Special Issue "Neonatal Screening for Critical Congenital Heart Defects"

A special issue of International Journal of Neonatal Screening (ISSN 2409-515X).

Deadline for manuscript submissions: closed (15 January 2018).

Printed Edition Available!
A printed edition of this Special Issue is available here.

Special Issue Editor

Prof. Andrew Ewer
Website
Guest Editor
Professor of Neonatal Medicine, Institute of Metabolism and Systems Research, University of Birmingham, Honorary Consultant Neonatologist, Birmingham Women's Hospital

Special Issue Information

Dear Colleagues,

Critical congenital heart defects (CCHDs) are potentially life-threatening malformations that remain a significant cause of neonatal mortality and morbidity. Failure to diagnose these condition shortly after birth may result in acute cardiovascular collapse and death.

Identification of CCHDs by routine newborn clinical examination is routine in many countries, but consistently misses over a third, and, although antenatal ultrasound screening can be very effective in early diagnosis, the provision of ultrasound and accuracy of ultrasound screening is highly variable.

As most CCHDs present with mild cyanosis (hypoxaemia), which is frequently clinically undetectable, pulse oximetry is a rapid, simple, painless method of accurately identifying hypoxaemia, which has gained popularity as a screen for CCHD.

This Special Issue of the International Journal of Neonatal Screening, devoted to "Neonatal Screening for Critical Congenital Heart Defects (CCHD)", will consider the evidence for CCHD screening with pulse oximetry, the acceptability and cost-effectiveness of this intervention the additional non-cardiac conditions, which may also be identified, and the international experiences of introducing CCHD screening across the globe.

Prof. Andrew K Ewer
Guest Editor

Manuscript Submission Information

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Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Neonatal Screening is an international peer-reviewed open access quarterly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 500 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • CCHD screening in USA

  • CCHD screening in Scandinavia, Australia and China

  • CCHD screening in UK and Europe

  • Achieving consensus statement in South and Central America

  • Variations in screening algorithms—how to decide which to use?

  • The acceptability of Pulse Oximetry (PO) screening

  • The cost effectiveness of PO screening

  • Identification of non-cardiac disorders with PO screening

  • PO Screening for births at home

Published Papers (12 papers)

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Editorial

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Open AccessEditorial
Pulse Oximetry Screening for Critical Congenital Heart Defects: A Life-Saving Test for All Newborn Babies
Int. J. Neonatal Screen. 2019, 5(1), 14; https://doi.org/10.3390/ijns5010014 - 12 Feb 2019
Abstract
Congenital heart defects (CHD) are the commonest congenital malformations and remain a major cause of neonatal mortality and morbidity in the developed world [...] Full article

Research

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Open AccessArticle
Pulse Oximetry Values in Newborns with Critical Congenital Heart Disease upon ICU Admission at Altitude
Int. J. Neonatal Screen. 2018, 4(4), 30; https://doi.org/10.3390/ijns4040030 - 31 Oct 2018
Cited by 2
Abstract
Pulse oximetry screening for critical congenital heart disease (CCHD) has been recommended by the American Academy of Pediatrics (AAP). The objectives of this study are to describe saturation data, and to evaluate the effectiveness of AAP-recommended pulse oximetry screening guidelines applied retrospectively to [...] Read more.
Pulse oximetry screening for critical congenital heart disease (CCHD) has been recommended by the American Academy of Pediatrics (AAP). The objectives of this study are to describe saturation data, and to evaluate the effectiveness of AAP-recommended pulse oximetry screening guidelines applied retrospectively to a cohort of newborns with known CCHD at moderate altitude (5557 feet, Aurora, Colorado). Data related to seven critical congenital heart disease diagnoses were extracted from electronic health records (pulse oximetry, prostaglandin administration, and oxygen supplementation). Descriptive epidemiologic data were calculated. 158 subjects were included in this analysis; the AAP pulse oximetry screening protocol was applied to 149 subjects. Mean pre-ductal and post-ductal pulse oximetry values of the infants known to have CCHD at 24 h of life were 87.1% ± 7.2 and 87.8% ± 6.3, respectively. Infants treated with prostaglandins and oxygen had lower oximetry readings. The screening algorithm would have identified 80.5% of infants with known CCHDs (120/149 subjects). Additionally, sequential pulse oximetry screening based on the AAP-recommended protocol was able to identify a true positive screen capture rate of 80.5% at moderate altitude. Full article
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Open AccessArticle
Early Detection with Pulse Oximetry of Hypoxemic Neonatal Conditions. Development of the IX Clinical Consensus Statement of the Ibero-American Society of Neonatology (SIBEN)
Int. J. Neonatal Screen. 2018, 4(1), 10; https://doi.org/10.3390/ijns4010010 - 01 Mar 2018
Cited by 3
Abstract
This article reviews the development of the Ninth Clinical Consensus Statement by SIBEN (the Ibero-American of Neonatology) on “Early Detection with Pulse Oximetry (SpO2) of Hypoxemic Neonatal Conditions”. It describes the process of the consensus, and the conclusions and recommendations for [...] Read more.
This article reviews the development of the Ninth Clinical Consensus Statement by SIBEN (the Ibero-American of Neonatology) on “Early Detection with Pulse Oximetry (SpO2) of Hypoxemic Neonatal Conditions”. It describes the process of the consensus, and the conclusions and recommendations for screening newborns with pulse oximetry. Full article
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Open AccessArticle
Congenital Critical Heart Defect Screening in a Health Area of the Community of Valencia (Spain): A Prospective Observational Study
Int. J. Neonatal Screen. 2018, 4(1), 3; https://doi.org/10.3390/ijns4010003 - 05 Jan 2018
Cited by 1
Abstract
Despite the progress in the fetal echocardiographic detection of congenital critical heart defects and neonatal physical examination, a significant number of newborn infants are discharged and readmitted to the hospital in severe condition due to cardiac failure or collapse. The aim of this [...] Read more.
Despite the progress in the fetal echocardiographic detection of congenital critical heart defects and neonatal physical examination, a significant number of newborn infants are discharged and readmitted to the hospital in severe condition due to cardiac failure or collapse. The aim of this study was to assess the incidence of undetected critical congenital heart disease (CCHD) by a pulse oximetry-screening program in the maternity wards of hospitals with Perinatal Services in a specific geographic area. This is a prospective observational study performed in in the health area corresponding to the city of Valencia. Eligible infants were consecutively admitted newborn infants in the maternities of the participating hospitals with negative fetal echocardiography after normal physical examination in the delivery room. All patients were screened following a specific pulse oximetry protocol before discharge. A total of 8856 newborn infants were screened. A total of three babies presented with severe congenital cardiac malformation and two babies presented with early onset sepsis. Sensitivity was 100% and specificity was 99.97%, with a positive predictive value of 60% and negative predictive value of 100%. Pulse oximetry screening programs in the early neonatal period constitute a valuable tool to avoid inadvertent hospital discharge of severe cardiac malformations and the subsequent life-threatening complications derived. Full article
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Open AccessArticle
A Single-Extremity Staged Approach for Critical Congenital Heart Disease Screening: Results from Tennessee
Int. J. Neonatal Screen. 2017, 3(4), 31; https://doi.org/10.3390/ijns3040031 - 20 Nov 2017
Cited by 2
Abstract
Tennessee initiated single-extremity staged screening by pulse oximetry for undetected CCHD in 2012. The algorithm begins with a saturation reading in the foot and allows an automatic pass if the foot pulse oximetry is 97% or greater. This was based on the principle [...] Read more.
Tennessee initiated single-extremity staged screening by pulse oximetry for undetected CCHD in 2012. The algorithm begins with a saturation reading in the foot and allows an automatic pass if the foot pulse oximetry is 97% or greater. This was based on the principle that it is not possible to have a greater than 4% difference in the pulse oximetry between upper and lower extremities if the lower extremity is equal to or greater than 97%. This approach eliminates over 75,000 “unnecessary” pulse oximetry determinations in Tennessee each year without affecting the ability to detect CCHD before hospital discharge. Full article
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Open AccessArticle
Critical Congenital Heart Disease Screening Using Pulse Oximetry: Achieving a National Approach to Screening, Education and Implementation in the United States
Int. J. Neonatal Screen. 2017, 3(4), 28; https://doi.org/10.3390/ijns3040028 - 19 Oct 2017
Cited by 2
Abstract
A national approach to screening for critical congenital heart disease (CCHD) using pulse oximetry was undertaken in the United States. Following the scientific studies that laid the groundwork for the addition of CCHD screening to the U.S. Recommended Uniform Screening Panel (RUSP) and [...] Read more.
A national approach to screening for critical congenital heart disease (CCHD) using pulse oximetry was undertaken in the United States. Following the scientific studies that laid the groundwork for the addition of CCHD screening to the U.S. Recommended Uniform Screening Panel (RUSP) and endorsement by professional societies, advocates including physicians, nurses, parents, medical associations, and newborn screening interest groups were able to successfully pass laws requiring the screen on a state by state basis. Public health involvement and screening requirements vary by state. However, a common algorithm, education, and implementation strategies were shared nationally as well as CCHD toolkits to aid in the implementation in hospitals. Health Resources & Services Administration (HRSA) grants to pilot states encouraged the development of a public health infrastructure around screening, data collection, and quality measures. The formation of a CCHD NewSTEPs technical advisory work group provided a systematic way to tackle challenges and share best practices by hosting monthly meetings and webinars. CCHD screening is now required in 48 states, with over 98% of U.S. births being screened for CCHD using pulse oximetry. A standard protocol has been implemented in most states. While the challenges related to screening special populations and quantifying screening outcomes through the creation of a national data repository remain; universal implementation is nearly complete. Full article
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Review

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Open AccessReview
Pulse Oximetry Screening in Germany—Historical Aspects and Future Perspectives
Int. J. Neonatal Screen. 2018, 4(2), 15; https://doi.org/10.3390/ijns4020015 - 28 Apr 2018
Cited by 1
Abstract
In January 2017, pulse oximetry screening was legally implemented in routine neonatal care in Germany. The preceding developments, which were the prerequisite for this step, are described in the specific context of Germany’s health care system. Continued evaluation of the method is imperative [...] Read more.
In January 2017, pulse oximetry screening was legally implemented in routine neonatal care in Germany. The preceding developments, which were the prerequisite for this step, are described in the specific context of Germany’s health care system. Continued evaluation of the method is imperative and may lead to modifications in the screening protocol, ideally in accordance with the efforts in other countries. Full article
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Open AccessReview
Pulse Oximetry Screening Adapted to a System with Home Births: The Dutch Experience
Int. J. Neonatal Screen. 2018, 4(2), 11; https://doi.org/10.3390/ijns4020011 - 30 Mar 2018
Cited by 1
Abstract
Neonatal screening for critical congenital heart defects is proven to be safe, accurate, and cost-effective. The screening has been implemented in many countries across all continents in the world. However, screening for critical congenital heart defects after home births had not been studied [...] Read more.
Neonatal screening for critical congenital heart defects is proven to be safe, accurate, and cost-effective. The screening has been implemented in many countries across all continents in the world. However, screening for critical congenital heart defects after home births had not been studied widely yet. The Netherlands is known for its unique perinatal care system with a high rate of home births (18%) and early discharge after an uncomplicated delivery in hospital. We report a feasibility, accuracy, and acceptability study performed in the Dutch perinatal care system. Screening newborns for critical congenital heart defects using pulse oximetry is feasible after home births and early discharge, and acceptable to mothers. The accuracy of the test is comparable to other early-screening settings, with a moderate sensitivity and high specificity. Full article
Open AccessReview
Barriers to the Implementation of Newborn Pulse Oximetry Screening: A Different Perspective
Int. J. Neonatal Screen. 2018, 4(1), 4; https://doi.org/10.3390/ijns4010004 - 11 Jan 2018
Cited by 3
Abstract
Pulse oximetry screening of the well newborn to assist in the diagnosis of critical congenital heart disease (CCHD) is increasingly being adopted. There are advantages to diagnosing CCHD prior to collapse, particularly if this occurs outside of the hospital setting. The current recommended [...] Read more.
Pulse oximetry screening of the well newborn to assist in the diagnosis of critical congenital heart disease (CCHD) is increasingly being adopted. There are advantages to diagnosing CCHD prior to collapse, particularly if this occurs outside of the hospital setting. The current recommended approach links pulse oximetry screening with the assessment for CCHD. An alternative approach is to document the oxygen saturation as part of a routine set of vital signs in each newborn infant prior to discharge, delinking the measurement of oxygen saturation from assessment for CCHD. This approach, the way that many hospitals which contribute to the Australian New Zealand Neonatal Network (ANZNN) have introduced screening, has the potential benefits of decreasing parental anxiety and expectation, not requiring specific consent, changing the interpretation of false positives and therefore the timing of the test, and removing the pressure to perform an immediate echocardiogram if the test is positive. There are advantages of introducing a formal screening program, including the attainment of adequate funding and a universal approach, but the barriers noted above need to be dealt with and the process of acceptance by a national body as a screening test can take many years. Full article
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Open AccessReview
Cost and Cost-Effectiveness Assessments of Newborn Screening for Critical Congenital Heart Disease Using Pulse Oximetry: A Review
Int. J. Neonatal Screen. 2017, 3(4), 34; https://doi.org/10.3390/ijns3040034 - 14 Dec 2017
Cited by 8
Abstract
Screening newborns for critical congenital heart disease (CCHD) using pulse oximetry is recommended to allow for the prompt diagnosis and prevention of life-threatening crises. The present review summarizes and critiques six previously published estimates of the costs or cost-effectiveness of CCHD screening from [...] Read more.
Screening newborns for critical congenital heart disease (CCHD) using pulse oximetry is recommended to allow for the prompt diagnosis and prevention of life-threatening crises. The present review summarizes and critiques six previously published estimates of the costs or cost-effectiveness of CCHD screening from the United Kingdom, United States, and China. Several elements that affect CCHD screening costs were assessed in varying numbers of studies, including screening staff time, instrumentation, and consumables, as well as costs of diagnosis and treatment. A previous US study that used conservative assumptions suggested that CCHD screening is likely to be considered cost-effective from the healthcare sector perspective. Newly available estimates of avoided infant CCHD deaths in several US states that implemented mandatory CCHD screening policies during 2011–2013 suggest a substantially larger reduction in deaths than was projected in the previous US cost-effectiveness analysis. Taking into account these new estimates, we estimate that cost per life-year gained could be as low as USD 12,000. However, that estimate does not take into account future costs of health care and education for surviving children with CCHD nor the costs incurred by health departments to support and monitor CCHD screening policies and programs. Full article

Other

Open AccessReply
A Reply to Comment on Kluckow M. Barriers to the Implementation of Newborn Pulse Oximetry Screening: A Different Perspective. Int. J. Neonatal Screen. 2018, 4(1), 4
Int. J. Neonatal Screen. 2018, 4(2), 14; https://doi.org/10.3390/ijns4020014 - 17 Apr 2018
Cited by 1
Abstract
The commentary provided by Gentles et al. argues for the implementation of a universal pulse oximetry screening program, and I agree that, if it is possible, this is the optimum way to introduce this important health care measure for all of the reasons [...] Read more.
The commentary provided by Gentles et al. argues for the implementation of a universal pulse oximetry screening program, and I agree that, if it is possible, this is the optimum way to introduce this important health care measure for all of the reasons set out by the authors.[...] Full article
Open AccessComment
Comment on Kluckow M. Barriers to the Implementation of Newborn Pulse Oximetry Screening: A Different Perspective. Int. J. Neonatal Screen. 2018, 4(1), 4
Int. J. Neonatal Screen. 2018, 4(2), 13; https://doi.org/10.3390/ijns4020013 - 14 Apr 2018
Cited by 1
Abstract
We read the review article by Kluckow M (Barriers to the Implementation of Newborn Pulse Oximetry Screening[...] Full article
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