{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T16:19:26Z","timestamp":1785946766836,"version":"3.56.0"},"reference-count":27,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2024,3,12]],"date-time":"2024-03-12T00:00:00Z","timestamp":1710201600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"French Ministry of Health","award":["PHRC 2004"],"award-info":[{"award-number":["PHRC 2004"]}]},{"name":"French Ministry of Health","award":["2008"],"award-info":[{"award-number":["2008"]}]},{"name":"AREMCAR (Association pour la Recherche et l\u2019Etude des Maladies Cardiovasculaires) Association","award":["PHRC 2004"],"award-info":[{"award-number":["PHRC 2004"]}]},{"name":"AREMCAR (Association pour la Recherche et l\u2019Etude des Maladies Cardiovasculaires) Association","award":["2008"],"award-info":[{"award-number":["2008"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Backgroud: Congenital heart defects (CHDs) are the most frequent group of major congenital anomalies, accounting for almost 1% of all births. They comprise a very heterogeneous group of birth defects in terms of their severity, clinical management, epidemiology, and embryologic origins. Taking this heterogeneity into account is an important imperative to provide reliable prognostic information to patients and their caregivers, as well as to compare results between centers or to assess alternative diagnostic and treatment strategies. The Anatomic and Clinical Classification of CHD (ACC-CHD) aims to facilitate both the CHD coding process and data analysis in clinical and epidemiological studies. The objectives of the study were to (1) Describe the long-term childhood survival of newborns with CHD, and (2) Develop and validate predictive models of infant mortality based on the ACC-CHD. Methods: This study wasbased on data from a population-based, prospective cohort study: Epidemiological Study of Children with Congenital Heart Defects (EPICARD). The final study population comprised 1881 newborns with CHDs after excluding cases that were associated with chromosomal and other anomalies. Statistical analysis included non-parametric survival analysis and flexible parametric survival models. The predictive performance of models was assessed by Harrell\u2019s C index and the Royston\u2013Sauerbrei RD2, with internal validation by bootstrap. Results: The overall 8-year survival rate for newborns with isolated CHDs was 0.96 [0.93\u20130.95]. There was a substantial difference between the survival rate of the categories of ACC-CHD. The highest and lowest 8-year survival rates were 0.995 [0.989\u20130.997] and 0.34 [0.21\u20130.50] for \u201cinteratrial communication abnormalities and ventricular septal defects\u201d and \u201cfunctionally univentricular heart\u201d, respectively. Model discrimination, as measured by Harrell\u2019s C, was 87% and 89% for the model with ACC-CHD alone and the full model, which included other known predictors of infant mortality, respectively. The predictive performance, as measured by RD2, was 45% and 50% for the ACC-CHD alone and the full model. These measures were essentially the same after internal validation by bootstrap. Conclusions: The ACC-CHD classification provided the basis of a highly discriminant survival model with good predictive ability for the 8-year survival of newborns with CHDs. Prediction of individual outcomes remains an important clinical and statistical challenge.<\/jats:p>","DOI":"10.3390\/jcm13061623","type":"journal-article","created":{"date-parts":[[2024,3,12]],"date-time":"2024-03-12T12:14:40Z","timestamp":1710245680000},"page":"1623","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Predicting Long-Term Childhood Survival of Newborns with Congenital Heart Defects: A Population-Based, Prospective Cohort Study (EPICARD)"],"prefix":"10.3390","volume":"13","author":[{"given":"Makan","family":"Rahshenas","sequence":"first","affiliation":[{"name":"Centre of Research in Epidemiology and Statistics (Inserm 1153, CRESS), Universit\u00e9 Paris Cit\u00e9, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Lelong","sequence":"additional","affiliation":[{"name":"Centre of Research in Epidemiology and Statistics (Inserm 1153, CRESS), Universit\u00e9 Paris Cit\u00e9, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8722-5805","authenticated-orcid":false,"given":"Damien","family":"Bonnet","sequence":"additional","affiliation":[{"name":"M3C-Necker, National Reference Center for Complex Congenital Heart Diseases, APHP, Universit\u00e9 Paris Cit\u00e9, H\u00f4pital Necker-Enfants Malades, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucile","family":"Houyel","sequence":"additional","affiliation":[{"name":"M3C-Necker, National Reference Center for Complex Congenital Heart Diseases, APHP, Universit\u00e9 Paris Cit\u00e9, H\u00f4pital Necker-Enfants Malades, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Babak","family":"Choodari-Oskooei","sequence":"additional","affiliation":[{"name":"MRC Clinical Trials Unit at UCL, Institute of Clinical Trials and Methodology, University College London, London WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mithat","family":"Gonen","sequence":"additional","affiliation":[{"name":"Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francois","family":"Goffinet","sequence":"additional","affiliation":[{"name":"Centre of Research in Epidemiology and Statistics (Inserm 1153, CRESS), Universit\u00e9 Paris Cit\u00e9, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4031-4915","authenticated-orcid":false,"given":"Babak","family":"Khoshnood","sequence":"additional","affiliation":[{"name":"Centre of Research in Epidemiology and Statistics (Inserm 1153, CRESS), Universit\u00e9 Paris Cit\u00e9, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,3,12]]},"reference":[{"key":"ref_1","unstructured":"Zipes, D.P., Libby, P., Bonow, R.O., Mann, D.L., Tomaselli, G.F., and Braunwald, E. 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