PEDIATRICS Vol. 118 No. 4 October 2006, pp. e1250-e1256 (doi:10.1542/peds.2005-3061)
SPECIAL ARTICLE |
Report of the Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease
a Divisions of Pediatric Cardiology
b General Pediatrics
f Neonatology, Monroe Carell Jr Children's Hospital, Vanderbilt Medical Center, Nashville, Tennessee
c Tennessee Department of Health, Nashville, Tennessee
d Watson Clinic Center for Research, Lakeland, Florida
e Division of Pediatric Cardiology, University of Arkansas, Little Rock, Arkansas
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ABSTRACT |
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 TOP ABSTRACT DATA PRESENTED TO THE...CONSENSUS OF THE TASK...DISCUSSION AND FINAL CONCLUSIONSNOTEREFERENCES |
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A member of the Tennessee state legislature recently proposed a bill that would mandate all newborn infants to undergo pulse oximetry screening for the purpose of identifying those with critical structural heart disease before discharge home. The Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Defects was convened on September 29, 2005. This group reviewed the current medical literature on this topic, as well as data obtained from the Tennessee Department of Health, and debated the merits and potential detriments of a statewide screening program. The estimated incidence of critical congenital heart disease is 170 in 100000 live births, and of those, 60 in 100000 infants have ductal-dependent left-sided obstructive lesions with the potential of presentation by shock or death if the diagnosis is missed. Of the latter group, the diagnosis is missed in
9 in 100 000 by fetal ultrasound assessment and discharge examination and might be identified by a screening program. Identification of the missed diagnosis in these infants before discharge could spare many of them death or neurologic sequelae. Four major studies using pulse oximetry screening were analyzed, and when data were restricted to critical left-sided obstructive lesions, sensitivity values of 0% to 50% and false-positive rates of between 0.01% and 12% were found in asymptomatic populations. Because of this variability and other considerations, a meaningful cost/benefit analysis could not be performed. It was the consensus of the task force to provide a recommendation to the legislature that mandatory screening not be implemented at this time. In addition, we determined that a very large, prospective, perhaps multistate study is needed to define the sensitivity and false-positive rates of lower-limb pulse oximetry screening in the asymptomatic newborn population and that there needs to be continued partnering between the medical community, parents, and local, state, and national governments in decisions regarding mandated medical care.
Key Words: critical congenital heart disease • screening • pulse oximetry • hypoplastic left heart syndrome
Abbreviations: AAP—American Academy of Pediatrics • NBS—newborn screening • HLHS—hypoplastic left heart syndrome • AS—aortic stenosis • COA—coarctation of the aorta • IAA—interrupted aortic arch
On occasion, an infant will present shortly after discharge from the newborn nursery to the emergency department or pediatrician's office in extremis with acidosis and multiorgan failure secondary to a ductal-dependent left-sided obstructive cardiac lesion (critical aortic stenosis [AS], critical coarctation of the aorta [COA], interrupted aortic arch [IAA], or hypoplastic left heart syndrome [HLHS]). Many of these infants will suffer permanent neurologic sequelae. A small number of patients with these defects will also die before diagnosis. Parents often ask, "Why wasn't this heart defect found earlier?" or, "Would the outcome have been different had the diagnosis been made in a timely fashion?"
The director of a support group for parents of children with congenital heart disease recently approached one of the members of the Tennessee state legislature, who in turn submitted a bill that would require universal pulse oximetry screening of all newborn infants before hospital discharge, followed by a cardiology evaluation for those who screened positive. This bill has enjoyed substantial grassroots support and has progressed to the finance committees of both chambers of the Tennessee legislature. When asked to provide input, the medical community convened a task force that met on September 29, 2005, in Nashville. There were 33 participants including pediatric cardiologists, neonatologists, and pediatricians from across Tennessee and neighboring states. Also in attendance were research personnel from the Tennessee Department of Health, representatives from the Tennessee state legislature, the above-mentioned director of the support group, and a member of the Executive Committee of the Cardiology Section of the American Academy of Pediatrics (AAP).
The following is the report of the September 29th Tennessee Task Force on Screening Newborn Infants for Critical Congenital Heart Disease. The purposes of this report are to (1) summarize the data presented to the task force, (2) recount how these data were used to provide the recommendations offered to the legislature, (3) provide the framework for other states considering this screening program, and (4) encourage additional research in this area of public health.
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DATA PRESENTED TO THE TASK FORCE |
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From a modification of the criteria originally developed by Wilson and Junger in 1968,1 we considered a newborn screening (NBS) program to be effective if:
- the incidence of the condition in the newborn population is sufficient to warrant screening;
- therapy provided to the patient before onset of clinical manifestations results in an improved outcome.
- the screening test is able to identify the patient while he or she is yet asymptomatic;
- the test has acceptable sensitivity and false-positive rates; and
- the cost of screening (financial and nonfinancial) is reasonable in light of the limited health care resources available and the large number of additional health care needs currently competing for funds.
The task force presentations were organized to address these 5 points.
What Is the Incidence of Critical Congenital Heart Disease in the Newborn Population, and How Many Patients Currently Have the Condition Undiagnosed and Would Benefit From a Screening Program?
Pediatric cardiologists commonly define critical congenital heart defects as those that are either ductal dependent or require surgical or interventional attention in the first month of life. Table 1 lists potential critical congenital defects as either left-sided obstructive or cyanotic lesions and provides the national incidence data from the meta-analysis performed by Hoffman and Kaplan,2 the corresponding Tennessee data3 for each lesion, and an estimated proportion of each defect that is considered critical.
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Infants with cyanotic lesions typically present with severe cyanosis recognized very early, a murmur, or cyanosis that is well tolerated. It is rare for a neonate with a cyanotic cardiac lesion to present with acidosis and shock after having been sent home.4 Nonetheless, a late diagnosis is of no benefit to the infant and perhaps may be detrimental. The parents, at a minimum, may be angered by the delayed finding, and the unexpected presentation of such an infant may place an added time and resource stress on those who provide cardiovascular services. With an estimated incidence of 110 in 100000 live births, these considerations cannot be minimized.
If the primary goal of screening is to minimize the proportion of infants who die before a diagnosis is made or who present in cardiogenic shock, then ductal-dependent left-sided obstructive lesions remain as candidates for screening (specifically, HLHS, critical AS, critical COA, and IAA). Table 1 shows the total incidence of these lesions to be between 110 and 120 in 100000 live births. However, not all infants born with AS or COA will have a critical stenosis, leaving 60 in 100000 that are ductal dependent. Many of these patients, however, will be identified by either prenatal ultrasound or clinical evaluation before discharge home. We estimate that 8% of this value (5 in 100000 live-born infants) is a conservative value for missed diagnoses in patients who present alive.5 On the basis of studies that include community searches for autopsy records,4,6 an additional 5% to 8% (4 in 100000) of patients with one of these conditions will not receive a diagnosis before their death, yielding 9 in 100000 live-born infants as the incidence of missed ductal-dependent left-sided obstructive lesions.
To summarize, the total incidence of critical congenital heart disease is 170 in 100000 live births, and of those, an estimated 60 in 100000 have left-sided obstructive lesions, and 9 in 100000 of them have the condition undiagnosed at discharge home, one half of whom die before receiving the diagnosis. For the state of Tennessee with 80000 live births per year, these values correspond to 136 infants per year born with a critical heart defect, 48 with a critical left heart obstructive lesion and 7 with a missed critical left heart lesion. By comparison, the Tennessee metabolic screening program yearly identifies 50 confirmed cases of neonatal hypothyroidism, 26 cases of galactosemia, 5 of phenylketonuria, 5 of biotinidase deficiency, 0 of maple syrup urine disease, and 0 of homocystinuria.3 This comparison suggests that if an effective screening method exists, the incidence numbers would warrant implementation of such screening.
Would a Presentation Other Than Shock With Multiorgan Failure Benefit the Infant? (What Is the Relative Mortality and Morbidity of Patients With Left-Sided Obstructive Lesions With Varying Modes of Presentation?)
The diagnosis of these lesions before death is of vital importance. It is also worthwhile to examine the outcomes of those who present alive after discharge home without the diagnosis. Mahle et al5 analyzed the neurologic outcome of patients with HLHS on the basis of varying modes of presentation and found that of those who received the diagnosis prenatally, 15% exhibited perioperative seizures or coma, compared with 26% who received the diagnosis postnatally. Of those patients sent home without the diagnosis, 46% developed seizures or coma (William Mahle, MD, personal communication, 2005). It is well established that perioperative seizures and coma are strong predictors for neurodevelopmental outcome in this patient population.7–9 We can conclude, therefore, that an effective screening program would not only impact mortality but would prevent serious neurologic morbidity in this population.
Is There a Screening Test That Could Identify the Condition in These Infants While They Are Asymptomatic?
Infants with HLHS after delivery have systemic oxygen desaturation.10 Clinical cyanosis may not be evident, however, and these infants are initially asymptomatic with normal perfusion and respiratory rates. As the pulmonary vascular resistance decreases, oxygen saturation levels typically rise, and these neonates may develop tachypnea and diminished distal perfusion. This trend is compounded by constriction of the ductus arteriosus or failure of the right ventricle, culminating in acidosis and shock. Because of the high pulmonary/systemic flow ratios, oxygen saturation values may be in the normal range during these later stages. Patients with the other left-sided obstructive lesions may have normal upper-extremity oxygen saturation measurements after delivery; however, their lower limbs should show desaturation as a result of partial or complete perfusion via the ductus arteriosus.
Lower-extremity pulse oximetry would seem to be a useful tool in the identification of critical ductal-dependent left-sided obstructive lesions in the asymptomatic neonate. This screen may be falsely negative, however, once the pulmonary vascular resistance decreases or the ductus becomes restrictive and, thus, must be used within the context of the patient's clinical status. There is the potential that this screen could provide a false reassurance if not applied properly.
How Effective Is the Screening Program? (What Are the Sensitivity and False-positive Rates of Pulse Oximetry Screening?)
Four major references were reviewed to address this question. Hoke et al11 published a case-controlled study performed in the Baltimore, Maryland, area that screened 2972 well newborns and compared them to 32 neonatal cases referred for suspected congenital heart disease. Oximetry measurements were made in the upper and lower extremity at <6 hours of age, at 24 hours of age, and/or at discharge. An abnormal test result was defined as a saturation in the lower extremity 7% lower than the arm or lower-limb saturation <92%. The data showed a false-positive rate in the well-infant cohort of between 0.3% and 0.7%. When the criterion for an abnormal test was changed to <95% saturation, the false-positive rate at 24 hours was 12%. The sensitivity was felt to be high because the investigators "did not identify any children who presented with congenital heart disease after being discharged with a normal screening test." With regard to the 13 patients with confirmed left-sided obstructive lesions in the cases cohort, 85% of them had an abnormal oximetry test result according to the authors' cutoff value of 92%, and 92% tested positive when a 95% saturation value was used as the discriminatory point.
Koppel et al12 reported an analysis of 11281 asymptomatic infants screened by a single postductal pulse oximetry determination at the time of the state screen or at discharge. An abnormal test result was defined as a value 
95%. Three patients with congenital heart disease were identified; however, none had a critical left-sided obstructive lesion. One patient with COA who had passed the screen was readmitted later with heart failure. When applied to the left heart obstructive lesions, the sensitivity of this test was, therefore, 0%. There was only 1 false-positive result (0.01%) in this large cohort.
Richmond et al13 screened >5000 newborn infants at a mean of 11.7 hours, with a repeat determination in 1 to 2 hours if results were abnormal. A lower-limb assessment was performed, and an abnormal result was defined as a value of <95%. The results and analysis of this study were quite complex; however, it seems that the false-positive rate was 1%. Of the 6 neonates ultimately diagnosed with COA, only 3 were identified by the oximetry test. No cases of HLHS, critical AS, or IAA were noted in this study.
Reich et al14 compared 2114 patients in a pulse oximetry screening program with 2851 patients not in such a program. No cases of critical left-sided obstructive lesions were identified or missed by screening; therefore, a sensitivity rate could not be established. Screening did not generate any abnormal echocardiograms, implying a false-positive rate of 0%.
The issue of pulse oximetry–equipment reliability was also discussed to address the question: Why does pulse oximetry, which has been touted as the "fifth vital sign,"15 miss critical cardiac defects in infants that should, according to the infants' physiology, be identified? Oxygen saturation differs from other vital signs in that it can not be reliably seen, felt, or heard, it is generated entirely via a complex algorithm in a removed, technologically based measuring tool, and is subject to internal and external variables. False-positive alarms, in which the oximeter displays a low saturation value in the face of normoxemia, are extremely common in NICUs and PICUs and are in large part caused by low peripheral perfusion and motion artifact.16,17 Equally worrisome from a mass-screening standpoint are false-negative alarms, in which the oximeters miss true hypoxia. Known etiologies of this phenomenon include ambient light, partial probe detachment, and dyshemoglobinemias.18 An example of this dilemma was documented by McCrory et al,19 who described a critically ill, obviously cyanosed 1-day-old infant who had 6 sequentially low PaO2 values by arterial blood gas, while pulse oximeter measurements ranged between 95% and 100% despite using multiple body-site locations, different probes, and different machines. Barker's20 controlled comparison of 20 pulse oximeter models in adults showed substantial variability in equipment reliability, with models using newer technologies being superior to older forms when results were compared by receiver operator characteristic curves. Sensitivity values for detecting a saturation <90% ranged from 28% to 98%, with a median value of 68%. Bohnhorst et al21 found that 5.4% of hypoxic events in neonates were missed even by a newer generational oximeter. Hay et al22 have also shown substantial sensitivity differences for detecting hypoxia in the newer-generational models. Finally, one of us (J.D.R.) has presented additional data that have been submitted for publication elsewhere that bring into further question the reliability of this modality when used for mass screening. Thus, although pulse oximetry has been accepted by the medical community for monitoring patients in the critical care arena, the sensitivity of the equipment, particularly of older models, may be inadequate for screening purposes.
The results of a short feasibility study performed in the well-infant nursery at Vanderbilt Medical Center were also reviewed. The last 70 infants born had pulse oximetry performed at 24 hours of age or later. The screen was performed by a nurse, and screening time was 2 to 3 minutes. All infants had values of 
95%, and on follow-up, none later presented with a previously undiagnosed congenital heart defect.
In summary, the false-positive rates derived from published data of a screening program are quite variable, in part related to timing of the screen, with results in the literature varying from 0.01% to 12% when <95% was selected as an abnormal saturation value. Sensitivity values of 0%, 50%, and 92% were derived from the data, with the lower values obtained by screening asymptomatic populations. Very few cases of left-sided obstructive lesions were identified in the 4 large studies. There are substantial concerns about the reliability of current pulse oximetry equipment when used for mass screening, and it is conceivable that cases of critical congenital heart disease could be missed as a result of the false reassurance rendered by a "normal" screening result.
How Much Would the Screening Program Cost? Is the Financial Cost/Benefit Relationship Favorable? Are There Nonfinancial Cost/Benefit Considerations?
The financial costs of a screening program include:
- cost of screening per patient: equipment, personnel, training, local and statewide administration fees, and others (the purchase cost of a late-model pulse oximeter is approximately $900, excluding sensors);
- false-positive management costs: additional hospitalization time, potential transport costs, and cardiac evaluation fees (usually including an echocardiogram); and
- additional management costs resulting from patients who currently present dead before diagnosis.
The financial benefits are:
- shorter hospitalization times of those currently presenting in shock with multiorgan failure;
- rehabilitation cost savings for those whose presentation has resulted in neurologic morbidity; and
- long-term productivity gains from those who will be able to provide for themselves rather than requiring chronic care.
The Tennessee Task Force was unable to generate a reasonable financial cost/benefit relationship because of the lack of the following key data: (a) accurate false-positive and sensitivity rates of pulse oximetry screening; (b) an estimate of the number of nurseries currently equipped with an adequate pulse oximeter (with a back-up unit available in case of equipment failure); (c) administration costs for a statewide program; and (d) a reasonable estimate of costs and benefits resulting from the cohort of patients identified by the program (as above).
There are also nonfinancial costs of screening that may be important:
- emotional stress to parents of infants who undergo unnecessary cardiac evaluation with possible prolonged hospitalization and transport issues because of false-positive results;
- strains to transport infrastructure, NICUs, and cardiology services; and
- the impact of false-negative results (neurologic morbidity, death, emotional factors, and legal implications).
Balancing these nonfinancial costs are the benefits of a neurologically intact child, which are incalculable.
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CONSENSUS OF THE TASK FORCE |
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TOPABSTRACTDATA PRESENTED TO THE...CONSENSUS OF THE TASK...DISCUSSION AND FINAL CONCLUSIONSNOTEREFERENCES |
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- Universal screening for critical congenital heart disease by pulse oximetry should not be implemented at this time by a state legislated mandate or within the context of a state- or national-level recommendation. The primary concerns of the Tennessee Task Force, which are based on the current medical literature, were (a) the unclear false-positive rates of a screening program, (b) the questionable reliability of current oximeter technology in the asymptomatic population, and (c) the inability to generate a reasonable cost/benefit estimate. This consensus was nearly unanimous.
- Additional study is needed to accurately define the effectiveness of a screening program. Characteristics of such a study would include:
- a prospective, government funded, very large, perhaps multistate effort to definitively determine the sensitivity and false-positive rates of pulse oximetry testing (on the basis of a targeted incidence of 9 in 100000, we estimate that 160000 newborn infants would be required for an 80% chance of demonstrating a statistically significant benefit in a screened versus nonscreened population by a 1-sided test);
- use of standardized, highly reliable equipment with the capacity to correlate the oxygen saturation value with the quality of the reading and also the capability to store or print a permanent data record; and
- a value of <95% oxygen saturation in a lower limb would likely be selected as the criterion for results of a test performed at 24 hours of age to be considered abnormal; protocols would need to be established for the management of infants found to have a subnormal saturation, and they would need to address the possibility of noncardiac conditions as the explanation for the cyanosis (and if such a noncardiac cause is not found, there would need to be timely communication between the caregiver of the neonate and a cardiologist or neonatologist at a referral center so that additional evaluation and management could be arranged).
- a prospective, government funded, very large, perhaps multistate effort to definitively determine the sensitivity and false-positive rates of pulse oximetry testing (on the basis of a targeted incidence of 9 in 100000, we estimate that
- If such a study would show favorable sensitivity and false-positive rates, then the estimated incidence of critical congenital heart disease (170 in 100000), ductal-dependent left-sided obstructive lesions (60 in 100000), and missed ductal-dependent left-sided obstructive lesions (9 in 100000) would make it comparable to many current NBS efforts. The medical community would need to be educated about the limitations of this screen and the need to consider it as a part of the complete discharge neonatal assessment to avoid potential false-negative scenarios.
- There needs to be continued partnering between the medical community, parents, and local, state, and national governments in decisions regarding mandated medical care. This partnering is also imperative for the development of the necessary infrastructure to support NBS advances. In this era of limited health care resources, the financial implications of any change cannot be dismissed without a thorough analysis.
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DISCUSSION AND FINAL CONCLUSIONS |
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TOPABSTRACTDATA PRESENTED TO THE...CONSENSUS OF THE TASK...DISCUSSION AND FINAL CONCLUSIONSNOTEREFERENCES |
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Since the Guthrie technique for phenylketonuria was developed in the early 1960s,23 NBS programs have evolved, and the number of potential disorders for which screening is now possible has ballooned to >60 as a result of advances in both our understanding of their pathophysiology and the technologies available for their detection.24 Each state has developed its own unique screening program, with only 3 diseases assessed by all 50. This variability led the AAP to issue its 2000 Newborn Screening Task Force report stating "the need for a uniform national policy on the selection of newborn screening tests, as well as common guidelines for newborn screening systems [to ensure] equitable access to newborn screening, and its potential benefits."25 In an effort to address this need for uniformity, the American College of Medical Genetics Newborn Screening Expert Group, using expert consensus and literature review, identified 84 potential metabolic, infectious, hematologic, endocrinologic, and miscellaneous conditions for scrutiny.24 Of those, 29 were thought to represent a "core panel," and another 25 were placed into the "secondary-target" category. Critical congenital heart lesions were not among the conditions considered for ranking, likely reflecting their emphasis on screening programs that use blood-sample analyses. Because of the incidence and impact of critical congenital heart disease, we would be interested in that expert group including cardiac lesions in their next determination.
A recent addition to the palette of diseases that has been integrated into NBS is hearing loss.26 In 2000, screening was recommended by the Joint Committee on Infant Hearing, of which the AAP was a part. The use of non–blood spot technology presented a novel variation from the routine screening performed and required birthing facilities to purchase separate equipment, train staff, be responsible for performing the actual test, and manage the identified patient after the test. Screening for congenital heart lesions by pulse oximetry poses similar obstacles to implementation.
In comparison to hearing screening, the implications of a false-positive or false-negative result are considerably more profound for left-sided obstructive lesions, with neonatal death being the ultimate consequence. As a result, there are many practical medical, financial, and legal questions that arise should a screening program for critical congenital heart disease be implemented. Some are more readily answerable than others: What is a provider to do with an abnormal test result? How should the evaluation paradigm be formatted to ensure that noncardiac causes of cyanosis are diagnosed in an appropriate fashion? Should a prostaglandin infusion be initiated before definitive echocardiographic diagnosis? How should these patients be managed if cardiology consultation or pediatric echocardiography is not readily available? What transport arrangements are acceptable in the situation where the infant is asymptomatic? Who pays for the screening program? Who pays for the management and evaluation of the false-positive cases? What are the legal implications of a false-negative test result? Who is liable if an infant is sent home with a normal "heart test" only to present later in extremis secondary to a ductal-dependent lesion? If oximetry testing were recommended by the medical community but not mandated by law, would birthing facilities be exposed to liability if they do not perform the new "standard of care?"
The AAP Newborn Screening Task Force report defines NBS as "more than testing—it should always be part of a system that includes screening tests, follow-up, diagnosis, treatment, and evaluation as necessary."25 NBS requires a coordinated effort between medical, social, financial, and policy stakeholders to develop a system that encompasses medical care, community education, cost analysis, and legislative oversight. Taken in this context, the variability of critical congenital heart disease and the questionable accuracy of pulse oximetry testing suggest that it is not a good candidate for routine neonatal testing at this time. Perhaps as more data becomes available to define the validity of the test and pulse oximetry equipment is modified for the purpose of mass determinations, screening for these terrible but often surgically remediable lesions will become worthwhile.
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NOTE |
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TOPABSTRACTDATA PRESENTED TO THE...CONSENSUS OF THE TASK...DISCUSSION AND FINAL CONCLUSIONSNOTEREFERENCES |
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Since the Tennessee Task Force met, the Health Technology Assessment Program, an advisory arm of the National Health Service in the United Kingdom, has published an analysis of NBS for congenital heart defects.27 Although they note that pulse oximetry is "promising" for this purpose, they also recommend that additional research be undertaken to evaluate the performance of this method.
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ACKNOWLEDGMENTS |
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We are grateful to Dr Angela Liske for editorial assistance.
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FOOTNOTES |
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Accepted Apr 19, 2006.
Address correspondence to Michael R. Liske, MD, Vanderbilt Children's Hospital, Division of Pediatric Cardiology, 2200 Children's Way, Suite 5230, Nashville, TN 37232-9119. E-mail: michael.liske{at}vanderbilt.edu
The authors have indicated they have no financial relationships relevant to this article to disclose.
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PEDIATRICS (ISSN 1098-4275). ©2006 by the American Academy of Pediatrics
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