State Legislation, Regulations, and Hospital Guidelines for Newborn Screening for Critical Congenital Heart Defects - United States, 2011-2014.

Critical congenital heart defects (CCHD) occur in approximately two of every 1,000 live births (1). Newborn screening provides an opportunity for reducing infant morbidity and mortality (2,3). In September 2011, the U.S. Department of Health and Human Services (HHS) Secretary endorsed the recommendation that critical congenital heart defects be added to the Recommended Uniform Screening Panel (RUSP) for all newborns (4). In 2014, CDC collaborated with the American Academy of Pediatrics (AAP) Division of State Government Affairs and the Newborn Screening Technical Assistance and Evaluation Program (NewSTEPs) to assess states’ actions for adopting newborn screening for CCHD. Forty-three states have taken action toward newborn screening for CCHD through legislation, regulations, or hospital guidelines. Among those 43, 32 (74%) are collecting or planning to collect CCHD screening data; however, the type of data collected by CCHD newborn screening programs varies by state. State mandates for newborn screening for CCHD will likely increase the number of newborns screened, allowing for the possibility of early identification and prevention of morbidity and mortality. Data collection at the state level is important for surveillance, monitoring of outcomes, and evaluation of state CCHD newborn screening programs. Congenital heart defects occur in approximately eight of every 1,000 live births, one fourth of which are considered to be CCHD (1). CCHD are defined as those requiring surgery or catheterization before age 1 year. In the absence of early detection, infants with CCHD are at risk for serious complications or death within the first few days or weeks of life (1). Newborn screening for CCHD uses pulse oximetry, a noninvasive technology to measure blood oxygen saturation. Low oxygen saturation indicates hypoxemia, an early clinical sign of CCHD. Additional testing (e.g., repeat screening, echocardiogram) is needed following an abnormal pulse oximetry screen (1) to determine whether CCHD are present (or to determine the cause of the abnormal result). Thus, unlike most newborn screening conditions, screening for CCHD is not based on performing a blood test. In addition, hypoxemia detected by screening could indicate a medical problem, and requires immediate follow-up before discharge from the hospital. When accompanied by early identification and treatment, newborn screening provides an opportunity to reduce infant morbidity and mortality (2,3). The Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children has provided national guidelines and recommendations on State Legislation, Regulations, and Hospital Guidelines for Newborn Screening for Critical Congenital Heart Defects — United States, 2011–2014