DOI: 10.1542/peds.2013-0269; originally published online July 15, 2013;Pediatrics

Olney, Jill Glidewell, Cynthia F. Hinton and Alex R. KemperRobert I. Koppel, Daniel Hirsch, Leslie M. Beres, Joseph Sweatlock, Richard S.

Lorraine F. Garg, Kim Van Naarden Braun, Mary M. Knapp, Terry M. Anderson,Screening Program

Results From the New Jersey Statewide Critical Congenital Heart Defects  

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located on the World Wide Web at: The online version of this article, along with updated information and services, is

 

of Pediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2013 by the American Academy published, and trademarked by the American Academy of Pediatrics, 141 Northwest Pointpublication, it has been published continuously since 1948. PEDIATRICS is owned, PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly

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Results From the New Jersey Statewide CriticalCongenital Heart Defects Screening Program

WHAT’S KNOWN ON THIS SUBJECT: Prenatal diagnosis andclinical examination do not identify all infants with criticalcongenital heart defects before hospital discharge. To improveearly critical congenital heart defect detection, New Jersey wasthe first state to implement legislatively mandated newborn pulseoximetry screening.

WHAT THIS STUDY ADDS: This report is the first to evaluatestatewide pulse oximetry screening implementation. New Jersey hada high statewide screening rate and identified 3 infants withpreviously unsuspected critical congenital heart defects thatotherwise might have resulted in significant morbidity and mortality.

abstractBACKGROUND AND OBJECTIVE: New Jersey was the first state toimplement legislatively mandated newborn pulse oximetry screening(POxS) in all licensed birthing facilities to detect critical congenitalheart defects (CCHDs). The objective of this report was to evaluateimplementation of New Jersey’s statewide POxS mandate.

METHODS: A 2-pronged approach was used to collect data on infantsscreened in all New Jersey birthing facilities from August 31, 2011,through May 31, 2012. Aggregate screening results were submittedby each birthing facility. Data on failed screens and clinical character-istics of those newborns were reported to the New Jersey Birth DefectsRegistry (NJBDR). Three indicators were used to distinguish the addedvalue of mandated POxS from standard clinical care: prenatal congen-ital heart defect diagnosis, cardiology consultation or echocardiogramindicated or performed before PoxS, or clinical findings at the time ofPOxS warranting a pulse oximetry measurement.

RESULTS: Of 75 324 live births in licensed New Jersey birthing facil-ities, 73 320 were eligible for screening, of which 99% were screened.Forty-nine infants with failed POxS were reported to the NJBDR, 30 ofwhom had diagnostic evaluations solely attributable to the mandatedscreening. Three of the 30 infants had previously unsuspected CCHDsand 17 had other diagnoses or non-CCHD echocardiogram findings.

CONCLUSIONS: In the first 9 months after implementation, New Jerseyachieved a high statewide screening rate and established surveillancemechanisms to evaluate the unique contribution of POxS. The screeningmandate identified 3 infants with previously unsuspected CCHDs thatotherwise might have resulted in significant morbidity and mortalityand also identified other significant secondary targets such as sepsisand pneumonia. Pediatrics 2013;132:e314–e323

AUTHORS: Lorraine F. Garg, MD, MPH,a Kim Van NaardenBraun, PhD,a,b Mary M. Knapp, MSN, RN,a Terry M.Anderson, MD,c Robert I. Koppel, MD,d Daniel Hirsch, MD,e

Leslie M. Beres, MS Hyg, CPM,a Joseph Sweatlock, PhD,a

Richard S. Olney, MD, MPH,b Jill Glidewell, MSN, MPH,b

Cynthia F. Hinton, MPH, MS, PhD,b and Alex R. Kemper, MD,MPH, MSf

aSpecial Child Health and Early Intervention Services, Divisionof Family Health Services, New Jersey Department of Health,Trenton, New Jersey; bNational Center on Birth Defects andDevelopmental Disabilities, Centers for Disease Control andPrevention, Atlanta, Georgia; cDepartment of Pediatrics, TheChildren’s Hospital of Philadelphia and University of PennsylvaniaPerelman School of Medicine, Philadelphia, Pennsylvania;dDepartment of Pediatrics, Cohen Children’s Medical Center, NewHyde Park, New York; eDepartment of Pediatrics, University ofMedicine and Dentistry New Jersey, Robert Wood JohnsonMedical School, New Brunswick, New Jersey; and fDuke ClinicalResearch Institute and Department of Pediatrics, Duke University,Durham, North Carolina

KEY WORDSoximetry, congenital heart disease/defects, newborn screening,surveillance and monitoring

ABBREVIATIONSCCHD—critical congenital heart defectCHD—congenital heart defectNICU/SCN—neonatal intensive care unit/special care nurseryNJBDR—New Jersey Birth Defects RegistryNJDOH—New Jersey Department of HealthPOxS—pulse oximetry screeningWBN—well-baby nursery

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Approximately 2 per 1000 live births inthe United States are infants bornwith critical congenital heart defects(CCHDs), of which an estimated 20%are neither diagnosed prenatally noridentified by clinical examination be-fore hospital discharge, with widevariation by specific type of CCHD.1–3

New Jersey was the first state to im-plement legislatively mandated new-born pulse oximetry screening (POxS)to improve early detection of CCHDs.Legislation signed into law on June 2,2011, and implemented 90 days later onAugust 31, requires all licensed birth-ing facilities to “perform a pulse oxi-metry screening a minimum of 24hours after birth, on every newborn inits care.”4 This requirement applies toall live births regardless of healthstatus, prenatal diagnosis of CCHDs, orlocation (eg, NICU/special care nursery[SCN], well-baby nursery [WBN]). Tofacilitate implementation of the screen-ing requirement, the New Jersey De-partment of Health (NJDOH) conveneda working group that developed a rec-ommended screening algorithm thatspecified screening in both the righthand and either foot between 24 and 48hours of life or when medically appro-priate after 24 hours of age (Appendix 1).In all cases it is recommended forscreening to be done before discharge.The NJDOH also developed an approachusing surveillance mechanisms to eval-uate the POxS program.

In September 2011, the US Secretary ofHealth and Human Services recom-mended that all states incorporatescreening for CCHDs into their new-born screening panel.5 At least 35 stateshave passed or introduced legislation tomandate this screening.6 We present thefirst 9 months of screening experiencein New Jersey, including screeningrates and case detection, to highlightthe benefits, challenges, and issues forconsideration that might help guideimplementation in other states.

METHODS

Defining CCHD

We defined CCHD to include thosedefects that usually cause hypoxemia(ie, hypoplastic left heart syndrome,pulmonary atresia, Tetralogy of Fallot,total anomalous pulmonary venousreturn, transposition of great arteries,tricuspid atresia, truncus arteriosus)and those significant defects thatsometimes, but less consistently, causehypoxemia (ie, coarctation of aorta,double outlet right ventricle, singleventricle, interrupted aortic arch,Ebstein anomaly).1,7

Data Sources

Screening Data Collection

All birthing facilities (52 facilities inoperation before October 2011 and 53after that date) submit aggregate dataon a quarterly basis to the NJDOH,which includes the number of livebirths, the number screened, and thenumber of failed screening results.For purposes of this evaluation, theterm “live births” refers to all infantsborn alive at the 53 licensed birthingfacilities in New Jersey. A standardform is used to collect these data andto facilitate consistent reporting, in-cluding information about newbornswho were not screened (eg, trans-fers, deaths, missed for unknownreasons) (Appendix 2). This reportreflects the first 9 months after POxSimplementation (August 31, 2011, toMay 31, 2012).

New Jersey Birth Defects Registry

Health careprofessionals are requiredby law to report infants with CCHDswho are New Jersey residents to theNew Jersey Birth Defects Registry(NJBDR) as soon as possible after di-agnosis.8 As a result of the mandate,the NJBDR was expanded to monitorfailed POxS. With the use of a new di-agnostic code for failed POxS, all

birthing facilities were requested toreport individual-level data on infants’failed POxS results, regardless ofpresence of CCHDs, to the NJBDR. Thisreport includes the time of birth,screening results, findings from thediagnostic evaluation (eg, echocardio-gram), any relevant prenatal diagnoses,and other clinical characteristics suchas whether the infant was asymp-tomatic at the time of screening. Forfailed screens, the NJBDR staff in-vestigated cases in which the infor-mation was unclear or if a CCHD wasidentified as a result of mandatedPOxS. The NJBDR staff also inves-tigated registered cases of CCHDs thathad no accompanying report of failedPOxS to the NJBDR.

Assessment of the Impact of theScreening Mandate

Aggregate reports were submittedevery 3 months and were used to de-termine overall screening coverage. Todetermine the proportion of eligiblelive births who were screened, weexcluded those live births who werenot screened because they died beforescreening, were not medically appro-priate to have been screened (eg, re-ceiving oxygen in the NICU), were,24hours old at the end of the reportingperiod, or were transferred to an-other hospital before 24 hours of age.To avoid possible double counting,we also excluded (1) live births whowere not screened and transferredout of a hospital after 24 hours of agefrom the total live-birth count (Fig 1,Appendix 2) and (2) live births whowere screened and transferred intoa New Jersey birthing facility at any-time during the reporting periodfrom the number of reported screens(Appendix 2).

The NJBDR was used to determineoutcomes for those with a failed screenand to monitor CCHD cases. Threeclinical indicators were used to

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determine whether the diagnosticevaluation was solely attributable toPOxS or whether it would otherwisehave occurred in the absence of thePOxS mandate. These indicators in-cluded a prenatal diagnosis of con-genital heart defects (CHDs), whethera cardiology consultation or echocar-diogram was indicated or performedbefore screening on the basis of clin-ical factors (eg,maternal history, signsof CHD), or whether there were clinicalfindings at the time of the screen that

would have otherwise warranted apulse oximetry measurement.

Data Analysis

x2 Tests of significance were used toexamine differences in the proportionsof missed screens over the three,3-month aggregate data reportingperiods.

This project was determined by theNJDOH Institutional Review Board to bepublic health practice.

RESULTS

Over the 9-month study period, 75 324live births were reported from thelicensed birthing facilities in NewJersey, of whom 97.3% (n = 73 320)were eligible for screening (Fig 1). Ofthose eligible to be screened, 99.1%underwent POxS. The proportion ofmissed screens decreased substan-tially over the study period from 1.8%in the first period to 0.2% in the thirdperiod (P, .001). Ninety-seven infantswere reported to the NJBDR witha failed POxS, CCHD, or both.

Infants With Failed POxS Reportedto the NJBDR

Forty-nine infantswere reported to theNJBDR with a failed POxS (Fig 2). Thirtyof the 49 infants (61%) with a failedPOxS had none of the 3 clinical indi-cators and therefore their diagnosticevaluation was solely attributable totheir failed screen. Of these 30 infants,3 had CCHDs that otherwise mighthave resulted in serious morbidity ormortality if diagnosis had beendelayed in the absence of screening.All 3 infants were in the WBN at thetime of the screen, none had a pre-natal diagnosis of CHD or a cardiacconsult or echocardiogram indicatedbefore the POxS, and none had clinical

FIGURE 1Aggregatepulseoximetry screening results fromall licensedNewJerseybirthing facilities for livebirthsfrom August 31, 2011, to May 31, 2012. aReported reason for having not been screened. NJ, New Jersey.

FIGURE 2Failedpulseoximetryscreensreported to theNewJerseyBirthDefectsRegistry forNewJersey–resident livebirthsscreened fromAugust31, 2011, toMay31, 2012.

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signs present at the time of the POxSthat would have otherwise warranteda pulse oximetry measurement(Table 1). Seventeen of the 30 infantswho received a diagnostic evaluationas a result of a failed POxS had otherdiagnoses, including culture-negativesepsis, pneumonia, pulmonary hyper-tension, other types of CHDs (eg,ventricular septal defect) or otherechocardiogram findings (eg, patentformen ovale or patent ductus arte-riosis). Ten of the 30 infants had nocardiac abnormalities found on echo-cardiogram, and no other diagnoses

were made on the basis of the avail-able follow-up information (Fig 2,Table 1).

The remaining 19 infants with a failedPOxS had 1 of the 3 clinical indicators,suggesting that the mandated POxSwould not have changed the alreadyindicated evaluation and subsequentclinical management of these infants(Fig 2). Therefore, their diagnosticevaluation was not solely attributableto the mandated POxS. Four of theseinfants had CCHDs (transposition ofthe great arteries, hypoplastic leftheart syndrome, total anomalous

pulmonary venous return, interruptedaortic arch). Of the remaining 15, 13had other forms of CHDs or echocar-diogram findings (eg, bicuspid aorticvalve, patent formen ovale, patentductus arteriosis) or other noncar-diac diagnoses (eg, pneumothorax),and 2 reportedly had no diagnosismade.

Infants With CCHDs but No FailedPOxS Reported to the NJBDR

Forty-eight infants were reported tothe NJBDR with CCHDs without anaccompanying failed POxS reported to

TABLE 1 POxS Results, Echocardiogram Findings, and Other Relevant Diagnoses for Infants Whose Diagnostic Clinical Evaluation Was Attributable toFailed POxS

Case Number Location Last Screen Result, % Main Echocardiogram Findings Other Relevant Diagnoses

Preductal Postductal

1a WBN 43 39 d-TGAb None2a WBN 91 92 TAb None3a WBN 97 84 Aortic coarctation, aortic arch hypoplasia, BAVb None4c WBN 88 90 Echocardiogram not done Clinical sepsis (culture negative)5c WBN 50 70 Echocardiogram not done Pneumonia6c WBN 91 91 VSD (left to right shunting), PFO None7c WBN 93 100 PFO (left to right shunting) versus ASD None8c WBN 87 80 PFO (direction of flow not stated) None9c WBN 94 100 PPS, PFO (bidirectional shunting), mild pulmonary

hypertensionNone

10c WBN 96 93 Small PFO with bidirectional flow None11c WBN 91 91 PDA, PFO (direction of flow not stated) TTN12c WBN 90 90 multiple VSDs, ASD, and PPSb None13c WBN 94 96 Small PFO (right to left shunting) None14c WBN 87 98 Small PFO (left to right shunting)b None15c WBN 95 100 Echocardiogram not done Poor suck-swallow coordination16 WBN 93 96 No reported cardiac abnormalities None17 WBN 94 99 No reported cardiac abnormalities None18 WBN 95 99 No reported cardiac abnormalities None19 WBN 94 98 No reported cardiac abnormalities None20 WBN 88 92 No reported cardiac abnormalities None21 WBN 96 100 Echocardiogram not done None22 WBN 100 96 Echocardiogram not done None23c NICU/SCN 97 91 PFO, PDA (left to right shunting), mild pulmonary

hypertensionbHistory of meconium aspirationd

24c NICU/SCN 92 96 VSD (left to right shunting), PFO (left toright shunting),

None

25c NICU/SCN 88 97 No reported cardiac abnormalities History of meconium aspirationd

26c NICU/SCN 91 98 No reported cardiac abnormalities History of mild respiratory distressd

27c NICU/SCN 94 94 Echocardiogram not done History of RDSd

28 NICU/SCN 94 97 Echocardiogram not done None29 NICU/SCN 94 97 Echocardiogram not done None30 NICU/SCN 97 94 Echocardiogram not done None

Data represent New Jersey resident live births screened from August 31, 2011, to May 31, 2012. ASD, atrial septal defect; BAV, bicuspid aortic valve; d-TGA, dextro transposition of great arteries;PDA, patent ductus arteriosus; PFO, patent foramen ovale; PPS, peripheral pulmonary stenosis; TA, tricuspid atresia; TTN, transient tachypnea of newborn; RDS, respiratory distress syndrome;VSD, ventricular septal defect.a Infants with CCHD.b Transferred out of birthing facility.c Infants with other relevant diagnoses or non-CCHD echocardiogram findings.d Infant appeared to have clinical resolution of this condition at time of screen.

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the registry (Table 2). These infantshad 3 identified reasons for nothaving an accompanying failed POxSregistered. The infants could have (1)passed the POxS, (2) failed the POxSwithout being reported, or (3) mightnot have been screened. Based onavailable clinical information, at least98% of these infants with CCHDs but noaccompanying failed POxS registered,experienced $1 of the following: aprenatal CHD diagnosis, transfer out ofthe birthing hospital within 1 day of life,clinical monitoring with pulse oximetryin the NICU/SCN, or passing POxS results.

DISCUSSION

This article represents the first pub-lished report in the United States froma legislatively mandated, statewidePOxS program for all live hospitalbirths. In the first 9 months after theNew Jersey mandate, ∼73 000 infantswere screened, leading to the identi-fication of 3 infants with previouslyunsuspected CCHDs. Consistent withfindings from other studies, New Jer-sey’s POxS program identified severalnewborns with other potentially im-

portant and unsuspected conditionsor CHDs.2,9–13 Similar to other time-sensitive biochemical newborn screen-ing tests for related metabolic andendocrine disorders, our findings sup-port that POxS can improve the timing ofdiagnosis of and intervention for CCHDs.

Although our findings address theclinical utility of POxS, information onrelated costs and resource use forstatewide POxS implementation is lim-ited. Recentdata fromarandomsampleof New Jersey hospitals indicate thatthe estimated costs of POxS per new-born ($14) is lower than that for labo-ratory metabolic screening ($20) andhospital-based hearing screening($36–$39, adjusted to 2011 dollars).14–16

However, these estimates do not includecosts incurred from follow-up diag-nostic evaluations and/or medical in-tervention as a result of the failed POxS.Our data indicated that the number ofechocardiograms conducted as a resultof failed mandatory POxS for infantswith no subsequent potentially signifi-cant findings was only 7 over this 9-month period. In addition, it does notappear that resources for transferringinfants who failed the POxS were usedunnecessarily because at least 5 of the6 infants whose transfers were trig-gered solely by the failed POxS had po-tentially significant echocardiogramfindings. Importantly, many of the in-fants who failed the mandatory screenhad previous clinical history or signsthat would have triggered a subsequentdiagnostic evaluation regardless of thestatewide mandate, hence requiring noadditional resources. Although we donot know how many of the 48 infantswith CCHDs without a registered failedscreen would have actually failed thePOxS, for most of these infants, themandated POxS likely would not havecaused additional resource use beyondthat which was already clinically in-dicated.

Implementing and evaluating state-wide POxS necessitated a bridging oftraditional newborn screening andbirth defects surveillance perspec-tives. Because most previous studiesassessing POxS excluded symptom-atic and/or prenatally diagnosedinfants,2,9–11,13,17–19 there was noexisting approach for implementingand/or evaluating the POxS for all livebirths, including infants in the NICUand those prenatally diagnosed withCCHDs. Within 90 days of the signingof the legislative mandate, the NJDOHdeveloped a recommended screeningalgorithm, and the POxS mandate wasimplemented for all live births in allNew Jersey birthing hospitals with94% of WBNs and 88% of NICU/SCNsreporting to have adopted the algo-rithm specifically recommended bythe NJDOH. Data from a recent in-vestigation in New Jersey revealedthat the addition of the new man-dated screening processes posedminimal burden on hospital nursingstaff.20

From the New Jersey experience, itis clear that to evaluate the addedvalue of a statewide POxS mandate,establishing mechanisms or usingexisting systems for surveillance isnecessary. In New Jersey, as is pos-sible in other states, there was nofeasible, immediate mechanism (eg,bloodspot card or electronic birthcertificate) to quickly incorporateindividual-level screening results anddetails on infants’ clinical charac-teristics into a statewide, systematicreporting system. Results from NewJersey’s universal newborn hearingscreening program, another state-wide point-of-care screening pro-gram, are captured within NJDOH’selectronic birth certificate. At thetime of implementation of POxS, theelectronic birth certificate was beingreengineered and was not modifiablestatewide in all birthing facilities. The

TABLE 2 Number of Children With CCHDsReported to the New Jersey BirthDefects Registry Without Failed POxS

Type of CCHD n (Total N = 48)

Tetralogy of Fallot 15Coarctation of aorta 13Transposition of great arteries 4Double outlet right ventricle 2Hypoplastic left heart syndromeand coarctation of aorta

2

Interrupted aortic arch 2TAPVR 2Truncus arteriosus 2Ebstein anomaly 1Hypoplastic left heart syndromeand single ventricle

1

Tetralogy of Fallot andpulmonary atresia

1

Transposition of great arteriesand single ventricle

1

Tricuspid atresia 1Tricuspid atresia, pulmonaryatresia, and TAPVR

1

Data represent New Jersey resident live births from Au-gust 31, 2011 to May 31, 2012. TAPVR, total anomalouspulmonary venous return.

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introduction of aggregate screeningdata reports and individual-levelreporting of failed screens to theNJBDR enabled us to quickly capturedata to evaluate our statewide man-date. We found that providing a pre-scriptive template for the aggregatedata reporting request was quiteuseful in obtaining consistent re-ports across all 53 birthing facilities.Understanding the discrepanciesbetween the number of live birthsand number of infants screened en-abled us to adjust the proportion oflive births screened to more accu-rately reflect infants eligible to bescreened. Therefore, we believe theaggregate-level data provide us witha close approximation of implemen-tation coverage. The planned imple-mentation of a new statewide electronicbirth reporting system will enable us tocollect individual-level data and there-fore provide a more accurate reconcil-iation of infants transferred. Use of theNJBDR to collect detailed relevant clin-ical characteristics (eg, whether CHDwas prenatally detected, a cardiacconsult was indicated before the POxS,and/or clinical signs were present atthe time of the POxS) of infants whofailed the POxS was crucial to evaluatethe unique contribution of POxS. Assuch, we anticipate that the NJBDR willcontinue to be a necessary tool forcollection of detailed information oninfants with failed POxS.

Our data collection efforts suggestedthat some degree of underreportingmay have occurred. In particular, theidentification of 48 infants with CCHDswithout a registered failed POxS re-vealed that pulse oximetry was notconsistently used or reported as ascreening tool. With high relevance toimplementation of a statewide man-date, these findings underscore thattraining and education are needed toemphasize that all infants are requiredtoreceive themandatedPOxSregardless

of their location in the hospital or pre-natal diagnosis and any infant that failsthe screening protocol should be re-ported to the NJBDR. Our findings alsohighlight the need to clarify and em-phasize the distinction between the useof pulse oximetry aspart of a diagnosticassessment and its use as a universalscreening tool. These potential sourcesof underreporting of failed screensappear to bemost relevant to NICU/SCNinfants or those with preexisting di-agnoses or symptoms rather thanthose in theWBN. In addition, the NJDOHreceived reports of several infantswhose failed screenswere erroneouslydeemed a “pass” by hospital staff. Thisfurther highlights the need for signifi-cant and ongoing education, outreach,and technical support for health careproviders and nursery staff. Perform-ing audits to determine the extent ofunderreporting at the hospital level isan essential part of future qualityassurance activities as is encourag-ing hospitals to establish their owninternal audit protocols. Quality as-surance and training efforts in NewJersey need to also focus on POxStechniques (eg, sensor placement)and interpretation of results to as-sess and ensure the validity of POxSimplementation.

The New Jersey experience producedmeaningful qualitative and quantitativeinformation for evaluating and im-proving our program as well as forassisting other states as they beginimplementation and evaluation of POxS.Nevertheless, generalization of ourobserved number of infants with failedPOxS and/or CCHDs to what otherstates might expect should be donewith consideration of a few limitations.The lower number of infants with CCHDsand/or failed POxS in New Jersey,compared with other studies,10,11 islikely attributable to several factors,with prenatal detection being a majorconsideration.21 Given the close geo-

graphic proximity to major out-of-statepediatric cardiac surgical centers, NewJersey resident mothers of infantswith prenatally diagnosed CCHDs maychoose to deliver in a neighboringstate. In addition, infants receiving careexclusively out of state are not rou-tinely reported to the NJBDR. Althoughthe overall proportion of New Jerseyresident infants with CCHDs prenatallydiagnosed is not known, the estimatemay be higher than the 36% to 50%previously reported1,11 due to the in-creased use of ultrasounds in routineprenatal care22 as well as the signifi-cant training efforts23 in parts of NewJersey. A higher prenatal detection ratecoupled with the possibility of infantswith prenatally detected CCHDs beingborn and solely receiving care out ofstate could result in an underes-timation of CCHD cases and infantswith reported failed POxS in the NJBDR.Although this report is well beyond theaverage time from birth to the age ofregistration in the NJBDR (∼50 days), itis still possible that additional infantswith CCHDs may be reported to theNJBDR due to late diagnosis or latereporting.24 Other potential sources ofunderascertainment include providersnot screening infants, providers notreporting infants with a failed POxS, andexclusion of infants born in New Jerseyto mothers who were residents of an-other state. Historically, the NJBDR hasonly included infants born to New Jer-sey residents, although it has recentlybeen modified to accommodate regis-tration of failed POxS for out-of-stateresidents born in New Jersey.

The NJDOH’s approach and data col-lection mechanisms used to evaluatethe statewide POxS mandate may pro-vide insight and guidance to otherstates implementing or consideringsimilar legislation. Despite the chal-lenges of a short implementation pe-riod and being first in the nation toimplement statewide POxS, the NJDOH

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was able to implement a statewidescreening effort with a reported highcoverage rate, a means to track theproportion of the population screened,and a mechanism by which to obtainrelevant clinical information on thoseinfants with a failed POxS. As otherstates plan and evaluate their POxSprograms, we encourage consider-ation of a number of factors includingobtaining information on clinical indi-cators, exploring the role of prenataldiagnosis, and assessing the ability ofsurveillance systems to ascertain allfailed screens and CCHD cases born in

and/or to residents of the state.25 Na-tional efforts are currently underwayto develop standardized protocols andminimal core data sets and to facilitatesharing of information between stateswith the ultimate goal of creating moreefficient and effective programs to im-prove the health of infants. Froma statewide experience, the resultsfrom the first 9 months in New Jerseyrevealed that mandated POxS suc-cessfully identified 3 infants with pre-viously undetected CCHDs who likelywould have been discharged from thehospital in the absence of a failed POxS,

potentially resulting in significant mor-bidity or death.

ACKNOWLEDGMENTSWe thank Dr Margaret Honein for herscientific review of the manuscript,Drs Matt Oster and Tiffany Riehle-Colarusso for their clinical insight onCHDs, Drs Suzanne Gilboa and ElizabethAiles for their input regarding prenatalidentificationofCHDs, and thestaff atall53 New Jersey birthing facilities fortheir participation in all aspects ofthe implementation of pulse oximetryscreening.

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19. Thangaratinam S, Brown K, Zamora J, KhanKS, Ewer AK. Pulse oximetry screening forcritical congenital heart defects in asymp-tomatic newborn babies: a systematic re-view and meta-analysis. Lancet. 2012;379(9835):2459–2464

20. Centers for Disease Control and Pre-vention. Rapid implementation of pulseoximetry newborn screening to detectcritical congenital heart defects—NewJersey, 2011. MMWR Morb Mortal Wkly Rep.2013;62(15):292–294

21. Walsh W. Evaluation of pulse oximetryscreening in Middle Tennessee: cases forconsideration before universal screening.J Perinatol. 2011;31(2):125–129

22. Friedberg MK, Silverman NH, Moon-GradyAJ, et al. Prenatal detection of congenitalheart disease. J Pediatr. 2009;155(1):26–31, e1

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23. Levy DJ, Pretorius DH, Rothman A, et al.Improved prenatal detection of congenitalheart disease in an integrated health caresystem. Pediatr Cardiol. 2013;34(3):670–679

24. Aamir T, Kruse L, Ezeakudo O. Delayed di-agnosis of critical congenital cardiovascular

malformations (CCVM) and pulse oximetryscreening of newborns. Acta Paediatr. 2007;96(8):1146–1149

25. Olney RS, Botto LD. Newborn screeningfor critical congenital heart disease:essential public health roles for birthdefects monitoring programs. Birth Defects

Res A Clin Mol Teratol. 2012;94(12):965–969

26. State of New Jersey Department of Health.Critical Congenital Heart Defects (CCHD)Screening Resources. Available at: www.nj.gov/health/fhs/nbs/cchd_resources.shtml.Accessed June 9, 2013

(Continued from first page)

Dr Garg conceptualized and designed all aspects of the study, assisted in data acquisition, analyzed and interpreted study data, drafted the initial manuscript,revised it critically for important intellectual content, and approved the final version for submission; Dr Van Naarden Braun conceptualized and designed all aspectsof the study, acquired study data, analyzed and interpreted data, drafted the initial manuscript, revised it critically for important intellectual content, and approvedthe final version for submission; Ms Knapp assisted in study design and conceptualization, acquired study data, analyzed and interpreted data, reviewed andrevised the manuscript critically for important intellectual content, and approved the final version for submission; Drs Anderson, Koppel, Hirsch, Olney, Hinton, andKemper assisted in interpretation of study data, reviewed and revised the manuscript critically for important intellectual content, and approved the final version forsubmission; Ms Beres assisted in study design and conceptualization, analyzed and interpreted data, reviewed and revised the manuscript critically for importantintellectual content, and approved the final version for submission; Dr Sweatlock analyzed and interpreted study data, reviewed and revised the manuscriptcritically for important intellectual content, and approved the final version for submission; and Ms Glidewell assisted in study conceptualization and interpretationof study data, reviewed and revised the manuscript critically for important intellectual content, and approved the final version for submission.

The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control andPrevention or the New Jersey Department of Health.

www.pediatrics.org/cgi/doi/10.1542/peds.2013-0269

doi:10.1542/peds.2013-0269

Accepted for publication May 20, 2013

Address correspondence to Lorraine F. Garg, MD, MPH, Division of Family Health Services, New Jersey Department of Health, PO Box 364, Trenton, NJ 08625-0364.E-mail: lori.garg@doh.state.nj.us

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).

Copyright © 2013 by the American Academy of Pediatrics

FINANCIAL DISCLOSURE: The authors indicated they have no financial relationships relevant to this article to disclose.

FUNDING: No external funding.

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APPENDIX 1New Jersey Department of Health’s recommended pulse oximetry screening algorithm used during thestudy period. Subsequent to the study period, the initial algorithm shown above was updated.26

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APPENDIX 2 Aggregate Data–Reporting Template

Date of reporting period:Hospital name:

Total(1) Number of live births born at birthing facility during current reporting perioda

(2) Number of live births screened with pulse oximetry during current reporting perioda

(3) Number of failed screens

Explanation of live births screened and not screened in current reporting periodLive births who were screened at your birthing facility in current reporting period(a) Number born and screened at your birthing facility in current reporting period(b) Number born at your birthing facility in previous reporting period, but screened

in current reporting period(c) Number not born at your birthing facility, but transferred into your birthing

facility and screened in current reporting period–Name(s) of hospital(s) transferred from and number of infants transferred

(d) Other explanation(s), provide number of infants for each explanation:

Live births born at your birthing facility during current reporting period who were notscreened in current reporting period(e) Number of expirations(f) Number not medically appropriate to screen(g) Number born at birthing facility, but ,24 hours of age at end of current

reporting period(h) Number transferred out of your birthing facility at ,24 hours

–Name(s) of hospital(s) transferred to and number of infants transferred(i) Number transferred out of your birthing facility at $24 hours

–Name(s) of hospital(s) transferred to and number of infants transferred(j) Other explanation(s), provide number of infants for each explanation:

Definitions for data items above:(1) Number of infants in the WBN and NICU/SCN who were born at your birthing facility during the current reporting period.This number does not represent all admissions (ie, excludes those transferred into your facility).(2) Number of infants screened at your birthing facility during the current reporting period. This number includes infantsborn at your facility and those transferred into your facility who were screened.(3) Number of infants who failed the pulse oximetry screening at your birthing facility in the current reporting period.(a) Number of infants both born and screened at your birthing facility during the current reporting period.(b) Number of infants born at your birthing facility during the previous reporting period, but who were screened in thecurrent reporting period. This number is likely to represent infants who were previously medically unstable or too young tobe screened in the previous reporting period.(c) Number of infants not born at your birthing facility but who were transferred into your birthing facility and screenedduring the current reporting period. Below list the name of the hospital the infant was transferred from. If transfers werereceived from multiple hospitals, create a row for each hospital. Include the number of infants who were admitted.(d) Explanation of why an infant was screened in the current reporting period, if not listed above (eg, home births).(e) Number of infants born at your birthing facility during the current reporting period that expired before conducting thepulse oximetry screening.(f) Number of infants born at your birthing facility during the current reporting period who were not medically appropriatefor pulse oximetry screening. This number is primarily applicable to NICU infants.(g) Number of infants born at your birthing facility during the current reporting period whowere,24 hours of age at the endof the current reporting period and were therefore were not screened during the current reporting period.(h) Number of infants born at your birthing facility during the current reporting period who were not screened and weretransferred out at,24 hours. Below list the name of the hospital the infant was transferred to. If infants were transferred todifferent hospitals, create a row for each hospital. Include the number of infants who were transferred.(i) Number of infants born at your birthing facility during the current reporting period who were not screened and weretransferred out of your birthing facility at $24 hours. Below list the name of the hospital the infant was transferred to. Ifinfants were transferred to different hospitals, create a row for each hospital. Include the number of infants who weretransferred.(j) Explanation of why an infant was not screened, if not listed (eg, discharged, 24 hours, parents refused screen, etc).a Important: Infants who were in both the WBN and the NICU or SCN during the current reporting period should only becounted once for all reporting items above.

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DOI: 10.1542/peds.2013-0269; originally published online July 15, 2013;Pediatrics

Olney, Jill Glidewell, Cynthia F. Hinton and Alex R. KemperRobert I. Koppel, Daniel Hirsch, Leslie M. Beres, Joseph Sweatlock, Richard S.

Lorraine F. Garg, Kim Van Naarden Braun, Mary M. Knapp, Terry M. Anderson,Screening Program

Results From the New Jersey Statewide Critical Congenital Heart Defects  

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