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Predicting Long-Term Childhood Survival of Newborns with Congenital Heart Defects: A Population-Based, Prospective
Makan Rahshenas1, Nathalie Lelong1, Damien Bonnet2
1Centre of Research in Epidemiology and Statistics (Inserm 1153, CRESS), Université Paris Cité, 75006 Paris, France.
Insights
The Anatomic and Clinical Classification of Congenital Heart Defects (ACC-CHD) effectively predicts survival in newborns. This classification system aids in understanding long-term outcomes for children with congenital heart defects.
Area of Science:
- Pediatrics
- Cardiology
- Public Health
Background:
- Congenital heart defects (CHDs) are the most common major congenital anomalies, affecting nearly 1% of births.
- CHDs present significant heterogeneity in severity, management, and etiology, necessitating precise classification for accurate prognostication and research.
- The Anatomic and Clinical Classification of CHD (ACC-CHD) was developed to standardize CHD coding and data analysis.
Purpose of the Study:
- To describe the long-term childhood survival rates of newborns diagnosed with CHDs.
- To develop and validate predictive models for infant mortality utilizing the ACC-CHD classification system.
Main Methods:
- A prospective, population-based cohort study (EPICARD) included 1881 newborns with CHDs, excluding those with chromosomal or other anomalies.
- Statistical analyses employed non-parametric and flexible parametric survival models.
- Model performance was evaluated using Harrell's C index and Royston-Sauerbrei RD2, with internal validation via bootstrap.
Main Results:
- The overall 8-year survival rate for isolated CHDs was 0.96 (95% CI [0.93-0.95]).
- Significant survival differences were observed across ACC-CHD categories, with rates ranging from 0.995 for interatrial/ventricular septal defects to 0.34 for functionally univentricular hearts.
- The ACC-CHD classification demonstrated strong predictive ability, with Harrell's C at 87% (alone) and 89% (full model), and RD2 at 45% (alone) and 50% (full model).
Conclusions:
- The ACC-CHD classification serves as a robust foundation for a highly discriminant survival model for newborns with CHDs.
- The classification system exhibits good predictive accuracy for 8-year survival.
- Individual outcome prediction for CHDs remains a complex clinical and statistical challenge.
Abstract:
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's C index and the Royston-Sauerbrei RD2, with internal validation by bootstrap. Results: The overall 8-year survival rate for newborns with isolated CHDs was 0.96 [0.93-0.95]. 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 [0.989-0.997] and 0.34 [0.21-0.50] for "interatrial communication abnormalities and ventricular septal defects" and "functionally univentricular heart", respectively. Model discrimination, as measured by Harrell's 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.
