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Published on: July 18, 2014
Which classification best predicts functional status in children with CHD?
Arthur Gavotto1,2, Ines Liard3, Helena Huguet4
1Department of Neonatal Medicine and Paediatric Intensive Care, Arnaud de Villeneuve Hospital, Montpellier University Hospital Centre, University of Montpellier, Montpellier, France.
Insights
The Uzark classification is the most reliable for predicting functional status and reproducibility in children with congenital heart disease (CHD). This simple tool aids in identifying at-risk children for early cardiovascular intervention.
Area of Science:
- Paediatric Cardiology
- Cardiovascular Research
- Congenital Heart Disease (CHD) Management
Background:
- Advances in CHD care shift focus from mortality to lifelong morbidity.
- Need for a simple, reproducible CHD classification to stratify disease severity.
- Current research prioritizes functional and patient-reported outcomes.
Purpose of the Study:
- To identify the optimal CHD classification for predicting functional status in children.
- To assess which classification best correlates with cardiopulmonary fitness.
Main Methods:
- Retrospective cohort study of 296 children with CHD.
- Involved baseline and final cardiopulmonary exercise tests over a mean follow-up of 4.1 years.
- Evaluated seven different CHD classifications.
Main Results:
- The Uzark et al. four-stage paediatric CHD classification best predicted functional status.
- Achieved an AUC of 0.62 for impaired cardiopulmonary fitness (VO2max or VAT < -1.64 Z-score).
- Demonstrated the highest inter-judge agreement (Kappa = 0.88).
Conclusions:
- The Uzark classification is a reliable tool for predicting functional status in pediatric CHD.
- It offers excellent reproducibility, crucial for clinical application.
- Facilitates early identification of children at risk for cardiopulmonary impairment, enabling timely interventions.
Background:
Medical advances in the management of CHD have shifted the focus from childhood mortality to life-long morbidity; therefore, clinical research in paediatric cardiology tends to replace mortality outcomes with functional and patient-reported outcomes. Despite these advances, the stratification of disease severity using a simple and reproducible CHD classification has not been established. The aim was to determine which classification best predicts functional status in children with CHD, in terms of cardiopulmonary fitness.
Method:
This retrospective cohort study was assessed from a cohort study of 296 children (mean age 11.3 ± 3.1 years, 129 female), who underwent a baseline and final cardiopulmonary exercise tests with a mean follow-up of 4.1 ± 1.6 years.
Results:
Seven CHD classifications were identified, assessing anatomical, physiological, therapeutic, or functional parameters in foetal, paediatric, or adult CHD populations. The four-stage paediatric CHD disease severity classification established by Uzark et al. best predicted functional status at the final cardiopulmonary exercise test assessment, with an area under the receiver operating characteristics curves of 0.62 (0.55-0.69) for impaired cardiopulmonary fitness (maximum oxygen uptake (VO2max) or ventilatory anaerobic threshold < -1.64 Z-score). The best inter-judge classification agreement was also observed for Uzark classification, with a Kappa coefficient of 0.88 (0.78-0.98).
Conclusion:
The CHD classification by Uzark is reliable in terms of functional status prediction and reproducibility in children with CHD. This simple classification may contribute to identifying children with CHD most at risk of cardiopulmonary fitness impairment and initiating early preventive cardiovascular interventions in paediatric cardiology.

