This multicentre prospective controlled study of a cohort of 249 children aged 5 to 7 years old assessed HRQoL of 124 children with CHD in comparison with 125 controls. This study also evaluated the relation between CHD severity and HRQoL and investigated some clinical determinants of HRQoL in young children with CHD.
This study highlighted that self-reported HRQoL in young children with CHD was good, with a total PedsQL score of 73.5 ± 1.2 (out of 100), and therefore similar to that of their contemporary peers recruited in schools (e.g. 72.8 ± 1.2). Such HRQoL levels probably reflect all tremendous medical advances in the screening and care of CHD in the last decades, in terms of prenatal diagnosis, non-invasive real-time imaging, neonatal cardiac surgery, paediatric intensive care, and cardiac catheterization. Indeed, in the current era, over 90% of children with a CHD are expected to survive more than 30 years after first cardiac surgery (1). In our study, 91% of children with CHD had no symptoms of heart failure (Ross class I). As a result, more attention has been recently given to patient related outcomes in the field of congenital cardiology, beyond the classical cardiac morbidity and mortality outcomes (32, 33). Yet, more consistent HRQoL data from controlled studies are necessary in young children with CHD. Nevertheless, our results are in line with the study from Pilla et al., reporting that psychosocial domain of self-reported HRQoL was similar between children with CHD and healthy children aged from 3 to 7 years old (34). Conversely, the single-centre controlled study from Uzark et al. concluded that HRQoL in children aged 2 to 17 y.o. in the USA was significantly impaired, however the 5–7 y.o. age group represented the smallest sample of the study (17). From a general perspective, French healthcare system, as in most European countries, provides full coverage from the social security for patients with CHD, which is not the case in the USA. Therefore, we should be aware that HRQoL in chronic diseases also reflects the quality of a health care system, beyond the medical care itself (35, 36). Moreover, modern medical care strategies in paediatric cardiology involve educational programs, shorter hospital stays, and home-based healthcare programs, which may contribute to achieve good HRQoL levels (19, 21, 37). Classically, patients with CHD are prone to cope with their health condition, and develop adaptive skills such as reponse shift (38) or sense of coherence (13, 39), which may result in good HRQoL levels, especially in the psychological domains (18). This adaptation has been particularly shown in non-progressive health conditions, such as cerebral palsy, or in children with a correctable congenital anomaly, such as CHD, but less so for children with a progressive disease (40). To our knolewdge, adaptation processes have not been fully investigated yet in young children with CHD, but our results may also suggest an early process of “resilience”.
When focusing on proxy reports, our study found that parents-reported HRQoL was significantly lower in the CHD group than in controls, with an approximate mean difference of 5 points (out of 100) in both mother and father reports. Our results are consistent with previous similar studies, but few HRQoL data involving both mothers and fathers of the same child have been reported in paediatric cardiology (15, 17, 18, 18, 21, 28, 41). Nevertheless, parents-reported HRQoL scores in our study ranged from 74 to 84 points (out of 100), which is good and remains higher than scores reported in previous studies (21). We may hypothesize that leaving home and attending kindergarten or the first years of elementary school may be a challenge for parents of children with a CHD (42). Indeed, our results found lower proxy-reported HRQoL levels in school and psychosocial domains. Parents of children with CHD classically overprotect them and may fear that their children will be stigmatized, bullied or put on the sideline at school (18, 32, 41, 43). In general, parents of children with chronic health diseases are prone to developp stress and anxiety, undermining the well-being or overall stability of their family, as well as their ability to cope (44–46). In paediatric cardiology, factors contributing to parental stress may involve a past trauma, as after prenatal diagnosis, an anguish of death during cardiac surgery, and an inability to empower their child (47). Therefore, it is necessary to integrate psychosocial support dedicated to such parents (17, 48) and develop multidisciplinary family-centred psychosocial care for patients with CHD, as recommendated by the AEPC psychosocial working group (49).
Our results also suggested that young children with the more severe CHD had significantly poorer HRQoL, as reported by themselves and their parents. In previous studies, disease severity has been found to impact the HRQoL of children with CHD (43). Indeed, HRQoL is significantly impaired in children with hypoplastic left heart syndrome, which is considered as the most severe CHD (50). In their study, Dempster et al. related HRQoL to the levels of impaired adaptive behavior, behavioral symptoms, and functional status (50). Similarly, HRQoL in children aged 2 to 18 y.o. after palliative single ventricle surgical repair, remains lower than in CHD eligible to biventricular surgical repair (44), such as tetralogy of Fallot (34). As in our previous studies in older children, this study confirms the negative impact of repeated invasive cardiac procedures (surgery or catheterization) on HRQoL (10, 51). Moreover, children with a CHD may suffer from an impaired physical capacity, which directly impacts the physical dimension of their HRQoL (6). Some children with CHD may develop an unpleasant feeling of dyspnoea, related to muscular deconditioning or restrictive lung function, which disrupts their physical well-being (22). Most of those children remain eligible to all sports, therefore healthcare providers, families and teachers should contribute to physical activity promotion in this population from an early age, to avoid the vicious deconditioning circle (37). Children with complex CHD may also present with an impaired HRQoL, as they are at higher risk of neurological or behavioural problems, including attention disorders, than children with a simple CHD (44).
In our study, HRQoL was predicted by the disease severity based on the infant heart failure classification from Ross et al (29). The Ross classification may therefore be more discriminating than the Bethesda classification (30) or the foetal prognosis classification from Davey et al (31). In the adult population, the strong correlation between NYHA functional status and HRQoL has been widely reported, in chronic heart failure (52), CHD (53) or pulmonary arterial hypertension (18). The Ross classification is mostly used in paediatric drug trials, therefore our results suggest using this scale in routine follow-up, to assess both functional status and HRQoL. We also suggest using self-reported HRQoL outcomes in paediatric cardiology trials.