Kristine Palmer with baby receiving pulse oximetry screening.

CCHD occurs when there is a defect in the heart structure that causes the blood to flow through the heart and lungs abnormally, which can lead to decreased oxygen levels in the blood. CCHD affects about eight out of every 1,000 newborns and accounts for up to 10 percent of infant deaths. While some defects of heart are minor, CCHD poses an increased risk of causing permanent tissue and organ damage due to decreased oxygen levels in the body or death in an infant if it is not diagnosed soon after delivery.

Some congenital heart disease can be detected prenatally or through physical examination after delivery, but it is estimated that about 30 percent of newborns with CCHD may leave the hospital after delivery before being diagnosed. Many of the newborns with CCHD are detected late or even after death has occurred. Universal screening of the newborn can help improve the prognosis of these children by increasing the chance of early detection.

W. Robert Morrow, MD, professor of pediatrics and associate dean, children’s affairs, at the UAMS School of Medicine, was one of the authors of an article, “Strategies for Implementing Screening for Critical Congenital Heart Disease,” published in the Journal of the American Academy of Pediatrics in November 2011. Morrow was part of a workgroup tasked with coming up with strategies for the implementation of safe, effective, and efficient CCHD screening.

“The work-group members found sufficient evidence to begin screening for low blood oxygen saturation through the use of pulse oximetry monitoring to detect CCHD in well-infant and intermediate care nurseries,” the article in Pediatrics stated. “Research is needed regarding screening in special populations (e.g., at high altitude) and to evaluate service infrastructure and delivery strategies (e.g., telemedicine) for nurseries without on-site echocardiography. Public health agencies will have an important role in quality assurance and surveillance. Central to the effectiveness of screening will be the development of a national technical assistance center to coordinate implementation and evaluation of newborn screening for CCHD.”

In September 2011 the Secretary of the U.S. Department of Health and Human Services endorsed adding pulse oximetry screening for CCHD as one of the recommended screens for all newborns after delivery. The American Heart Association, American Academy of Pediatrics and American College of Cardiology also support newborn pulse oximetry screening for CCHD.

Morrow said they expect to identify 35-40 babies per year in Arkansas through the screening program to be performed between 24 and 48 hours of life. The screening was implemented at UAMS Medical Center Jan.2.

Morrow was also one of the authors of a report published in Pediatrics in January 2012 titled, “Endorsement of Health and Human Services Recommendation for Pulse Oximetry Screening for Critical Congenital Heart Disease.”

The seven types of congenital heart disease which the screenings are designed to identify are those which cause the baby to have lower than normal oxygen levels and which have significant morbidity or mortality early in life, particularly if not identified early, said Kristine Palmer, MD, associate professor of pediatrics,

UAMS.

“These kind of congenital heart disease can be difficult to detect just on physical exams,” Palmer said. “When detected early, the babies do better long term. If it is not detected before the baby leaves the hospital, the baby can come back into the hospital very ill from the heart defect. The idea is to pick up heart disease before the blood vessel closes. When that happens, babies will sometimes get very sick.”

The newborns being screened will be normal babies from the labor or delivery ward or newborn nursery who would usually go home at the age of one or two days.

Pulse oximetry screening is an inexpensive, non-invasive test that uses a lighted probe placed on the finger to get the oxygen saturation level that is then compared to the same measurement in the foot.

“If the levels measured are lower than expected, then that is a positive screening which would prompt us to do further testing to look for heart disease,” Palmer said.

Mary Ann Salazar, MNSc, WHNP-BC, RNC-MNN, an advanced practice partner with the UAMS Medical Center Perinatal Unit, said the test that only takes five minutes and costs only $5 will help save lives and prevent illness. She said by using the pulse oximetry screening guideline and protocol developed, they will be able to use these readings to aid the medical team in identifying CCHD before the newborn is discharged.

“The cost and time involved in performing the pulse oximetry screen on a newborn is minimal,” Salazar said. “But even if only one newborn’s CCHD is detected early, the rewards are priceless.”

The Arkansas Legislature has not yet mandated this screen for all newborns, but there is a group of medical advocates and experts working on making this a reality for all newborns delivered in Arkansas.

“As a leader in healthcare and research in Arkansas, we here at UAMS Medical Center have an obligation and a commitment to our state to serve as a role model for other medical institutions and advocate for universal CCHD pulse oximetry screening,” Salazar said.

As a nurse Salazar has seen firsthand the tragedy and loss that can result when a disease process is not detected and treated early. “And as a mother of four children myself, I know that my child’s health and life is priceless not just to me, but also to the health of our state,” she said.

Infants identified to be at risk for CCHD are immediately referred to the medical team in the UAMS Neonatal Intensive Care Unit for additional monitoring and follow-up. An echocardiogram of the infant’s heart is then performed to identify structural defects in the heart that may need repair. If CCHD is diagnosed, these newborns are transferred to Arkansas Children’s Hospital and into the expert care of pediatric cardiologists.

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