oxygen saturation screening for critical congenital … · oxygen saturation screening for critical...

DOI: 10.1542/neo.13-12-e7242012;13;e724Neoreviews

Balaji Govindaswami, Priya Jegatheesan and Dongli SongOxygen Saturation Screening for Critical Congenital Heart Disease

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Oxygen Saturation Screening for CriticalCongenital Heart DiseaseBalaji Govindaswami,

MBBS, MPH,

Priya Jegatheesan, MD,

Dongli Song, MD, PhD

Author Disclosure

Drs Govindaswami,

Jegatheesan, and Song

have disclosed no

financial relationships

relevant to this article.

This commentary does

contain a discussion of

an unapproved/

investigative use of

a commercial product/

device.

Educational Gaps

1. Prenatal diagnosis of CCHD in the United States is 25% to 50%. Clinical

examination remains nonspecific and insensitive. Does universal saturation screening

provide additional opportunity to prevent neonatal mortality from undiagnosed

CCHD?

2. There is no linkage of the oxygen saturation screening and birth hospital data to

congenital heart disease databases and registries to identify all false-negative cases.

3. Cost-effectiveness data for oxygen saturation screening are lacking, particularly

regarding the cost of long-term mortality and morbidity related to delayed diagnosis.

AbstractCritical congenital heart disease (CCHD) refers to lesions of the cardiovascular system,present at birth, which if left untreated in early infancy, will severely compromise theinfant’s well-being and survival. Transposed great arteries, hypoplastic left heart, totalanomalous pulmonary venous drainage, coarctation of the aorta, and interrupted aor-tic arch account for more than 70% of CCHD. Until recently, clinical examinationfollowed by blood gas analysis (100% oxygen challenge) and echocardiogram havebeen the mainstays for diagnosis. However, these methods are unsatisfactory in pre-venting missed diagnosis before discharge in hospital-born infants. Prenatal diagnosisresults in 25% to 50% detection of CCHD in the United States at the present time. Inthe last 10 to 15 years, noninvasive transcutaneous pulse oximetry has provided themeans and impetus for blood oxygen saturation screening as an adjunct to traditionalscreening methods. It now seems that sufficient evidence exists to embrace universaloxygen saturation screening as one of the newborn screening tests before dischargefrom the hospital. The optimal method for universal oxygen saturation screening re-mains debatable, continues to be studied, and is evolving. Nevertheless, the currentstate of universal oxygen saturation screening should help provide reassurance tothe scientific, medical, and health policy communities that this is yet another exampleof good preventive medicine at work in pediatrics and newborn medicine.

Objectives After completing this article, readers should be able to:

1. Define critical congenital heart disease (CCHD) and recognize its clinical importance.

2. Describe current knowledge gaps in optimal universal screening for CCHD.

3. Understand limitations of universal oxygen saturation

screening in detecting CCHD.

4. Describe performance test characteristics for universal

oxygen saturation screening based on the most recent

evidence-based reviews.

IntroductionConsensus statements by the American Academy of Pediat-rics and the American Heart Association support a growingmovement regarding universal oxygen saturation screening

Abbreviations

CCHD: critical congenital heart diseaseCHD: congenital heart diseaseCI: confidence intervalHLH: hypoplastic left heartPPI: peripheral perfusion indexTGA: transposition of the great arteries

Santa Clara Valley Medical Center, San Jose, CA.

Article cardiovascular disorders

e724 NeoReviews Vol.13 No.12 December 2012



for critical congenital heart disease (CCHD) currentlydetected by using transcutaneous pulse oximetry. (1)(2)(3) There is good evidence (2)(3) for universalscreening, but cost-effectiveness data remain a concern.In the United Kingdom, previous estimates (4) sug-gested lack of cost-effectiveness, but more recent esti-mates indicate otherwise. (5)(6) Data from Sweden(7) imply that the screening programs are cost-effective,and several European nations have embraced universalscreening. (8)(9) Preliminary data from the UnitedStates suggest that such screening is likely to be cost-effective. (10) The United States, along with other na-tions with much larger populations of live births (eg,China), are now beginning to initiate universal oxygensaturation screening programs in newborns.

DefinitionCongenital heart disease (CHD) affects 8 per 1,000 livebirths and refers to structural disorders of the heartand great vessels that are present at birth. Globally,CHD affects over one million live births annually andis the leading cause of infant mortality attributable tobirth defects. (11) Although there is no universallyagreed on definition of critical CHD (CCHD), it usuallyrefers to CHD that requires surgical or interventionalcardiologic management in the first year after birth, typ-ically in early infancy, to prevent mortality and/or se-vere morbidity. The most common types of CCHDinclude transposition of the great arteries (TGA), hypo-plastic left heart (HLH), total anomalous pulmonary ve-nous drainage, and coarctation/interruption of theaorta. Pulmonary atresia, tricuspidatresia, critical obstruction of eitherventricular outflow tracts, and sev-eral other complex cardiac lesionsare less common forms of CCHD.

Advocacy for UniversalOxygen Saturation ScreeningUniversal newborn screening has itsroots in parent advocacy, and uni-versal oxygen saturation screeningis no exception. Mothers and fami-lies with infants affected by CCHD,some of whom did not survive in-fancy, were led by Annamarie Saa-rinen and inspired the Secretary’sAdvisory Committee on HeritableDisorders in Newborns and Chil-dren to add CCHD to its list ofdisorders that receive universal

screening before hospital discharge. By early 2010, per-suasive arguments had been made by pediatric cardiolo-gists in the lay press (12) and in the medical literature:“There is no reason not to use pulse oximetry in thenewborn nursery.” (13) The delay of universal screeningfor CCHD in the United States has been compared withdelays in instituting the “Back to Sleep” campaign forsudden infant death syndrome, and the implicationsfor mortality may be parallel. Affected mothers also en-listed the help of US institutions already involved in uni-versal screening, including those led by a pediatriccardiologist in Washington, DC, as well as our own earlyexperience from San Jose, California. In October 2010,these combined advocacy efforts led to the Secretary’sAdvisory Committee on Heritable Disorders in New-borns and Children recommendations to Health andHuman Services Secretary Kathleen Sebelius to includeCCHD in universal screening. Subsequently, severalstakeholders from affected families, governmental agen-cies, the March of Dimes, professional societies, indus-try, and others have had two meetings at Heart House,the headquarters for the American College of Cardiol-ogy in Washington, DC (under the leadership of theAmerican Heart Association, the American College ofCardiology, and the American Academy of Pediatrics)in January 2011 and February 2012 (Fig 1). Currently,efforts are underway to shape national legislation inhealth policy in several states, actively led by parent ad-vocacy groups such as the Newborn Coalition and1:100, which maintain updates of progress on the na-tional front (Fig 2).

Figure 1. Timeline illustrating landmark events related to universal oxygen saturationscreening in the United States. AAP[American Academy of Pediatrics; AHA[AmericanHeart Association; SACHDNC[Secretary’s Advisory Committee on Heritable Disorders inNewborns and Children.

cardiovascular disorders critical congenital heart disease

NeoReviews Vol.13 No.12 December 2012 e725



Epidemiology and Global PerspectivePrenatal Detection

Up to 25% to 30% of CHD detected in the first monthafter birth may be critical. Current national estimatesof prenatal detection of CCHD are as low as 25%, withbest published US estimates at 50%. (14) There existsmuch promise for improved antenatal detection rates(15) with outflow tract views (91% sensitivity) comparedwith four chambered views (63% sensitivity). Other stud-ies, however, have found that the average time for trans-fer of this kind of evidence to widespread practice is 17years. (16)

Mortality and MorbidityHistorically, TGA and HLH are the leading causes ofdeath due to CCHD. It is known that the most severeof cardiac lesions (17) may be masked by an initiallywell-appearing infant with sudden onset of decompensa-tion a few hours to days after birth. Data from 898 infantsin California (18) who died of CCHD at age 1 to 364days included 152 infants with a missed CCHD diagnosisat a median age of 13.5 days; data were from 1984 to2004. More than one half of these infants died at homeor in a hospital emergency department. Although the an-nual number of these deaths declined in 1989 to 1999,the number remained unchanged from 2000 to 2004.

Most deaths were attributable to HLH and coarctationof the aorta. Up to 30 deaths per year in California wereattributable to missed or late diagnoses of CCHD, lead-ing to an overall incidence of missed CCHD of 1.7 per100,000 live births. Extrapolated to national live birthrates, the absence of universal oxygen saturation screen-ing could lead to at least 70 to 100 infant deaths due toundiagnosed CCHD in the United States annually. Sim-ilarly, data fromWisconsin suggest that death or readmis-sion events in neonates younger than 2 weeks old (N ¼24,684) occurred at a rate of 4 per 100,000 neonates(19) between 2002 and 2006. Obstruction to the leftside of the heart was again the most common cause.

In contrast to the US experience, a large Swedishstudy (7) found that there were no deaths (of 60 overall)from undiagnosed duct-dependent circulation in WestGotaland, where universal oxygen saturation screeningwas performed (N ¼ 39,821) compared with 5 deaths(of 100 overall) from other Swedish regions that didnot screen. Thus, estimate of deaths due to unrecognizedduct-dependent circulation from these other referring re-gions was 4.6 per 100,000 live births (95% confidence in-terval [CI]: 0.6–8.6). This study offers hope thatuniversal oxygen saturation screening might have the po-tential to reduce or near-eliminate deaths due to undiag-nosed CCHD.

Figure 2. Critical congenital heart disease (CCHD) screening legislation in different states. Reprinted from cchdscreeningmap.com.





It seems logical to propose that early detection wouldhelp to minimize morbidity and alleviate some of thestress in parental experiences. Delayed diagnosis worsensthe preoperative condition and postoperative outcome ofnewborns. (20) Prenatal diagnosis of TGA has been asso-ciated with decreased mortality and morbidity due to ear-lier diagnosis, treatment, and prevention of preoperativecomplications. (21) An unanswered question about uni-versal oxygen saturation screening is whether there ismore benefit compared to risk of iatrogenic harm accom-panied by early diagnosis. This question is true for prena-tally detected CHD as well. Although population-basedstudies have shown no benefit from the level of care at thebirthing hospital in those prenatally diagnosed withCHD, (22) there are practical considerations such aspostnatal transport. A single-center retrospective reviewof 202 infants receiving prostaglandin E1 suggests that“the risks of prophylactic intubation before the transportof otherwise stable infants on prostaglandin E1 must beweighed carefully against possible benefits.” (23)

Oxygen Saturation Screening Method:Evidence-Based Review

Screening Test CharacteristicsSensitivity of oxygen saturation screening wasmost recentlyestimated at 76.5% (95% CI: 67–83.5), with a specificity of99.9% (95% CI: 99.7–99.9). (24) The false-positive rate inthis recent meta-analysis was estimated at 0.14% (95% CI:0.06–0.33). The false-positive rate at 24 hours, as estimatedby using mathematical modeling (25) and in study condi-tions with screening at 24 hours, was as low or lower than0.01% (or 1:10,000). (26) In the United States, accuratedata on the false-negative rate will require linking newbornoxygen saturation screening data to existing agencies de-voted to improving CHD care, such as the Society of Tho-racic Surgeons’ national database and the ImprovingPediatric and Adult Congenital Treatment registry.

A recent meta-analysis of 13 eligible studies (of 552studies screened) included 229,491 infants who under-went universal oxygen saturation screening. (24) Thesestudies included 151 infants detected with CCHD (a prev-alence ofw1:1,500). When the three studies that involvedprenatally diagnosed lesions were excluded, the prevalenceof CCHD lesions detected with universal oxygen satura-tion screening was 114 of 190,867 infants screened(w1:1,700). Of these 114 infants, 88 were true-positives(77.2%) and 26 were false-negatives (22.8%). Thus, uni-versal oxygen saturation screening will pick up the majorityof CCHDwhile missing up to 1 in 4 to 5 infants who havethe disease. The supplementary appendix as provided by

the authors (24) is illuminating for the types of lesions de-tected (true-positives) or missed (false-negatives). The ma-jority of false-negatives included coarctation of the aorta orinterrupted aortic arch. Thus, coarctation and aortic archinterruption remains the Achilles’ heel of universal oxygensaturation screening for CCHD. Figure 3 illustrates thefrequency distribution of CCHD, although it oversimpli-fies the complexity of the CCHD lesions detected.

Methods of ScreeningDifferent strategies using preductal and postductal versuspostductal alone have used varying saturation thresholdsto detect CCHD. Postductal screening alone has beenrecommended by some authors (8) but not by others.(6)(7)(27) Some of the fundamental findings regardingdistribution of oxygen saturation come from studies thatmeasured oxygen saturation in the first 24 hours afterbirth. (28)(29)(30)(31)(32) Preductal oxygen saturationis higher than postductal in the first 4 hours after birth.(28)(29)(33) This difference has been shown to disap-pear by 24 hours of age. (26) Our experience fromscreening more than 12,000 newborns is that postductaloxygen saturation is greater than or equal to preductalsaturation in 78% of newborns screened between 18and 60 hours after birth. (33) Figure 4 summarizes datafrom three studies (28)(29)(33) that measured preductaland postductal oxygen saturation in the first 24 to 60hours after birth. It illustrates that the direction of the dif-ference between preductal and postductal saturationvaries with postnatal age. The clinical significance, phys-iological explanation, and validity of these findings bearfurther exploration. Better understanding of such physi-ological variability is essential for defining threshold cri-teria for universal oxygen saturation screening. Withwidespread adoption of universal screening, it is criticalto collect and evaluate oxygen saturation values in largepopulations to determine optimal screening methods.

Current evidence suggests that despite the universaloxygen saturation screening used, serial physical exami-nations remain crucial to narrowing the diagnostic gapand maximizing sensitivity in detecting CCHD. (6)(7)(8)(9)(25) A comprehensive clinical evaluation thatincludes environmental exposures, family history, andother anomalies is essential. While most CHDs are non-syndromic, isolated lesions, both syndromic and isolatedlesions may have an underlying molecular geneticdefect. Thus, early involvement of a dymorphologist/geneticist is essential to optimal management. Themolecular genetics for CHD are rapidly expanding andhave been extensively reviewed. (34)(35) The geneticbasis may have a major impact on long-term prognosis





and has implications for management of expectations byaffected families.

Screening Equipment and TechnologyCurrent universal oxygen saturation screening uses pulseoximetry to measure oxygen saturation. Knowledge ofthe type of oxygen saturation measured in the institu-tional screening device is essential. When defining thresh-old values for pulse oximetry screening for CCHD,consideration needs to be given to the type of oxygen sat-uration that is displayed: functional versus fractional. (25)Functional saturation measures the oxyhemoglobin andreduced hemoglobin alone, and fractional saturationmeasures the oxyhemoglobin and reduced hemoglobin,and approximates carboxyhemoglobin and methemoglo-bin. Hence, functional oxygen saturation tends to be1.6 to 2 points higher than the fractional oxygen satura-tion. (36) Motion artifact and different averaging timeof the pulse oximeter may also influence saturation

readings. Longer (8–10 seconds) time averaging is prefer-able in this context (7), whereas shorter (2 seconds) timeaveraging has traditionally been used in the delivery roomto obtain the quickest possible reading. There may be an-other 2% discrepancy between pulse oximetry saturationand arterial saturation by co-oximetry, with an increasingbias at lower oxygen saturation levels. (37) Understandingthese technical differences in the devices used is critical tostandardizing screening methods to detect CCHD.

Peripheral perfusion index (PPI) is an indirect, non-invasive measure of peripheral perfusion, expressingpulsatile signal as a percentage of nonpulsatile signal.(38) PPI is one potential opportunity for exploration inCCHD screening.Many institutions, including those pres-ently screening for CCHD, have monitors capable of thisfunction. De-Wahl Granelli established a nomogram from10,000 healthy newborns and found that five of nine leftheart obstructive lesions had abnormal PPIs less than 0.7.(39) Our nomogram in over 90 healthy newborns in the

Figure 3. Distribution of the 114 critical congenital heart disease (CCHD) lesions from 10 studies (excluding three studies that hadprenatally diagnosed CCHD) included in the meta-analysis by Thangaratinam et al. (24) Saturation screened, N[ 190,867; true-positives,n [ 88; false-negatives, n [ 26. AS[aortic stenosis, CoA/IAA[coarctation of the aorta/interrupted aortic arch, HLH[hypoplasticleft heart, PA[pulmonary atresia, PS[pulmonary stenosis, TA[tricuspid atresia, TAPVD[total anomalous pulmonary venous drainage,TGA[transposition of the great arteries.





first 45 minutes after birth (40) found that preductal PPIsare similar to those described by de-Wahl Granelli in in-fants between 1 and 120 hours after birth.

Our preliminary observation in two infants, one withcoarctation of the aorta and the other with interruptedaortic arch, showed pre- and postdifferential PPIs indepen-dent of oxygen saturations that led to the clinical diagnosisof aortic arch obstruction before echocardiography.

Future DirectionsUniversal oxygen saturation screening for CCHD in new-borns is gaining acceptance in much of the industrializedworld. The optimal timing, protocols, and methods con-tinue to evolve. In the context of newborn screening ingeneral, this remains the second point-of-care test afterthe newborn hearing screen, and thus it presents its ownunique challenges, different from traditional blood spotscreens. HLH and coarctation of the aorta/interruptedaortic arch continue to pose diagnostic challenges in theera of universal oxygen saturation screening. The formeris likely to be addressed by slow and steady improvementsin prenatal diagnosis, while this progress is much less likelywith the latter. Very preliminary data suggest that PPI mayprovide added opportunity for CCHD detection, particu-larly with aortic arch anomalies. It is incumbent on pro-grams using similar screening protocols to gather dataand publish their experiences to further inform potentialbetter practices for universal newborn oxygen saturationscreening. Manufacturers of product lines relevant to thispractice need to work closely with clinicians and health pol-icy advocates to streamline informatics, data collection, andreporting to further inform optimal public health policy inuniversal oxygen saturation screening for newborns.

ACKNOWLEDGEMENTS. This article is dedicated toJacqueline Anne Noonan, MD, and Abraham Rothman,

MD, who inspired our interest ininfant cardiology, and to Anne deWahl Granelli, PhD, who moti-vated our interest in universal oxy-gen saturation screening. Specialthanks to families everywhere withinfants affected by CHD, exemplifiedby Annamarie Saarinen and her teamof mothers in the United States whoadvocated passionately and most ef-fectively for the cause of universal ox-ygen saturation screening for infantswith CCHD. Their passion contin-ues to inspire us all.

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Figure 4. Mean difference between preductal and postductal oxygen saturation.

American Board of Pediatrics Neonatal-PerinatalContent Specifications

• Know how to interpret arterial blood gasmeasurements and noninvasive methodsfor estimating arterial oxygenation.

• Know the indications, limitations, andtechniques for newborn screening forgenetic disorders.

• Understand factors that affect the rationale for screening fora condition or disease (eg, prevalence, test accuracy, risk/benefit, disease burden, presence of a presymptomatic state).





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A 4-day-old female infant who was born at 39-weeks’-gestation comes to the emergency room with tachypnea,tachycardia, hepatomegaly, weak pulses, and very poor capillary refill time. Echocardiography shows hypoplasticleft ventricle. Prostaglandin infusion is initiated, but the infant has a cardiac arrest and does not respond toresuscitative measures.

1. Which of the following is correct about detection of critical congenital heart disease (CCHD)?

a. Data from several countries show that screening for CCHD using pulse oximetry is cost ineffective.b. Prenatal diagnosis results in 25%-50% detection of CCHD in the United States at the present time.c. Prenatal diagnosis results in 75%-90% detection of CCHD in the United States at the present time.d. The use of universal oxygen saturation screening as one of the newborn screening tests prior to discharge

from hospital is not yet supported by evidence.e. Transposed great arteries, hypoplastic left heart, total anomalous pulmonary venous drainage, coarctation

of the aorta, and interrupted aortic arch account for less than 30% of CCHD.

2. Advocacy for universal newborn screening for CCHD has its primary roots in which of the following?

a. Academic researchb. Governmentc. Industryd. Parent advocacye. The lay press

3. Historically, the leading causes of death from CCHD are:

a. Hypoplastic left heart and transposition of the great arteriesb. Interrupted aortic arch and total anomalous pulmonary venous returnc. Tricuspid atresia and coarctation of the aortad. Truncus arteriosus and interrupted aortic arche. Ventricular septal defect and Tetralogy of Fallot

4. According to the meta analysis cited by the authors, universal screening will:

a. Include interrupted aortic arch or coarctation of the aorta as the majority of false-negativesb. Not be cost effectivec. Not be useful in preterm infantsd. Only be effective if applied after 24 hours after birthe. Pick up the majority of CCHD while missing up to 1 in 10 babies with disease

5. In terms of the method of postnatal screening, which of the following is correct?

a. Direction of the difference in postductal and preductal saturations do not differ in terms of postnatal age.b. Functional saturation measures the oxyhemoglobin and reduced hemoglobin alone, and the fractional

saturation measures the oxyhemoglobin, reduced hemoglobin, and approximates carboxyhemoglobin andmethemoglobin. Hence, functional oxygen saturation tends to be 1.6 to 2 points higher than the fractionaloxygen saturation.

c. Peripheral perfusion index (PPI) relies on collection of a small quantity of venous and arterial blood.d. Postductal saturation measurements are clearly inferior to preductal.e. Preductal saturation measurements are considered standard of care for CCHD screening.





DOI: 10.1542/neo.13-12-e7242012;13;e724Neoreviews

Balaji Govindaswami, Priya Jegatheesan and Dongli SongOxygen Saturation Screening for Critical Congenital Heart Disease

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