Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease

Background Impaired pulmonary function is an independent predictor of mortality in adult congenital heart disease (CHD), but has been scarcely studied in the paediatric CHD population. Aims To compare the pulmonary function of children with CHD to healthy controls, and evaluate its association with clinical outcomes, exercise capacity, and quality of life. Methods Cross-sectional multicentre study among 834 children (555 CHD and 279 control subjects) who underwent a complete spirometry and a cardiopulmonary exercise test (CPET). The 5th centile (Z-score = − 1.64) was used to define the lower limit of normal. The association of clinical and CPET variables with spirometry was studied using a multivariate analysis. Children and their parents filled in the Kidscreen health-related quality of life questionnaire. Results Forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) Z-scores values were lower in children with CHD than controls (− 0.4 ± 1.5 vs. 0.4 ± 1.3, P  Conclusion These results suggest that pulmonary function should be monitored early in life, from childhood, in the CHD population.