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INTRODUCTION — Congenital heart disease (CHD) is the most common congenital disorder in newborns [1-3]. Critical CHD, defined as requiring surgery or catheter-based intervention in the first year of life (table 1), occurs in approximately 25 percent of those with CHD . Although many newborns with critical CHD are symptomatic and identified soon after birth, others are not diagnosed until after discharge from the birth hospitalization [5-8]. In infants with critical cardiac lesions, the risk of morbidity and mortality increases when there is a delay in diagnosis and timely referral to a tertiary center with expertise in treating these patients [9-11].
Newborn screening for critical CHD using pulse oximetry will be reviewed here. The presentation of critical CHD and management of specific cardiac conditions are discussed separately. (See "Identifying newborns with critical congenital heart disease" and "Cardiac causes of cyanosis in the newborn" and "Diagnosis and initial management of cyanotic heart disease in the newborn".)
DEFINITION AND TARGETED LESIONS — Critical CHD refers to lesions requiring surgery or catheter-based intervention in the first year of life (table 1). This category includes ductal-dependent and cyanotic lesions as well as less severe forms of CHD that are not dependent on the patent ductus arteriosus (PDA). Critical CHD accounts for approximately 25 percent of all CHD.
CHD lesions targeted by pulse oximetry screening include defects that typically a) require intervention in the first year of life and b) present with hypoxemia some or most of the time [12-14]. These include but are not limited to:
●Pulmonary atresia (PA) (figure 2) (see "Pulmonary atresia with intact ventricular septum (PA/IVS)")
●Tetralogy of Fallot (TOF) (figure 3) (see "Pathophysiology, clinical features, and diagnosis of tetralogy of Fallot")
●Transposition of the great arteries (TGA) (figure 5) (see "Pathophysiology, clinical manifestations, and diagnosis of D-transposition of the great arteries")
●Coarctation of the aorta (COA) (figure 8) (see "Clinical manifestations and diagnosis of coarctation of the aorta")
●Double-outlet right ventricle (DORV)
●Ebstein's anomaly (figure 9) (see "Clinical manifestations and diagnosis of Ebstein anomaly")
●Interrupted aortic arch (IAA) (figure 10)
PREVALENCE OF CRITICAL CHD — CHD is the most common congenital disorder in newborns with a birth prevalence of approximately 1 percent [1-3]. Up to 25 percent of infants with CHD have a "critical" defect. Numerous familial, maternal, and pregnancy-related factors have been reported to be associated with an increased risk of CHD (table 2). In addition, CHD is a common finding in a number of genetic syndromes (table 3). The epidemiology of critical CHD is discussed in detail separately. (See "Identifying newborns with critical congenital heart disease", section on 'Epidemiology'.)
CONSEQUENCES OF LATE DETECTION — Most infants with critical CHD are diagnosed either prenatally or upon clinical examination during the birth hospitalization. However, up to 30 percent of infants with critical CHD appear normal on routine examination and signs of critical CHD may not be apparent in the first days of life [15,16]. Cyanosis may not be clinically apparent in patients with mild desaturation (>80 percent saturation) or anemia . In darkly pigmented infants, cyanosis can be especially difficult to appreciate. (See "Identifying newborns with critical congenital heart disease", section on 'Postnatal diagnosis'.)
The timing of presentation varies with the underlying lesion and its dependence upon a patent ductus arteriosus (PDA). In patients with ductal-dependent lesions (table 1), closure of the PDA within the first few days of life can precipitate rapid clinical deterioration with potentially life-threatening consequences (ie, severe metabolic acidosis, seizures, cardiogenic shock, cardiac arrest, or end-organ injury) . Other patients may have lesions that are not dependent on the patency of the PDA (eg, total anomalous pulmonary venous return, truncus arteriosus), yet delayed diagnosis can similarly lead to poor outcomes. For infants with critical CHD who are not diagnosed during the birth hospitalization, the risk of mortality is as high as 30 percent [9,11,19].
In a population-based observational study of 3603 infants with critical CHD born in 1998 to 2007 (prior to institution of routine pulse oximetry screening) identified through a state Birth Defects Registry, about one-quarter of patients were not diagnosed during the birth hospitalization . In this group of late detected critical CHD (n = 825), 15 deaths were deemed to be potentially preventable (1.8 percent). In addition, adjusted multivariable analysis showed that infants with late detected critical CHD had a greater number of admissions, more hospitalized days, and higher inpatient costs than those diagnosed prenatally or during the birth hospitalization.
In a simulation model based upon estimates of birth prevalence, prenatal diagnosis rates, late detection rates, and sensitivity of pulse oximetry screening, one study estimated that 875 infants with critical CHD will be detected annually in the United States through newborn screening . An additional 880 false-negative screenings are expected.
BENEFITS OF SCREENING — The primary benefit of newborn screening for critical CHD screening with pulse oximetry is timely identification of infants with critical CHD prior to discharge from the birth hospitalization thereby minimizing the morbidity and mortality associated with delayed diagnosis.
In a large prospective study (2004 to 2007), universal screening with pulse oximetry was better at detecting infants with critical CHD compared with physical examination alone . In this cohort, there was a lower rate of missed diagnoses of critical CHD for infants in the region that screened with universal pulse oximetry compared with infants born in regions of the country where universal screening with pulse oximetry was not performed (8 versus 28 percent). In addition, no infant died from a ductal-dependent lesion in the region utilizing routine pulse oximetry versus five deaths from regions without routine oximetry.
In a report of one statewide screening program (2011 to 2012) that successfully screened 99 percent of 73,320 eligible infants born during the study period, 49 infants had a positive screen and underwent further diagnostic evaluation . Of the 49 infants with positive screens, 19 had additional signs and symptoms that would have triggered a diagnostic evaluation, whereas 30 underwent evaluation based solely upon the screening result. Of these, three had a previously undiagnosed critical CHD.
A secondary benefit of pulse oximetry screening is the identification of conditions other than critical CHD. Common noncardiac causes of hypoxemia that are identified through newborn pulse oximetry screening include sepsis, respiratory distress syndrome (RDS), persistent pulmonary hypertension of the newborn (PPHN), meconium aspiration, hypothermia, hemoglobinopathy, pneumonia, and pneumothorax [14,21,22]. Of the 30 infants identified in the statewide screening program mentioned above, 17 were found to have an important underlying medical condition other than critical CHD . (See "Overview of cyanosis in the newborn", section on 'Evaluation'.).
HARMS OF SCREENING — The benefits of screening in reducing mortality and morbidity and mortality associated with delayed diagnosis must be weighed against the downside of false positives. In the statewide screening program mentioned above, the false-positive rate was 0.06 percent . Infants with false-positive screening results undergo additional testing and/or transfer to centers with more advanced pediatric cardiac care. This additional testing has the potential to cause discomfort or harm to the newborn and to cause anxiety in the parents. It is important to recognize, however, that in many cases the evaluation results in identification of other causes of hypoxemia.
In a study evaluating the acceptability of pulse oximetry testing to the parents of newborns, parents were mostly satisfied with screening, perceived it as an important test, and would recommend it to others . Mothers given false-positive results were not found to be more anxious after screening than those given true negative results, although they were less satisfied with the test.
SCREENING RECOMMENDATIONS — Universal newborn screening for critical CHD is endorsed by the American Academy of Pediatrics (AAP), American Heart Association (AHA), and the American College of Cardiology (ACC) [24,25]. A 2015 report from the Centers for Disease Control and Prevention (CDC) found that in the United States, almost all states have legislation, regulations, or hospital guidelines in place supporting newborn screening for CHD . Screening programs are also in place in some European countries and other parts of the world [27-30].
Screening procedure — In the United States, the AAP guideline is the most commonly used algorithm for critical CHD screening (algorithm 1). Alternative algorithms include the New Jersey and the Tennessee algorithms.
Timing — Screening should be performed after 24 hours of life or as late as possible if early discharge is planned. Screening within the first 24 hours of life is not as specific as later screening because hypoxemia commonly occurs during the transition from intrauterine to extrauterine life conditions [31-34]. (See "Overview of neonatal respiratory distress: Disorders of transition".)
Technique — Screening should be performed by qualified and trained personnel . Oxygen saturation (SpO2) is measured in the right hand (preductal) and either foot (postductal) (algorithm 1). Screening at both locations can occur simultaneously or in direct sequence. Postductal measurement of SpO2 is important because defects with right-to-left shunting of desaturated blood through the ductus arteriosus will not be detected with only preductal measurement.
The screening should be performed using a motion-tolerant pulse oximeter. Either disposable or reusable probes can be used. Reusable probes reduce the cost of screening, but must be appropriately cleaned to minimize the risk of infection. Measurements should not be performed when the infant is crying or moving because this reduces the quality of the signal and the accuracy of the test [31,36]. In addition, pulse oximetry testing may fail to detect hypoxemia if there is interference from ambient light, partial probe detachment, electromagnetic interference, poor perfusion at the site of measurement, and/or hemoglobinopathy . (See "Pulse oximetry in adults".)
Criteria for positive screen — Criteria for a positive screen using the American Academy of Pediatrics (AAP) algorithm (algorithm 1) include any of the following:
●Oxygen saturation (SpO2) measurement <90 percent in either extremity
●SpO2 measurement 90 to 94 percent in both upper and lower extremities on three measurements, each separated by one hour
●SpO2 difference >3 percent between the upper and lower extremities on three measurements, each separated by one hour
A cutoff SpO2 value of <95 percent is used as it provides a sensitivity around 75 percent and specificity >99 percent [22,38]. In a 2012 meta-analysis of 13 studies (all of which used a cutoff SpO2 threshold of <95 percent), the sensitivity of pulse oximetry for detection of critical CHD was 76.5 percent (95% CI 67.7-83.5) and specificity was 99.9 percent (95% CI 99.7-99.9) .
The characteristics of the screening test will depend on which algorithm is being used . The New Jersey algorithm, which considers SpO2 <95 percent in either extremity on three measurements to be a positive screen, has a higher sensitivity but lower specificity than the AAP algorithm. The Tennessee algorithm, which initially tests only the lower extremity and considers an initial SpO2 of at least 97 percent to be a negative screen, has lower resource utilization than the AAP algorithm, but may have lower sensitivity.
As the SpO2 threshold is decreased, the sensitivity of pulse oximetry to detect critical CHD decreases and the specificity increases [31,40]. In a study that evaluated different criteria for an abnormal pulse oximetry test, lowering the SpO2 threshold from <95 to <90 percent resulted in greater specificity (88 versus 100 percent, respectively) but lower sensitivity (75 versus 53 percent, respectively) . Hence, using a lower SpO2 threshold decreases the number of false positives and thus may avoid unnecessary transfers, echocardiograms, and pediatric cardiology consultations. However, this comes at the cost of potentially missing some infants with critical CHD.
In a large multicenter prospective study of 122,738 newborn infants born between 2011 and 2012, the sensitivity of detecting critical CHD was greatest using the combination of pulse oximetry plus clinical assessment (93 percent) compared with either pulse oximetry alone (84 percent) or clinical assessment alone (77 percent) .
Assessment of infants with positive screens — A neonate with hypoxemia should be not discharged from the hospital without excluding potentially life-threatening conditions. Infants with positive screening results should undergo evaluation to identify the cause of hypoxemia. Determination of critical CHD as the cause should include high-quality echocardiography with interpretation by a clinician with expertise in the diagnosis of CHD. Patients should have access to these diagnostic services at the birth center, via telemedicine, or via short-distance transport. Each birthing institution should establish a protocol to ensure a timely evaluation for newborns with a positive screening test. However, evaluation of the baby with low oxygen saturation using other means (eg, chest radiograph, blood work) should not be delayed while awaiting an echocardiogram. (See "Identifying newborns with critical congenital heart disease", section on 'Diagnostic approach'.)
Common noncardiac causes of hypoxemia that may be identified through newborn pulse oximetry screening include sepsis, respiratory distress syndrome (RDS), persistent pulmonary hypertension of the newborn (PPHN), meconium aspiration, pneumonia, and pneumothorax [21,22]. In infants in whom an alternative cause (other than critical CHD) is identified and treated, an echocardiogram may not be needed if the hypoxemia resolves. (See "Overview of neonatal respiratory distress: Disorders of transition".)
If critical CHD is identified on echocardiography, urgent consultation with a pediatric cardiologist and/or transfer to a medical facility with pediatric cardiology expertise is warranted. Infants with ductal-dependent lesions are at increased risk for death and significant morbidity unless interventions are initiated to maintain patency of the ductus arteriosus, ensure adequate mixing of deoxygenated and oxygenated blood, and/or relieve obstructed blood flow. (See "Diagnosis and initial management of cyanotic heart disease in the newborn", section on 'Initial management'.)
NEGATIVE SCREEN — Infants with negative screening results who are clinically well without signs concerning possible CHD (eg, cardiac murmur, weak femoral pulses) do not require additional evaluation. However, it is important to recognize that infants with a negative screen may still have critical CHD because hypoxemia may not be present all of the time in some CHD lesions. It is estimated that universal newborn screening with pulse oximetry may miss as many cases of critical CHD as it detects . Screening with pulse oximetry cannot "rule out" the presence of a critical CHD . If there is clinical suspicion for critical CHD, additional evaluation should be pursued even in the setting of a normal pulse oximetry result.
High altitude — False-positive rates are higher in centers at high altitude . The pulse oximetry screening guidelines recommended by the American Academy of Pediatrics (AAP) are feasible up to an elevation of 2643 feet (806 meters) without any needed adjustments [44,45]. Criteria have not been validated for newborns cared for at centers at higher altitudes . A modified protocol has been proposed for testing at moderate altitude .
Out-of-hospital settings — For infants delivered out-of-hospital (ie, home births and birth centers), critical CHD screening using pulse oximetry can be performed outside of the hospital using portable pulse oximetry probes [48-52]. Care providers in these situations should have protocols in place to manage the infant who fails screening in accordance with published guidelines. (See "Planned home birth", section on 'Newborn care' and "Birth centers".)
Neonatal intensive care unit — There are no clear guidelines for performing screening in the neonatal intensive care unit (NICU) setting, yet these infants are similarly at risk for undetected critical CHD. Most neonates admitted to NICUs have pulse oximetry performed as part of their routine care; however, protocols used in newborn nurseries to identify critical CHD may not be appropriate for the NICU [53,54]. Premature infants may have a higher false-positive rate due to having lower saturations at baseline as compared with term newborns. False-negatives may also occur in this population because pulse oximetry may overestimate the arterial oxygen saturation (as compared with direct measurement via cooximetry) . In addition, pulse oximetry screening may be delayed because many neonates in the NICU setting require supplemental oxygen during the initial days of life . Unless mandated by state law, the child who has had a postnatal echocardiogram may not separately need pulse oximetry testing to be performed. Further work in this area is needed.
COST-EFFECTIVENESS — The cost of a universal critical CHD screening program includes the direct costs of pulse oximetry (equipment, training of personnel, staff time required for screening), and the costs of further evaluation and possible transfer of patients who fail the initial screening oximetry test . The cost and quality of follow-up vary depending on the accessibility and cost of pediatric cardiac subspecialty care and the need for transfer. In the United States, the additional cost for pulse oximetry universal screening has been estimated to be around $5 to 6 per newborn [56,57].
Critical CHD screening may result in reducing the costs associated with delayed diagnosis of critical CHD. As mentioned above, in a population-based observational study of 3603 infants with critical CHD, there were a greater number of admissions, more hospitalized days, and higher inpatient costs among infants with late-detected CHD (n = 825) compared with those who were diagnosed prenatally or during the birth hospitalization. The authors suggest that screening may lead to decreased costs, but further prospective studies are needed to confirm this.
In studies of the cost-effectiveness of pulse oximetry screening in newborns, the incremental cost of pulse oximetry plus clinical examination compared with examination alone have been estimated to be $20,000 to 35,000 per timely diagnosis [57,58]. The cost per life-year gained is estimated to be about $40,000 (2011 US dollars) . The greatest variation in costs between centers is in the use of equipment, with the use of reusable probes leading to considerable cost savings as compared with disposable probes .
IMPLEMENTATION STATUS IN THE UNITED STATES — Screening was added to the United States Recommended Uniform Screening Panel (RUSP) in 2011. Since then, almost all states in the United States now have legislation, regulations, or hospital guidelines in place supporting newborn screening for CHD . The Centers for Disease Control and Prevention (CDC) provide a map with updated information on legislation on newborn critical CHD screening for each state.
In 2012, an expert panel developed the following consensus recommendations for implementation of newborn pulse oximetry screening :
●Selection of screening equipment, which should be approved for hospital use in neonates by the US Food and Drug Administration (FDA), should also be tolerant of motion, use a neonatal sensor, and not require a fixation method. Of note, the FDA has not tested the performance of oximeters in critical CHD screening protocols.
●Establishment of reporting standards for each birth facility and state public health monitoring. This includes patient demographic information, results of oximetry screening, type of protocol and oximeter used, and the requirements for reporting by birth facilities to public health programs.
●Training of healthcare providers and education of families – Development of educational material for both staff and families.
●Ongoing assessment of the outcome of screening, particularly in the context of other screening efforts (eg, fetal ultrasound), noncardiac conditions, the quality of the equipment, cost of screening including educational efforts, and reimbursement.
Implementation of critical CHD screening varies by state. Clinicians should refer to the guidelines of their state to determine the appropriate algorithm and protocols for their state.
SUMMARY AND RECOMMENDATIONS
●Congenital heart disease (CHD) is the most common congenital disorder in newborns. Critical CHD, defined as requiring surgery or catheter-based intervention in the first year of life (table 1), occurs in approximately 25 percent of those with CHD. In infants with critical cardiac lesions, the risk of morbidity and mortality increases when there is a delay in diagnosis and timely referral to a tertiary center with expertise in treating these patients. (See 'Introduction' above.)
●The goal of critical CHD screening in newborns is to reduce mortality and morbidity associated with delayed diagnosis by identifying infants with critical CHD in a timely manner. There is evidence that universal screening with pulse oximetry improves the identification of patients with critical CHD compared with physical examination alone. (See 'Introduction' above and 'Prevalence of critical CHD' above.)
●CHD lesions targeted by pulse oximetry screening include defects which typically a) require intervention in the first year of life, and b) present with hypoxemia some or most of the time. (See 'Definition and targeted lesions' above.)
●We suggest that critical CHD screening using pulse oximetry (algorithm 1) be performed in all newborns after 24 hours of life or as late as possible if early discharge is planned (Grade 2C). Oxygen saturation (SpO2) should be measured in the right hand (preductal) and either foot (postductal). (See 'Screening recommendations' above.)
●Criteria for a positive screening test using the American Academy of Pediatrics (AAP) algorithm include any of the following (see 'Criteria for positive screen' above):
•SpO2 measurement <90 percent
•SpO2 measurement <95 percent in both upper and lower extremities on three measurements, each separated by one hour
•SpO2 difference >3 percent between the upper and lower extremities on three measurements, each separated by one hour
●Infants with positive screening results using pulse oximetry should undergo evaluation to identify the cause of hypoxemia. If critical CHD is identified on echocardiography, urgent consultation with a pediatric cardiologist and/or transfer to a medical facility with pediatric cardiology expertise is warranted. (See 'Assessment of infants with positive screens' above and "Diagnosis and initial management of cyanotic heart disease in the newborn", section on 'Initial management'.)
●Newborns with a negative screen may still have critical CHD because hypoxemia may not be present all of the time in some CHD lesions. If there is clinical suspicion for critical CHD, additional evaluation should be pursued even in the setting of a normal pulse oximetry result. (See 'Negative screen' above.)
●The screening procedure to detect critical CHD in newborns may require modification in the certain settings such as high altitude, out-of-hospital births (ie, home births and birth centers), and infants admitted to neonatal intensive care units (NICUs). (See 'Special settings' above.)
●In the United States, almost all states require mandatory newborn screening for critical CHD. Clinicians should refer to the guidelines of their state to determine the appropriate algorithm and protocols for their state. (See 'Implementation status in the United States' above.)
ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Carolyn A Altman, MD, who contributed to an earlier version of this topic review.
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