According to Varni et al. [24] and Huang et al. [39], who identified cut-offs for patient-reported and parent-reported generic QoL, the majority of our patients (75%) and their parents (mothers: 61–80%; fathers: 74–84%) reported high levels of generic QoL, above the clinical cut-offs. These QoL levels may reflect the medical advances in screening and care for CHD in recent decades [40, 41]. Indeed, to date, over 90% of children with a CHD are expected to survive more than 30 years after the first cardiac surgery [3, 10, 40,41,42]. In addition to this, our patients and their parents are involved in a multidisciplinary standardized follow-up care. They are submitted to a comprehensive evaluation, including psychological consultations (providing support for patients and caregivers) that may contribute to enhancing their perceived QoL. According to these results, the literature has also shown that patients with congenital CHD are prone to cope with their health condition and develop adaptive skills earlier than their peers [21, 43, 44]. Indeed, according to previous studies, they might have learned early in their life how to develop a strong ‘‘sense of coherence’’ (understandability of the internal and external stimuli received during childhood; perception of the resources available to deal with stressful situations, such as living with heart disease; the ability of the individual to believe that his life has meaning, find motivation and assume the control over his life) and select the right coping strategies that may result in good QoL levels, especially in the psychological domains [11, 44]. Several studies, worldwide, have found good levels of QoL in paediatric CHD patients [4, 10, 11, 20, 22, 42, 45]. For example, Abassi et al. [11] examined a sample of 5- to 7-year-old French children and their parents; the mean total generic PedsQL scores from patient reports, mother reports, and father reports were 73.5, 76.1 and 79.2, respectively. Reiner et al. [22], in a sample of CHD patients from Germany aged 7–17 years, found a total generic QoL mean score of 78.6, and Moreno-Medina et al. [10] found at baseline a total score of 74.4 for the generic module and 79.6 for the cardiac module from a sample of 5–18-year-old CHD patients from Colombia; parents had a total score of 68.4 for the generic module and 73.6 for the cardiac module.
Nevertheless, the majority of these studies have mainly investigated the QoL of CHD patients through the PedsQL generic module, not including the cardiac-specific module. Moreover, they have focused only on specific age ranges and did not include both mothers and fathers in proxy-report evaluations. For these reasons, it is difficult to compare our results with this previous heterogeneous body of literature; moreover, no previous studies have been published on the QoL of Italian pediatric CHD patients. Our study has provided a comprehensive description of both generic and condition-specific QoL among Italian pediatric CHD patients at different ages (from 2 to 18 years), considering clinical factors such as CHD diagnosis and including both maternal and paternal reports. Our results can usefully inform clinical practice and guide clinicians’ efforts aimed at prevention and intervention.
Differences in generic and cardiac-specific QoL related to age and clinical factors
With regard to the QoL levels of our patients in relation to their age and clinical factors, our results showed several differences between ages, CHD diagnoses, and between patients who underwent surgical interventions and/or are taking cardiac medications at present, highlighting the importance of considering sociodemographic and clinical factors when assessing the QoL of CHD patients. The main results showed that children aged 5–7 years reported the lowest generic- and cardiac-specific total QoL levels. During the transition from home to preschool or primary school, children should acquire skills that promote emotion recognition and regulation, empathy for others, problem-solving, and positive social interactions [46]. Furthermore, they are expected to show increasing independence and separation from their caregivers as they adjust and hone their social interactions with peers and adults outside the home [47]. As a result, this period may represent new challenges for young children with CHD and their families [11]. Children with CHD are frequently absent from school and their parents may overprotect them because they may fear that their children will be stigmatized or bullied at school [11, 27, 44, 46]. Therefore, leaving home and attending kindergarten or the first years of elementary school may be a challenge for children with CHD and their parents [11, 48]. In contrast, adolescents and their parents reported a worse perceived physical appearance (e.g., I feel I am not good looking, I don’t like other people to see my scars, I am embarrassed when others see my body) than children in the other groups. Adolescents with CHD not only have to face the challenges of living with a chronic condition but also need to accomplish the normative tasks of their developmental period, such as their body image development, which contributes in a fundamental way to the identity of adolescents. Due to their susceptibility to external influence and cultural values [49], adolescents are at high risk of developing a negative body image, which, in turn, can negatively influence their QoL [50]. For these reasons, we may speculate that adolescents with CHD are at higher risk of developing a negative body image than their healthy peers due to physical limitations and scars related to surgical intervention and to their chronic illness in general. In addition, mothers and fathers of our adolescent patients reported lower perceived treatment adherence. During adolescence, the responsibility for managing a chronic disease should gradually shift from the parents to the adolescents themselves [51]; however, the results from previous studies showed that adherence diminished when management of the medical treatment shifts from the parents to the adolescent. According to the literature, several factors such as deductive thinking, independence, enhancement of self-efficacy and parental involvement could impact adherence to treatment in adolescence [51]. However, to date, no studies have previously identified potential risk and protective factors for CHD Italian paediatric patients’ adherence to treatment. Therefore, future studies are needed to corroborate our findings.
Our results also showed that significant differences emerged between UVH patients and other patients when clinical factors were considered. Previous studies have shown a correlation between QoL and the severity of CHD. For example, Marino and colleagues [26], using the Pediatric Cardiac Quality of Life Inventory, found that different CHD population groups obtained a particular QoL score range based on the disease severity and the medical, catheter-based, and surgical therapy required. This correlation between QoL and the severity of CHD seems to be particularly true with regard to the physical dimension of QoL [11, 18, 19, 40, 48, 52, 53]. Indeed, children with a CHD may suffer from an impaired physical capacity (e.g., unpleasant feeling of dyspnoea, related to muscular deconditioning or restrictive lung function [54]); thus, according to these studies, we may speculate that UVH patients reported lower levels of QoL on physical dimensions due to their clinical history (they have undergone more operations during their life—at least three surgical steps—and are forced to take drugs lifetime). In addition to this, results from our study showed that patients who both are taking medications at present and underwent surgery, and their parents, perceived more cardiac symptoms; in contrast, patients who had been operated on only and their parents reported a worse perceived physical appearance. Even in this case, these results are in line with the literature that showed that undergoing surgery and/or tanking cardiac medications resulted in a lower level of QoL in the physical dimensions [55]. Moreover, our study revealed that patients who underwent surgery reported a lower total score on the generic PedsQL and lowered school functioning levels (e.g., I miss school because of not feeling well, I miss school to go to the doctor or hospital) than children who take medications only. These results should be interpreted with caution due to the small sample of the Medications-only group (n = 24). However, we may speculate that CHD patients who underwent surgery may have reported lower levels of school functioning because of the frequent absence from school related to medical examinations and/or hospitalizations.
It is important to mention that the results from our study showed that beyond these differences based on the CHD diagnosis, interventions and medications, our patients and their parents reported high levels of QoL, above clinical cut-offs [24, 39]. These results are in line with other studies that revealed how the severity of CHD, in terms of the clinical symptoms and the number of surgical procedures or health interventions, seemed to have a marginal effect on CHD patients’ QoL [56], and that paediatric CHD patients reported lower levels of QoL in the physical domain regardless of the surgery intervention [12]. According to the literature, the combination of medical and social stress seems to have the strongest negative impact on the quality of life in diseased children or adolescents, regardless of its severity [57]. Therefore, we may speculate that a family with sufficient resources, such as higher levels of social support [58], can cope better and restrain the adverse effects on the quality of life, even with a severe disease condition.
Agreement and directional disagreement between child/adolescent and parent reports on generic and cardiac-specific QoL
Pediatric patient self-report should be considered the standard for measuring patients' QoL. However, there are situations in which parent proxy-reports are needed [24, 25, 59]. Moreover, according to the literature, parental reports could provide important complementary information about children’s QoL [29]. For example, previous studies have shown that parents can report physical and medical aspects in more detail, especially compared with very young children. In contrast, children and adolescents can report detailed information about social exclusion or inclusion [36]. Previous studies reported mixed results, showing agreement between both parental and patient reports and parental overrating of patients’ problems [10]. Overall, our results showed higher percentages of agreement than of disagreement both on generic and condition-specific QoL between mothers and fathers and between parents and children/adolescents. According to Patel and colleagues, a possible explanation for agreement between parental and patient reports could reflect medical improvements in the screening and care of CHD in recent decades. As surgical techniques improve, parents observe fewer differences between their children with CHD and their healthy peers (especially in the physical domains), leading to higher assessments of their quality of life [23] and reducing the degree of disagreement in self-report questionnaires.
Nevertheless, our results revealed a high disagreement rate between parents and patients aged between 5 and 7 years. Specifically, we observed that mothers underrated their children’s anxiety symptoms related to treatment for condition-specific QoL. Moreover, children reported higher scores on cognitive problems than both their mothers and their fathers, and a low agreement rate (23%) was found between patients and fathers in communicative skills. With regard to generic QoL both mothers and fathers showed a trend of underrating their 5–7-years-old children’s problems with generic physical, emotional, social and school functioning. According to previous studies, psychosocial domains related to thoughts and feelings (e.g., anxiety symptoms) are often more difficult for parents to discern through observation, increasing the potential for discrepancies [15, 24, 27, 28, 60]. Moreover, as discussed above, chronic illness, such as CHD, could impact the development of socioemotional, problem-solving and interaction skills during this age range, thereby limiting the ability of young children to identify and express their concerns to parents [57]. However, according to previous studies on CHD patients, the differences in QoL perception might also be due to the different expectations regarding the patients’ social, cognitive and intellectual abilities between parents and the patients themselves [10, 11], especially in the case in which parents underrate their children’s problems. Our results, especially on generic QoL, are in line with those of Uzark and colleagues [15] who showed that children with mild cardiovascular disease (CVD) reported poorer psychosocial QoL than those perceived by their parents and with other studies [56]. According to Hemingsson et al. [61], these results may reflect that in domains where children report more difficulties or lower QoL than parents, children’s opinions may not always be elicited in pediatric settings. Instead, their parents are the primary informants.
For the first time, our study has explored agreement and directional disagreement between Italian CHD pediatric patients and their parents, both mothers and fathers, identifying possible conditions under which parent proxy-report instruments achieve better agreement with child self-report instruments that could facilitate clinicians’ interpretation of QoL outcomes. Including caregivers’ assessment can be an essential aspect in evaluating a child with CHD from the perspective of the use of healthcare facilities and the quality of communication between patients and their caregivers [7, 10]. In addition, our study highlighted the importance of including both mothers and fathers in the QoL proxy-report evaluations because they could perceive their children’s QoL dimensions differently. In our sample of children aged 5–7 years, even though mothers and fathers both generally agreed with their children, mothers showed higher disagreement rates on anxiety symptoms while fathers showed higher disagreement rates on communicative skills. According to the literature, mothers are most often the primary caregivers, and they report higher distress and lower QoL, factors that can influence proxy ratings compared to fathers [62]. However, to date, no studies have previously explored agreement and directional disagreement in Italian CHD patients with both fathers and mothers. Thus, future studies are needed to corroborate our findings.
The present study results could increase the use of patient-related outcomes in clinical practice and guide clinicians’ efforts aimed at prevention and intervention. Moreover, they could enable clinicians and researchers to broaden their understanding of CHD pediatric patients’ well-being [31] by providing a comprehensive understanding of CHD pediatric patients’ QoL due to the inclusion of both patients’ and parents’ perspectives during clinical assessments.
Limitations
Our study is not without limitations. First, it does not include a control group of healthy children. In future studies a multicenter approach should be taken into account in order to corroborate our findings. Second, it does not evaluate the role of potential risk and protective factors related to the QoL of CHD pediatric patients. Future studies should perform multivariate analyses, including as covariates patients’ age, type of CHD diagnosis, other clinical factors (e.g., number of surgeries, comorbidities, time since last surgery) and patients’ and parental psychological wellbeing. Another limitation is the cross-sectional research design. Follow-up measurements of patients’ QoL are needed to monitor patients’ QoL and familiar adjustment longitudinally. Finally, our study does not include an evaluation of QoL of CHD patients with neurodevelopmental disabilities and/or intellectual disabilities, and parental mental health.
