.
The clinical report Newborn Screening for Critical Congenital Heart Disease: A New Algorithm and Other Updated Recommendations is from the Section on Cardiology and Cardiac Surgery, Section on Hospital Medicine and Committee on Fetus and Newborn. It is available at https://doi.org/10.1542/peds.2024-069667 and will be published in the January issue of Pediatrics.
Incidence of CCHD, benefits of screening
Congenital heart disease (CHD) is the most common birth defect and causes the highest number of deaths in infants younger than 1 year due to congenital malformations. Roughly eight in 1,000 infants have CHD, while critical forms of CHD, or life-threatening CCHD, impact roughly two to four of every 1,000 births.
Many infants with CCHD will not present with murmur or visible cyanosis. The use of pulse oximetry screening for asymptomatic newborns helps identify at-risk infants, allowing them to receive lifesaving interventions prior to hospital discharge.
Screening is performed on well-appearing newborns at or around 24 hours of life or just prior to hospital discharge. A pulse oximeter is attached to the newborn’s right hand and either foot to measure blood oxygen level. Infants who do not pass the screen undergo further evaluation to determine if they have a serious heart defect.
Screening works best in conjunction with prenatal ultrasound and newborn physical examination. When performed together, roughly 95% of infants with CCHD are detected prior to hospital discharge.
When CCHD screening was added to the RUSP in 2011, questions remained regarding its effect on morbidity and mortality, cost and resource utilization. Since then, one study showed a 33% decrease in infant mortality after states implemented screening policies as well as decreases in emergency hospitalization due to CCHD (Abouk R, et al. JAMA. 2017;318:2111-2118).
Concerns regarding cost and resource overutilization also have been alleviated, as few additional echocardiograms are needed when noncardiac causes of hypoxia are investigated first.
Algorithm simplification
The AAP is endorsing use of a simplified CCHD screening algorithm published in 2020.
The algorithm revisions include:
- eliminating the second rescreen if a newborn falls into the retest category (this allows for earlier evaluation and treatment) and
- changing the oxygen saturation requirement for passing the screen from 95% or greater in the hand or foot to 95% or greater in the hand and
While the number of false positives may increase slightly, screening may detect noncardiac conditions such as sepsis and pneumonia that benefit from early identification and treatment.
States that are required to follow AAP guidelines should implement the updated CCHD screening algorithm.
Key actions for pediatricians
- Be aware that for each case of CCHD identified, there typically are four to five cases of infections or respiratory causes of low oxygen saturations. For infants who fail screening, rule out infections and respiratory causes; for those who pass, remain aware that some forms of CCHD may not present with hypoxia in the neonatal period.
- Do not rely on screening with pulse oximetry alone to determine whether an infant has CCHD.
- CCHD screening was designed to apply to asymptomatic infants in well-baby nurseries. As such, infants should not be screened while on supplemental oxygen.
Improved data collection, future considerations
The clinical report highlights opportunities to support public health programs’ efforts to improve data collection, data sharing and improved access to care. Implementing electronic data exchanges and strengthening collaborations between birth hospitals and public health programs are essential to understanding and improving CCHD outcomes.
The report also identifies areas to improve CCHD screening, including innovations to improve sensitivity and studies to ensure the accuracy of pulse oximetry in detecting hypoxemia in infants with different skin pigmentations.
Ms. Hom, Dr. Oster and Dr. Martin are lead authors of the clinical report.
Resources
• Information on pulse oximetry screening from HealthyChildren.org
