Implications of Using Pulse Oximetry to Screen for Critical Congenital Heart Disease in Newborns

Implications of Using Pulse Oximetry to Screen for Critical Congenital Heart Disease in Newborns JJOAN OAN ANDREA

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HAT STARTED AS a routine home visit by a nurse/lactation consultant turned into an emergency transfer of a newborn to the hospital for critical congenital heart disease (CCHD). The mother in this case was concerned that her full-term newborn wasn’t breastfeeding as well as her first baby did, and this alerted the nurse to possible complications. Without the visiting nurse’s knowledge and recognition of what’s to be expected in a newborn compared to a 5-day-old newborn, the prognosis could have been deadly. Two days earlier, the seemingly healthy newborn had been discharged. The astute nurse’s observation of central cyanosis and heart murmur, together with feeding difficulties, alerted her to the missed diagnosis of CCHD. At birth, these signs and symptoms are often observed as the newborn transitions from intrauterine to extrauterine life. But soon after birth, these signs and symptoms are resolved. Recognition of congenital heart defects after birth can be challenging. The Centers for Disease Control and Prevention (CDC) reports that congenital heart defects occurs in 1 of 100 births in the United States (CDC, 2014a). Twenty-five percent of these newborns have a CCHD, a subgroup of congenital heart defects, including 12 different conditions. Pulse oximetry screening can potentially identify seven of those conditions as identified in Box 1 (CDC, 2014b). Abstract: In recent years, pulse oximetry screening for critical congenital heart disease (CCHD) in newborns has been added to the list of recommended uniform screening panels and recommended by several health care organizations. Most states use pulse oximetry to screen for CCHD. Studies have identified problems with compliance and higher failure rate at moderate altitudes than at sea level, suggesting the need for alternate algorithms. Altitude, time, health status of newborns and type of cardiac defect appear to affect results. Early detection of CCHD improves health outcomes and reduces morbidity and mortality. Barriers to screening include out-of-hospital births, cost and knowledge deficits among health care professionals. DOI: 10.1111/1751-486X.12217 Keywords: congenital heart defect | critical congenital heart disease | newborn screening | pulse oximetry

In addition to physical assessment, pulse oximetry, a simple, noninvasive inexpensive screening approach, can be performed before discharge with a sensitivity of 76.5 percent and a specificity of 99.9 percent to help identify newborns with CCHD (Thangaratinam, Brown, Zamora, Khan, & Ewer, 2012). Yet, noncompliance and errors in interpretation of the algorithm and new recommended guidelines are occurring. The importance of nurses’ roles in understanding the protocol of this simple, noninvasive, inexpensive screening test is imperative. As more hospitals incorporate this screening into their routine screening, new research findings have resulted in questions and created new focus on how this screening can influence health outcomes. This article discusses emerging problems and barriers to screening, along with potential remedies.

About CCHD Signs and symptoms of CCHD can be absent until the ductus arteriosus, a fetal shunt, begins to close. Prior to pulse oximetry screening, as many as 22 percent to 43 percent of newborns with CCHD were diagnosed 3 days or more after discharge (CDC, 2014a). Late diagnosis of CCHD resulted in 8 percent of these newborns eventually dying in the first year of life (Fixler, Xu, Nembhard, Ethen, & Canfield, 2014). Even with special ultrasounds, called fetal echocardiograms, which aren’t available universally, diagnosis of CCHD during pregnancy is difficult (Ailes et al., 2014). In the early days after birth, symptoms of CCHD can be subtle or not apparent with physical assessment. One in three cases of CCHD in a newborn aren’t detected (Andrews, Ross, Salazar, Tracy, & Burke, 2014; CDC, 2014b, 2014c; Martin, Kemper, & Bradshaw, 2012a, 2012b). Not until families have taken these babies home will serious life-threatening complications begin. Until recent years, no routine screening protocol existed. The goal of screening with pulse oximetry is to identify those asymptomatic newborns with invisible hypoxia resulting from structural complications in the heart and great vessels. Screening with pulse oximetry for CCHD has become the standard of care in the United States with strong support from the American Academy of Pediatrics (AAP), CDC, March of Dimes, American Heart Association (AHA) and American College of Cardiology Foundation (Mahle et al., 2012).

Detecting Low Oxygen Levels in Newborns In the first few hours after birth, a healthy term newborn’s oxygen level gradually rises as transition from intrauterine to extrauterine circulation occurs. Expedient recognition of deviation from

these transitions is crucial in identifying complications. Classification of congenital heart defects is based on hemodynamic characteristics, such as whether the anomaly causes decreased or increased pulmonary blood flow, obstruction of the flow of blood out of the heart or a mixture of saturated and desaturated blood flowing through the heart and great vessels. Pulse oximetry is useful because it can detect low oxygen levels before visible cyanosis occurs (Rohan & Golombek, 2009). Abnormalities in adaptation may be identified within minutes of birth. Hypoxia, deprivation of oxygen to the cells, is typically manifested as bluish purple or gray discoloration of skin, mucous membranes and tongue. Unlike in an adult who has very little fetal hemoglobin, 60 percent to 80 percent of a newborn’s total hemoglobin is fetal hemoglobin. Fetal hemoglobin has higher oxygen affinity and plays an important role in transporting oxygen from the mother to the fetus. Consequently, a neonate can tolerate lower PaO2 levels without discernable cyanosis. It’s not until PaO2 levels drop to approximately 75 percent to 80 percent before cyanosis is visible (see Box 2; Rohan & Golombek, 2009). Tolerance of lower oxygen levels serves the fetus well while in an environment that competes for oxygen from the mother through the placenta, but makes it difficult to visually detect cyanosis after birth. Once a newborn manifests cyanosis, oxygen insufficiency is significant. Laboratory testing can quantify the oxygen saturation and reveal deficiencies before physical assessment findings are apparent (Martin et al., 2012a).

Screening Procedure The recommendation for screening for CCHDs with pulse oximetry focuses on infants in well- and intermediate-care nurseries (Kemper et al., 2011). Screening is to be done on newborns after 24 hours of age or shortly before discharge if less than 24 hours old. Implementing the screening when the newborn’s

BOX 1

SEVEN CRITICAL CONGENITAL HEART DEFECTS THAT CAN BE SCREENED FOR WITH PULSE OXIMETRY

Opening photo © iStcok Collection / thinkstockphotos.com

Importance of Screening

Hypoplastic left heart syndrome Pulmonary atresia (with intact septum) Tetralogy of Fallot Total anomalous pulmonary venous return Transposition of the great arteries Tricuspid atresia

Joan Andrea, DNP, FNP, IBCLC, is an associate professor at North Park University in Chicago, IL. The author reports no conflicts of interest or relevant financial relationships. Address correspondence to: jandrea@ northpark.edu.

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Truncus arteriosus

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newborns, be motion-tolerant and have 2 percent rootmean accuracy, a calculation of variation. Pulse oximetry indirectly measures PO2 levels based on a formula of light absorption through oxyhemoglobin and deoxyhemoglobin in the arteries. A 1 percent to 2 percent

Photo © Elena Petrova / thinkstockphotos.com

EVEN WITH SPECIAL ULTRASOUNDS, CALLED FETAL ECHOCARDIOGRAMS, WHICH AREN’T AVAILABLE UNIVERSALLY, DIAGNOSIS OF CCHD DURING PREGNANCY IS DIFFICULT

ductus arteriosus is closed, usually by 24 hours of age, is more likely to detect the ductal dependent lesions, allowing for treatment with prostaglandin to keep the ductus arteriosus open until surgical intervention can occur. Screening after 24 hours reduces the number of false-positive results (CDC, 2014b). The falsepositive rate dropped from 0.14 percent to 0.05percent when pulse oximetry was performed after 24 hours from birth without any compromise in overall sensitivity (Thangaratinam et al., 2012). Interestingly, there may be value in false-positives readings. False-positive readings may indicate other life-threatening disorders of noncardiac origin that produce pulse oximetry changes, such as group B streptococcal pneumonia and pulmonary hypertension (Thangaratinam et al., 2012). It’s possible for a newborn with a CCHD or other congenital heart defect to have an oxygen level within the expected range and consequently result in a false-negative reading. The presence of multiple congenital malformations, low birth weight, prematurity, intrauterine growth restriction and certain types of congenital heart defects seem to be linked to missed CCHD diagnosis (Dawson et al., 2013). For accurate readings, the pulse oximetry must be approved by the U.S. Food and Drug Administration (FDA) for use in

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variation, from the “true” value is expected with pulse oximetry machines. Motion and noise can produce erroneous readings. Probes need to be close fitting to the skin and the type recommended by the manufacturer of the pulse oximetry to ensure accurate readings. Reusable or one-time-use disposable probes can be used. Reusable probes must be properly cleaned between utilization to prevent infection. Probe sensors are placed on the right hand and sole of either foot. Adult pulse oximetry clips are not to be used because they can give inaccurate readings (Kemper et al., 2011). Some manufacturers are designing pulse oximetry with the CCHD algorithm embedded, but this isn’t necessary. Several practice suggestions can promote optimal accuracy of pulse oximetry readings (see Box 3). Anecdotal reports suggest that readings are more accurate if done when the newborn is alert. Newborns can be breastfed during the procedure. The newborn may need to be warmed to promote adequate perfusion in the extremity to produce accurate pulse oximetry reading (Hines, 2012). Prior to administration, parents should be informed of the purpose of screening for CCHD, along with other newborn screening procedures. The CDC and state health departments often have literature to answer parents’ questions regarding pulse oximetry screening. Videos in English, Chinese, French, Arabic, Russian and Spanish demonstrating the CCHD screening procedure to parents are available at a website called “Baby’s First Test” (www.babysfirsttest.org/newborn-screening/conditions/criticalcongenital-heart-disease-cchd). Parents need to be informed of abnormal results and the follow-up process that will occur.

Interpreting Results Pulse oximetry measures the oxygen in the right hand (preductal) and either foot (postductal). Preductal relates to that

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BOX 2

VISIBLE CYANOSIS AND OXYGENATION LEVEL Oxygenation Level

Skin color

Approximately<80 percent

80 percent to 94 percent

>95 percent

Visible cyanosis

Invisible hypoxia

Well oxygenated

part of the aorta proximal to the aortic opening of the ductus arteriosus. The oxygen level in either foot is called postductal and refers to that part of the aorta distal to the aortic opening of the ductus arteriosus. A “pass” score is obtained when either the right hand (preductal) or foot (postductal) has a reading of greater than or equal to 95percent PaO2 level with the difference between the hand and foot reading less than or equal to 3 percent. A “pass” or “negative” score on the pulse oximetry screening means that the newborn didn’t have low levels of blood oxygen and is then unlikely to have a CCHD. The newborn has three chances to pass the screening, unless one of the readings in the right hand (preductal) or foot (postductal) is <90 percent. A pulse oximetry result of <90 percent is positive, a failed screening and requires immediate follow-up. Readings between 90 percent and 95 percent or >3 percent difference requires rescreen in 1 hour and again in another hour if the readings aren’t adequate. Failing all three screenings is considered “fail” or “positive” and indicates the need for additional testing to account for the low levels of blood oxygen (CDC, 2014a; Ramjattan & Allen, 2013). An echocardiogram is recommended to diagnose a potential CCHD (CDC, 2014b; see CDC, 2014d for the CDC’s algorithm for screening). An app is available to assist in interpretation of the algorithm (pulseoxtool.com). A nurse-driven, color-coded algorithm has been suggested as an aid to guide nurses implementing the protocol. In such an algorithm, pulse oximetry levels ≥96 percent are green and means the newborn can be discharged home. Pulse oximetry levels of ≤90 percent are in red and require immediate notification of provider. Ninety-one percent to 95 percent are coded yellow and require confirmation and repetition after checking newborn’s temperature, repositioning or replacing the probe. An excessively cold newborn has decreased perfusion and yields inaccurate readings (Hines, 2012).

Barriers to Screening Recommendations The AAP recommends pulse oximetry screening for CCHD of all newborns. Many states have adopted and implemented

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the recommendation into state toolkits. Currently, there are few screening protocols for babies born outside of the hospital (Kemper et al., 2011; Lhost et al., 2014). Research leading to the current screening algorithm didn’t include out-of-hospital births. Many states mandate testing for all neonates born in a hospital who are in the well-newborn and intermediate-care nurseries. Other states include all neonates born in birthing facilities. Problems arise for neonates born at home where there

BOX 3

PRACTICE SUGGESTIONS FOR PROMOTING OPTIMAL ACCURACY OF PULSE OXIMETRY READINGS Place the light emitter portion of the probe directly opposite the light emitter and secure with wrap provided by manufacturer of the pulse oximetry. Blood flow is needed to get accurate readings and produce a waveform. Avoid placing probes on an extremity with automatic blood pressure cuff that would impede blood flow producing a false reading. Bilirubin lamps, surgical lights and other bright or infrared lights can also affect accuracy of the reading. In a bright environment, covering the probe with an opaque covering will minimize inaccurate readings. Dried blood on the extremities where the probe is placed can yield inaccurate readings. Skin should be clean and dry prior to probe being placed. Skin color and jaundice will not affect the reading. Source: Martin et al. (2012b).

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THE AAP RECOMMENDS PULSE OXIMETRY SCREENING FOR CCHD OF ALL NEWBORNS asymptomatic newborns. Evidence is emerging that pulse oximetry screening protocol is applicable to a newborn discharged from NICU (Iyengar, Kumar, & Kumar, 2014). It’s assumed that CCHD would be recognized in newborns in the NICU with their longer hospital stays and frequent tests, monitoring and examinations. Some, but not all, may even have had echocardiograms prior to or after birth looking for structural heart malformations. Others may undergo continuous pulse oximetry monitoring during their hospitalization. CCHD can be missed in the NICU, especially if the pulse oximeter probe isn’t changed from limb to limb occasionally. Approximately 5 percent of all newborns in the NICU go home with either no pulse oximetry or with SpO2 <95 percent (Iyengar et al., 2014). The only sign of CCHD in some newborns is a 4 percent difference in the pre-post ductal oxygen saturation. Therefore, in an effort to detect infants with CCHD, one author (Suresh, 2013) suggests three options for pulse oximetry screening for CCHD in NICU infants. The first is to institute a policy of obtaining oxygen saturation in an infant’s leg

Photo © iStock Collection / thinkstockphotos.com

is no access to pulse oximetry screening. The home birth attendant, midwife or physician without the equipment may refer the newborn to the hospital for the screening, but hospitals often refuse a “nonpatient” access to this service. Health policies need to ensure that all newborns receive the standard recommended pulse oximetry screening, regardless of where they are born. In July 2014, the Wisconsin Department of Health Services mandated pulse oximetry screening of babies born out of hospital in their recommendation (Wisconsin State Laboratory of Hygiene, 2014). Wisconsin Amish and Mennonite women often choose to give birth outside of the hospital. A study of this community found more missed CCHD diagnoses. Screening for this population, with a known higher heritable rate of conditions, such as Ellis von Creveld, which is associated with CCHD, is vital (Lhost et al., 2014). The Wisconsin Screening Hearts in Newborns (SHINE) project (www.wisconsinshine. org), through the University of Wisconsin School of Medicine and Public Health, the Medical College of Wisconsin and the State of Wisconsin, set out recommendations to educate health care providers, improve access to screening and diagnostic technology and create a statewide CCHD screening and data collection system. Other states could benefit from similar recommendations and legislation. No evidence-based protocol for pulse oximetry screening for CCHD exists for newborns discharged from neonatal intensive care units (NICU). The current recommendation of pulse oximetry screening for CCHD in newborns >35 weeks gestation in well-newborn nurseries is based on studies of

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at some point close to discharge when the baby is more stable. The second option is to perform selective screening based on gestational age (≥35 weeks) or birth weight, excluding those babies who have already had an echocardiogram. A third option is to screen all newborns admitted to the NICU, thereby creating a uniform system that also includes preterm infants less than 35 weeks (Suresh, 2013). Universal screening of infants in NICUs does yield more false-positive results, but may still be a feasible option (Manja, Mathew, Carrion, & Lakshminrusimha, 2015).

services required for high-risk infants that’s available at comprehensive specialized level 3 nurseries. Babies cared for in lower level nurseries were significantly more likely to receive a late diagnosis or missed compared to higher level nurseries (Dawson et al., 2013; Liberman et al., 2014; Oster et al., 2013). More than a quarter of newborns with CCHD are diagnosed after 4 days, some had no record of referral and others were made on autopsy (Fixler et al., 2014; Peterson, Grosse et al., 2014).

Altitude

Training staff promotes accurate implementation and interpretation of the pulse oximetry algorithm. Breaches in process have led to hospital discharge of newborns, who failed the screening and did not receive proper follow-up evaluation ending in devastation (Kochilas et al., 2013). False-positive screenings lead to unnecessary retesting and echocardiography. Nurses must also be aware that a “passing” level on the screening doesn’t guaran-

Higher CCHD pulse oximetry failure rates occur at hospitals at moderate altitudes rather than at sea level, necessitating alternate algorithms in these locations (Samuel et al., 2013; Wright, Kohn, Niermeyer, & Rausch, 2014). Lower saturations and wider ranges tend to occur more frequently in these areas. One explanation is that the transition from fetal to neonatal circulation is delayed due to lower partial pressure of oxygen, limiting pulmonary vasodilation. Pulmonary to aortic shunting through the ductus arteriosus results in postductal desaturation. More research is needed to develop alternative screening protocols to reduce the number of false-positive readings at higher altitudes (Wright et al., 2014).

Late Diagnosis of CCHD Late, also called delayed, diagnosis refers to those newborns who didn’t receive a CCHD diagnosis until after discharge from the hospital. Late diagnosis results in more disabilities, complications and mortality. Delayed diagnosis of CCHD is associated with ductal dependent lesions, requiring the ductus arteriosus to remain open to prevent pulmonary or systemic circulatory collapse. Coarctation of the aorta, a ductal dependent defect, can present later in life and is frequently missed in the first 3 days after birth (Peterson, Ailes et al., 2014). Approximately 62 percent of newborns with coarctation of the aorta are diagnosed late (CDC, 2014e). If narrowing associated with coarctation of the aorta is severe and not detected, the newborn can deteriorate within the first week of life as the ductus arteriosus closes. Pulmonary atresia is the next defect most likely to be diagnosed late (CDC, 2014e; Peterson, Grosse et al., 2014). Recent studies are finding a trend of more late or missed CCHD diagnoses associated with the level of nursery. The majority of babies are born in hospitals with level 1 or 2 nurseries. Nurses working in level 1 nurseries provide basic care for healthy term newborns and use pulse oximetry less frequently. Level 2 special-care nurseries look after babies born around 32 weeks gestation or greater who need closer monitoring and intervention. Level 3 NICUs provide care for babies born after 28 weeks and require the highest level of care. Levels 1 and 2 nurseries don’t maintain the full spectrum of

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Screener Training

MORE TRAINING IN IMPLEMENTING PULSE OXIMETRY SCREENING MAY BE ESPECIALLY HELPFUL FOR THOSE NURSES LESS FAMILIAR WORKING WITH THIS SKILL tee that a CCHD doesn’t exist. Nurse leaders who champion pulse oximetry screening can find excellent resources, such as the Congenital Heart Disease Screening Program Toolkit published by the Children’s National Medical Center (2009), which provides initial resources to guide health care providers in initiating screening for CCHD. The toolkit has evidence-based resources for training individuals responsible for screening, and resources in both English and Spanish for educating families. Many states have created their own toolkits. More training in implementing pulse oximetry screening may be especially helpful for those nurses less familiar working with this skill. Rigorous education of nurses is vital to ensure screening effectiveness, especially in hospitals with only level 1 or 2 nurseries, where newborns seem to have the greatest benefit from pulse oximetry screening for CCHD (Dawson et al., 2013). A study of 6,000 neonates cared for in level 1 or 2 nurseries demonstrated successful detection of CCHD without excessive numbers of false-positives when staff is adequately educated and prepared with the equipment and the protocol. Success of this study was largely attributed to education of staff

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and assigning one or two trained staff members to consistently perform the screenings (Bradshaw et al., 2012). The Congenital Heart Disease Screening Program Toolkit recommends having several nurses assigned to administer daily pulse oximetry screenings. Initial training typically consists of 1 hour of presentation by someone experienced with the protocol and the representative of the pulse oximetry product. A pre- and posttest requiring passing score of ≥90 percent, along with demonstrated pulse oximetry proficiency, is recommended. Accurate reading and documentation of the pulse oximetry readings is also essential. Annual renewal of competencies keeps staff engaged (Children’s National Medical Center, 2009).

cardiology. And some hospitals lack the ability to digitally transmit the echocardiogram to a pediatric cardiologist, while a few don’t even have echocardiography. Telehealth can help stabilize the patient until transfer to a facility with the required resources can be arranged. Neonatal transport teams are faced with managing uncertain diagnoses. An accurate and early diagnosis of the type of CCHD has implications on the treatment and transfer priorities. Whether the congenital heart defect is duct-dependent or not will dictate treatment. Telehealth can help determine the type of defect. Treatment with prostaglandin keeps the ductus arteriosus open for a while and prevents clinical deterioration in a newborn with a ductdependent defect. But this same treatment is unnecessary and may produce unwanted side effects in a newborn with a nonduct-dependent defect. Telehealth can help guide treatment during transportation and stabilize the newborn until surgery is scheduled (Gupta, Kamlin, Cheung, Stewart, & Patel, 2014).

Photo © Fuse Collection / thinkstockphotos.com

Clinical Implications

Follow-Up on Positive Screenings Follow-up treatment includes echocardiograms and referrals to pediatric cardiologists. Hospitals need to have protocols established regarding who and when to call for a positive screening. Decisions on when to transfer and how to treat could mean the difference between life and death. Echocardiography can provide a definitive diagnosis. Larger hospitals in metropolitan areas have access to echocardiograms, in-house residents and pediatric cardiologists, yet hospitals in nonmetropolitan areas may not have echocardiographers experienced in pediatric

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Pulse oximetry screening is a noninvasive, inexpensive and expeditious method to detect CCHD. It’s valuable for identifying disease before symptoms occur. Early diagnosis can potentially improve health outcomes in newborns with CCHD. The costs of the screening are associated with the type of equipment used, number of births at a facility and time for staff to perform the procedure. Pulse oximetry machines generally cost between $900 and $4,000. The probes are about $125. Using the reusable sensors can reduce the cost. Some estimates are that the screen costs from $5 to $15 per infant, although these estimates don’t take into account the cost of follow-up for newborns with a positive screen or the training of personnel who administer the screening. Overall, the cost of screening outweighs the cost of a missed diagnosis. A report from Sweden found that preventing one case of circulatory collapse resulting from a failed diagnosis of CCHD exceeded the cost of screening 2,000 newborns (Kemper et al., 2011). Nurses generally don’t find the time involved in performing the screening burdensome. It takes about 10 minutes or less and is frequently done at the same time as the hearing screening (Peterson, Ailes et al., 2014b).

Looking to the Future Ongoing and future opportunities include continued collection of data to further evaluate effectiveness of screening and

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accuracy of screening for various populations. New research is needed to determine the value of screening newborns outside of hospitals, premature newborns and those in NICUs. Training and frequent updating of knowledge for staff nurses is imperative. Developing electronic health record systems that connect to pulse oximetry devices and automatically trigger when values fall outside the protocol cutoff threshold can help prevent missed follow-up procedures (Kochilas et al., 2013). When pulse oximetry screening is positive, individual hospital protocols need to clearly communicate the next steps.

NURSES CAN BE POWERFUL VOICES AND ADVOCATES IN STATES THAT ARE PURSUING LEGISLATION TO REQUIRE HOSPITALS TO SCREEN FOR CCHD Available resources need to be identified, including who to call for pediatric cardiology consult, where the closest diagnostic echocardiogram is located, telehealth options and available neonatal transport teams. A pulse oximetry screening algorithm may prove useful in identifying other noncardiac secondary conditions. Screening newborns younger than 24 hours old identified life-threatening respiratory conditions and infectious diseases, allowing for prompt intervention and treatment before significant physical deterioration (Kemper et al., 2011; Singh, Rasiah, & Ewer, 2014).

Conclusion Nurses are at the forefront of development, implementation and adaptation of evidence-based protocols and standardized algorithms for screening within their clinical organizations, where they guide the use of technology, are involved in reporting outcomes and serve as facilitators of communication resources for families. Nurses can be powerful voices and advocates in states that are pursuing legislation to require hospitals to screen for CCHD (Kemper, Fant, & Clark, 2005; Maryland & Gonzalez 2012). They are instrumental in uniform collection of birth defect data for surveillance efforts, improvement of health outcomes and legislative action. Individual state action is growing (AAP, 2014). Pulse oximetry screening is allowing more newborns with CCHD to live longer and healthier lives than in the past. Some even go on to have children of their own. Pulse oximetry, a simple, inexpensive, noninvasive screening approach, can make the difference between a healthy, vital life or a life of disability or even death. NWH

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References Ailes, E., Gilboa, S., Colaousso, T., Johnson, C., Hobbs, C., Correa, A., Honein, M., and the National Birth Defects Prevention Study. (2014). Prenatal diagnosis of nonsyndromic congenital heart defects. Prenatal Diagnosis 34(3), 214-222. doi: 10.1002/pt.4282. American Academy of Pediatrics (AAP). (2014). Newborn screening for critical congenital heart disease (CCHD)—2014 state actions. Elk Grove Village, IL: Author. Retrieved from www.aap.org/ en-us/advocacy-and-policy/state-advocacy/Documents/ 2014%20CCHD%20Newborn%20Screening%20Bills,%20 Regulations,%20and%20Executive%20Orders%20-%20 AAP%20Division%20of%20State%20Govt%20Affairs.pdf Andrews, J. P., Ross, A. S., Salazar, M. A., Tracy, N. A., & Burke, B. L., Jr. (2014). Smooth implementation of critical congenital heart defect screening in a newborn nursery. Clinical Pediatrics, 53(2), 173–176. doi:10.1177/0009922813502850 Bradshaw, E., Cuzzi, S., Kiernan, S., Nagel, N., Becker, J., & Martin, G. (2012). Feasibility of implementing pulse oximetry for congenital heart disease in a community hospital. Journal of Periantalogy, 32, 710-715. doi: 10.1038/jp.2011.179 Centers for Disease Control and Prevention (CDC). (2014a). Congenital heart defects: Tracking and research. Atlanta, GA: Author. Retrieved from www.cdc.gov/ncbddd/heartdefects/research.html Centers for Disease Control and Prevention (CDC). (2014b). Congenital heart defects: Facts about critical congenital heart defects. Atlanta, GA: Author. Retrieved from www.cdc.gov/ncbddd/ heartdefects/cchd-facts.html Centers for Disease Control and Prevention (CDC). (2014c). CDC’s tracking and research for the prevention of congenital heart defects. Atlanta, GA: Author. Retrieved from www.cdc.gov/ncbddd/ heartdefects/documents/chd-fact-sheet_508.pdf Centers for Disease Control and Prevention (CDC). (2014d). Congenital heart defects: Information for healthcare providers. Atlanta, GA: Author. Retrieved from www.cdc.gov/ncbddd/heartdefects/ hcp.html Centers for Disease Control and Prevention (CDC). (2014e). Congenital heart defects: Key findings—Estimating the impact of newborn screening for critical congenital heart defects in the United States. Atlanta, GA: Author. Retrieved from www.cdc.gov/ ncbddd/heartdefects/features/keyfindings-screening-impactcchd.html Children’s National Medical Center. (2009). Congenital heart disease screening program toolkit. Retrieved from www.archildrens. org/documents/CHDSPToolkitV210.2011.pdf Dawson, A. L., Cassell, C. H., Riehle-Colarusso, T., Grosse, S. D., Tanner, J. P., Kirby, R. S., … Olney, R. S. (2013). Factors associated with late detection of critical congenital heart disease in newborns. Pediatrics, 132(3), e604–e611.doi:10.1542/peds.2013-1002 Fixler, D. E., Xu, P., Nembhard, W. N., Ethen, M. K., & Canfield, M. A. (2014). Age at referral and mortality from critical congenital heart disease. Pediatrics 134(1), e98–e105. doi:10.1542/ peds.2013-2895 Gupta, N., Kamlin, C., Cheung, M., Stewart, M. & Patel, N. (2014). Improving diagnostic accuracy in the transport of infants with suspected duct-dependent congenital heart disease. Journal of Paediatrics and Child Health, 50(1), 64-70. doi: 10.1111/jpc.12410

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Hines, A. J. (2012). A nurse-driven algorithm to screen for congenital heart defects in asymptomatic newborns. Advances in Neonatal Care 12(3), 151–157. doi:10.1097/ANC.0b013e3182569983 Iyengar, H., Kumar, P., & Kumar, P. (2014). Pulse-oximetry screening to detect critical congenital heart disease in the neonatal intensive care unit. Pediatric Cardiology, 35(3), 406–410. doi:10.1007/s00246-013-0793-2 Kemper, A., Fant, K., & Clark, S. (2005). Informing parents about newborn screening. Public Health Nursing 22(4): 332-338. Kemper, A. R, Mahle, W. T., Martin, G. R., Cooley, W. C., Kumar, P., Morrow, W. R., … Howell, R. R. (2011). Strategies for implementing screening for critical congenital heart disease. Pediatrics 128(5), e1259–e1267. doi:10.1542/peds.2011-1317 Kochilas, L. K., Lohr, J. L., Bruhn, E., Borman-Shoap, E., Gams, B. L., Pylipow, M., … Thompson, T. (2013). Implementation of critical congenital heart disease screening in Minnesota. Pediatrics,132(3), e587–e594. doi:10.1542/peds.2013-0803

Oster, M. E., Lee, K. A., Honein, M. A., Riehle-Colarusso, T., Shin, M., & Correa, A. (2013). Temporal trends in survival among infants with critical congenital heart defects. Pediatrics, 131(5), e1502–e1508. doi:10.1542/peds.2012-3435 Peterson, C., Ailes, E., Riehle-Colarusso, T., Oster, M. E., Olney, R. S., Cassell, C. H., … Gilboa, S. M. (2014). Late detection of critical congenital heart disease among US infants: Estimation of the potential impact of proposed universal screening using pulse oximetry. JAMA Pediatrics, 168(4), 361–370. doi:10.1001/ jamapediatrics.2013.4779 Peterson, C., Grosse, S. D., Glidewell, J., Garg, L. F., Van Naarden Braun, K., Knapp, M. M., … Cassell, C. H. (2014). A public health economic assessment of hospitals’ cost to screen newborns for critical congenital heart disease. Public Health Reports 129(1), 86–93. Ramjattan, K., & Allen, P. J. (2013). Pulse oximetry screening for critical congenital heart disease in the newborn. Pediatric Nursing 39(5), 250–253, 256.

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