Screening for critical congenital heart defects (CCHD) is now standard across the country, but the Centers for Disease Control and Prevention is calling for continued improvement on how data is collected and shared from the screenings.
Jill Glidewell, MSN, MPH
Critical congenital heart defect (CCHD) screening has been implemented in all 50 states and the District of Columbia,1 according to a new report from the Centers for Disease Control and Prevention (CDC), but there is still room for improvement.
Jill Glidewell, MSN, MPH, health scientist for the CDC’s National Center on Birth Defects and Developmental Disabilities and lead author of the report, says that, despite the call for more improvement, it’s important to recognize that all newborns in the United States are now screened for CCHD.
“Some babies born with a critical congenital heart defect appear healthy at first, and they may be sent home before their heart defect is detected. These babies are at risk of having serious complications within the first few days or weeks of life, and often require emergency care,” Glidewell says. “Newborn screening is a tool that can identify some of these babies so they can receive prompt care and treatment. Timely care may prevent disability or death early in life.”
Why screening is so important
Critical congenital heart disease occurs in 2 of every 1000 live births and can go undetected at birth. Infants with CCHD are at risk for substantial morbidity and death without early detection and, as a result, the US Department of Health and Human Services in 2011 endorsed a recommendation from the Advisory Committee on Heritable Disorders in Newborns and Children to add CCHD to the universal newborn screening panel.
According to the CDC, deaths from CCHD and other cardiac issues decreased from 2007 to 2013 in 8 states that had implemented CCHD screening at birth. The CDC, along with the American Academy of Pediatrics (AAP) and the Association of Public Health Laboratories’ Newborn Screening Technical assistance and Evaluation Program (NewSTEPs) released an updated report in 2015 on strategies for adopting and implementing policies to support CCHD screening, the CDC notes.
Although the CDC reports that as of 2018 every state had added CCHD to newborn screening-with the exception of California, which offers but does not mandate the screening-the agency notes that more work is needed to use the screening to its full potential. Whereas the number of states (including the District of Columbia) offering the screening jumped from 22 in 2013 to 51 by 2018, not all states have put in place data tracking systems to assess screening results and incomes, the CDC reports. Further development of such systems could help identify areas for future improvement and monitor the efficacy of early CCHD identification, the agency notes.
Simple test yet effective
According to the CDC, the infant test for CCHD is simple and noninvasive pulse oximetry. Hypoxemia, or low levels of oxygen saturation, detected by pulse oximetry screening can signal CCHD or other abnormalities.
“Newborn screening for critical congenital heart defects involves a simple bedside test called pulse oximetry. This test estimates the amount of oxygen in a baby’s blood,” Glidewell says. “Low levels of oxygen in the blood can be a sign of a critical congenital heart defect.” However, pulse oximetry doesn’t have to be the end for screenings, she notes.
“Pulse oximetry screening does not replace a complete history and physical examination, which sometimes can detect a critical congenital heart defect before oxygen levels in the blood become low,” Glidewell says. “Pulse oximetry screening, therefore, should be used along with the physical examination.”
Glidewell says screening is done when a baby is at least 24 hours of age, or as late as possible if the baby is to be discharged from the hospital before he or she is aged 24 hours.
More data collection is needed
As far as how much further states need to go in terms of collecting and reporting screening data, the CDC reports that 41 jurisdictions received screening data from hospitals or birthing centers. Of those, 32 received individual-level screening results, while 12 reported only whether infants passed or failed the screening. Five simply reported the number of infants screened and the number of CCHD cases identified, and 4 reported only individual screening results for infants in whom CCHD was detected. Taking it a step further, the CDC reports that 19 jurisdictions shared data on birth defects surveillance and newborn screening, 5 of which had linked electronic systems in place for both newborn screenings and birth defect surveillance, 2 had shared data systems for both CCHD screening and birth defects, and 12 had data sharing through manual communications such as e-mails and reports.
Complete data collection is an important part of surveillance, the CDC says, and it is needed to monitor outcomes, improve processes, and evaluate CCHD screening programs. The CDC report endorses the need for more improvement in fostering collaboration between public health programs to improve CCHD and birth defect surveillance and reporting.
Glidewell says it was interesting to note that many state screening programs get screening information from hospitals or birthing centers, but not all states have systems in place for tracking screening results or sharing information with birth defects tracking programs.
“It is important that screening programs and birth defects tracking programs share information to allow for evaluation of screening,” Glidewell says. “Despite some limitations in data collection and sharing, all US newborns, regardless of the state they are born in, now have the opportunity to be screened for critical congenital heart defects.”
1. Glidewell J, Grosse SD, Riehle-Colarusso T, et al. Actions in support of newborn screening for critical congenital heart disease-United States, 2011-2018. MMWR Morb Mortal Wkly Rep. 2019;68(5):107-111.