Quality of Life and Cognitive Function in Pediatric Patients Undergoing Surgery for Heart Diseases: A Rapid Systematic Review and Meta-Analysis

Abstract

Background:

Scientific attention is increasingly being drawn to the emotional impact and neurodevelopmental difficulties experienced by children and adolescents with heart disease. Therefore, this article aimed to review the literature from the last decade on health-related quality of life (HR-QoL) and cognitive functions (CFs) in children and adolescents with heart disease, highlighting its implications for these populations.

Methods:

This rapid systematic review and meta-analysis were conducted using the Web of Science (WoS) database. We used the Scale for the Assessment of Narrative Review Articles (SANRA) as a quality control check and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) checklist. Two meta-analyses were conducted to synthesize intelligence quotient (IQ) and QoL outcomes using data extracted from eligible studies. Random-effects models, Hedges’ G or mean differences, and I² statistics derived from Cochran’s Q were applied to assess effect sizes and between-study heterogeneity.

Results:

A total of 133 articles were identified, and 23 were eligible. The main results suggested a relationship between the characteristics and consequences of heart disease and neurodevelopment, which influence QoL and functional areas. The meta-analysis revealed a significant decrease in total IQ in patients compared to controls. We also found that the psychosocial QoL of the patient was significantly lower than the physical QoL.

Conclusions:

Neurodevelopment and QoL are fundamental aspects that must be addressed in a preventive manner.This review responds to the challenges faced by children and adolescents with congenital heart disease (CHD) who have undergone one or more surgical interventions, particularly regarding neurodevelopmental outcomes and executive function deficits, as examined through cohort and cross-sectional studies. However, a critical gap remains in the literature regarding longitudinal studies that evaluate the impact of short-, medium-, and long-term interventions specifically designed for this population.

Graphical abstract

Keywords

heath / pediatric heart disease / quality of life / surgery / cognitive function

Introduction

Pediatric populations with complex medical conditions (CMCs) require long hospitalization periods and re-admissions, associated with permanent disabilities, major frailty, and medical complexity [1]. Consequently, addressing a CMC directly challenges not only patients and caregivers but also siblings and family social relationships. Therefore, pediatric chronic disease conditions alter the roles and lifestyle of the whole family [2]. Children with heart disorders are one of the largest chronic pediatric populations, with high comorbidity and recurrent healthcare service contact [3]. Congenital cardiac disturbances are one of the most prevalent CMCs and the most predominant congenital disorders [4, 5].

Concept of Health and Impact of Pediatric Health and Heart Disease Needs on Children and Family Well-Being

Evolution of the Health Concept and Health Needs

The concept of health has evolved over the years. Indeed, public health, health determinants and promotion, well-being and quality of life (QoL), healthy habits, health literacy, psychoeducation in hospitals and health settings, and neurodevelopment must now be considered holistically. Concurrently, technological innovation and advances, such as medical devices, robotics, artificial intelligence, and bioethics, must be integrated.

In 1946, the World Health Organization (WHO) [6] defined health as a state of complete physical, mental, and social well-being, rather than the mere absence of disease. In 1976, the definition was expanded to include the condition of harmony, encompassing the functional, psychological, and psychic balance of an individual dynamically integrated into their natural and social environment: a way of living that is increasingly autonomous, supportive, and happy [7]. However, Gil-Roales [8] discussed illness responses, conceptualizing them as a form of illness behavior. Conversely, Bircher and Hanh [9] consider human health as an adaptive complex system that changes throughout life, adhering to a definition of health established by Boers and Cruz Jentoft [10], conceptualizing health as resilience, or the ability to cope, maintain, restore integrity, and balance a sense of well-being in physical, mental, and social domains. Hence, frailty was considered the weakening of health.

Various organizations focus on facilitating the right to health, such as the Pan American Health Organization (PAHO) [11]. Notably, the PAHO was the first of its kind and has been dedicated to ensuring health rights through public health policy work in the American region since 1902. The PAHO focuses on improving equity to ensure health accessibility and universal health coverage. Meanwhile, another example is the International Network of Health Promoting Hospitals and Health Services, which was established as an initiative of the WHO in 1988 to improve health by improving the quality of care (among hospitals, health services, the community, and the environment), as well as enhancing conditions and satisfaction of patients, families, and staff. The United Nations International Children’s Emergency Fund [12] implements health care projects focused on maternal, newborn, and child survival, as well as, child and adolescent health and well-being; meanwhile, the Global Health Council [13] advocates for global health awareness and legislation; Doctors of the World [14] states that healthcare is not a privilege but a human right are among a plethora of other non-profit international entities committed to global health rights; The Foundation for Global Community Health [15] promotes healthy schools for healthy children.

McPherson et al. [16] proposed the need for developing community service systems aimed at special health needs. This highlights the importance of developing programs to address the specific needs of pediatric populations with special healthcare needs, such as those in pediatric cardiology. Edwards and Goodman [17], in a review addressing the needs of children with severe chronic illness, determined various important concepts, such as complex chronic conditions described by Feudtner et al. [18] and Pinto et al. [19] as children with complex health conditions, with a symptom duration that exceeds 12 months (unless death occurs), involving different systems or only one, but severe enough to require specialized pediatric care and possibly a certain period of hospitalization in a highly specialized center [19, 20]. Therefore, including previously excluded diagnoses such as children with chronic critical illness (CCI; [21]) and severe neurological impairment (SNI); [22, 23]), or referring children and adolescents dependent on a medical device to compensate for the loss of a vital bodily function with substantial and ongoing nursing care to prevent death or further disability (technology dependent in children and adolescents) [24]. In this article, we adopt the term “visualized” from Muñoz-Violant et al. [25], who used it in the context of a CMC study examining well-being factors in childhood and adolescence versus the hospitalization experience. This concept evolved from the term “children with medical complexity”, coined by Cohen et al. [3] and followed by Berry et al. [26].

Impact of Pediatric Health on the Well-Being and Cognitive Function of Children and Families With CMC and Heart Disease

Recent studies about parental experiences of caring for children with CMC indicate that family function and changes are prevalent. Parental involvement in the care of the children is associated with improved family functioning; however, it also decreases the psychological health of caregivers, thereby increasing their own healthcare needs [27, 28]. Bowden et al. [29] revealed stress–emotional symptoms in both parents if one of them had psychological affectations, which could disrupt family roles. Moreover, pediatric illness may cause fearful caregiver feelings related to the uncertainty of the need for numerous pediatric healthcare services and an unpredictable or unknown situation. This lack of confidence is especially accentuated in situations unknown to the family, such as the diagnosis moment, instant health information, and hospital admission and discharge [30]. Parental stress and anxiety levels when children are undergoing surgery for congenital heart disease are significantly higher compared to other types of surgeries [31, 32, 33]. Recent research suggests that professionals should consider the roles, anticipation, and coping strategies of the parents, and that professional support addressing parental stress can be provided [34]. All of this should help to self-manage the high risk of the surgery on the child, but also to attenuate the risk of mental and emotional alterations following immediate and late postoperative heart surgery [28].

Heart disease, congenital or acquired, requires routine family changes, hospital-reiterated visits, strict medical management, heart surgeries, and invasive and non-invasive tests, and involves a real mortality risk [35]. Therefore, heart disease is associated with specialized care and early critical needs [36]. Disease severity and associated complications maintain a higher risk of morbidity and disability development, both during childhood and adulthood [37]. However, emotional and behavioral problems are higher in pediatric patients with heart disease than in other pediatric populations [38]. Therefore, anxiety and emotional disturbances may be an important factor in the effect of cardiovascular pediatric surgery. Conversely, cardiovascular pediatric surgery can evoke negative emotions in a new and stressful situation, although it can also be viewed as a healing and QoL improvement mechanism [34]. The perioperative period can be emotionally devastating for children and adolescents who suffer from anxiety and distress in the pediatric surgical field, which has a considerable number of invasive and non-invasive procedures [39, 40]. This phenomenon is associated with an event having an uncertain outcome and is frequently accompanied by fear or distress [39]. Therefore, importance must be given to reducing anxiety levels and increasing the control of a new medical event if we focus on pediatric cardiac surgery [41]. Recently, several studies have emphasized the importance of focusing attention on the neurodevelopmental difficulties shown in children and adolescents with heart disease. Specifically, Marino et al. [42] concluded that there is an increased risk of developmental disabilities or delays, as well as difficulties in cognitive function [43, 44, 45] and various other domains [42, 46, 47]. Brosig et al. [48] described a population presenting problems with mathematical operations, pronouncing words, spelling, handwriting, attention deficits, and difficulties with sequential instructions.

Therefore, this article aimed to review the literature from the last decade on health-related quality of life (HR-QoL) and cognitive functions (CFs) in children and adolescents with heart disease, highlighting its implications for these populations.

Material and Methods of the Review

Literature Review on Health-related Quality of Life and Cognitive Functions in Children and Adolescents With Heart Disease

We reviewed the literature from the last decade regarding HR-QoL and CFs in children and adolescents with heart disease. The literature was reviewed in a search of the Web of Science Core Collection database. The scale for assessing narrative review articles (SANRA) [49] and the PRISMA checklist [50] were used as a quality control check of this review. The advanced search was conducted using the equation: (((TS=((“quality of life” OR “health-related quality of life”))) AND TS=((child* OR adolescent* OR youth OR teenager))) AND TS=((“Heart Disease”))) AND TS=((“Cognitive function” OR “Executive function”)). The articles reviewed were screened based on title, publication, population, and keywords (Table 1).

VOSviewer software (version 1.6.18, Leiden University, Leiden, The Netherlands) was used to construct and visualize the relationships among the most frequently occurring terms in the titles and abstracts of the selected articles. The maps generated displayed a network of nodes and links, with colors representing different clusters. The size of the nodes reflected the occurrence frequencies, while the thickness of the links between nodes indicated the co-occurrence frequencies. The analysis settings were as follows: the counting method was full counting, the minimum number of occurrences of a term was set to 5, and we selected 60% of the terms with the highest relevance score to build the maps.

This review includes two meta-analyses. The first one is focused on the studies included in the review that provided total intelligence quotient (IQ) scores collected using either different editions of the Wechsler Intelligence Scale for Children (WISC) or the Differential Abilities Scales-Second Edition (DAS-II) questionnaires. To standardize data to a common scale, we calculated Hedges’ g as an estimator of the standardized mean difference (SMD) [51] versus a normative population (100 \pm 15) or control samples. The second meta-analysis focused on studies that provided physical and psychosocial QoL data collected using the Pediatric Quality of Life Inventory (PedsQL). We calculated the mean differences (MDs) and 95% confidence intervals (95% CIs) for these two QoL dimensions. In both meta-analyses, between-study heterogeneity (I²) was assessed using Cochran’s Q statistic, which follows a chi-square distribution, and a random-effects model was applied when heterogeneity (I²) was \ge40%. Analysis of sensitivity (z) was conducted to evaluate the reliability of the results. Publication bias was assessed using Egger’s test based on the funnel plot. In the total IQ meta-analysis, a linear regression model was used to determine the effects of patient age and questionnaire type on the meta-analysis outcome. Differences were considered significant with p-values 0.05. All analyses were performed using the meta package in R [52]. R packages were executed in RStudio 2022.12.0-353 for Windows (Posit.co, Boston, MA, USA) and R 4.2.2 for Windows (The Comprehensive R Archive Network -CRAN-, Vienna, Austria).

Results

The search was from 2015 to 2024. From the 133 identified articles, 85 were eligible after removing duplicates (48 articles). A two-stage screening process was applied, where all eligible studies were initially screened by title and abstract (31 studies were eligible). Then, the full text was reviewed (23 articles were eligible) (Fig. 1).

Highlight the Implications of Having Heart Disease and Undergoing Surgery as a Child or Adolescent Narrative Analysis

We synthesized the principal elements of each study, including sociodemographic and medical variables, QoL and psychosocial aspects, the domains of neurodevelopmental impairments, and the main results (Table 2, Ref. [45, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74]).

The 23 reviewed articles examine QoL and psychosocial aspects, as well as domains of neurodevelopmental impairments, involving children and adolescents with heart disease. Collectively, these studies analyze, compare, and establish associations between sex/gender, race, and medical characteristics, including the type of cardiopathy and variables related to surgical intervention.

A significant number of the included studies (65.21%) analyzed this population in relation to different types of heart diseases [54, 59, 63, 69, 73] or about different types and/or numbers of surgical interventions [53, 56, 60, 61, 64, 65, 66, 67, 71, 72]. Few studies included a control group of children without heart disease [45, 57, 58, 70, 74]; however, Cainelli et al. [68] included other pediatric medical conditions. Meanwhile, two studies were theoretical/reports [55, 62]. It is important to note that some studies include adults or young adults in their sample [59, 71, 73]. Additionally, some authors present results on the use of psychometric tests and neuroimaging [55, 57, 70, 74].

In some articles, parents or legal guardians played a significant role in detecting and assessing the situations and QoL of the children and adolescents. For example, Wotherspoon et al. [65] indicated that adolescents with congenital heart disease (CHD) had better family relations than the standardized sample. Nine articles evaluated the QoL or the situation from the point of view of the parents for the daughters and sons [45, 54, 58, 60, 63, 65, 69, 72, 73]. According to Svensson et al. [63], this assessment demonstrated strong agreement in the physical, social, and school function domains of the PedsQL 4.0 test, as well as a large correlation in all domains.

Seven of the selected articles contained several direct references to QoL [54, 56, 61, 63, 65, 71, 72], whereas 11 articles mentioned QoL indirectly [45, 55, 57, 58, 59, 60, 62, 64, 67, 69, 73]. Some authors analyzed the relationship between QoL and cognitive functions, including verbal skills. Dardas et al. [72] revealed different results depending on the evaluation scale. On the one hand, a positive correlation between IQ and QoL was found between Wechsler’s scores and parent-reported QoL scores, especially in the social and educational dimensions, but not in Raven’s scores.

Spillmann et al. [67] described the education and leisure activities situation of children with congenital heart disease, focusing on children who underwent cardiopulmonary bypass surgery. Spillmann et al. [67] determined that educational support, including additional years of schooling, grade retention, transition/introductory classes, and extra tutoring, was necessary for 26.4% of the children with CHD who received education in regular schools. Leisure activities were defined as sports (72%), musical activities (46%), and other social activities (20.8%). Finally, Spillmann et al. [67] concluded that lower IQ and less participation in leisure activities were associated. In line with this, Gerstle et al. [54] found that survivors with single-ventricle lesions had significantly lower school QoL compared with those with two-ventricle lesions. Moreover, Gerstle et al. [54] found that metacognition was the strongest predictor of school-related problems, compared to IQ and sociodemographic variables.

Finally, some studies assessed depression and/or anxiety variables [55, 57, 63, 64, 69]. Adolescents with single ventricle heart disease had higher anxiety scores than the control subjects [57], and caregivers reported elevated scores in children and adolescents for depression and anxiety if these individuals had a history of Fontan disease [69]. Somewhat similarly, Tester et al. [64] affirmed that anxiety and avoidant behavior could be related to psychological mobilities in supraventricular tachycardia (SVT) experience in a pediatric sample. Otherwise, Latal [55] analyzed the behavioral problems and concluded that clinical assessment could be necessary with the most complex CHD.

Jackson et al. [59] explained the relationship between executive problems and internalizing symptoms with primary, secondary, and disengagement control coping. The sample included adolescents and emerging adults (AEAs), as well as young adults (YAs). However, Jackson et al. [59] did not find a relationship between the AEA results and coping; meanwhile, the authors suggested that low coping strategies in YAs were associated with more executive function problems and internalizing symptoms. According to Sanz et al. [61], executive dysfunction, defined as weaknesses in cognitive processes, was a strong predictor of childhood psychosocial QoL. This was especially important to CHD children with injury or related factors that could affect brain maturation. Additionally, Farr et al. [58] conducted a cross-sectional study with a sample of 1416 children and adolescents with special healthcare needs and heart disease (CSHCN), comparing their functional limitations and educational difficulties with those in a standardized group.

The results were heterogeneous in the use of different tests to assess the cognitive and metacognitive elements that influence neurodevelopment (Table 2). Therefore, an effort was made to maintain the various concepts used by the authors in this classification. Overall, the findings can be grouped into three main categories: (1) IQ, (2) executive functions and metacognition, and (3) academic performance (as a consequence of the development of the previous two categories). The most relevant results for each group are presented in Table 2.

Statistical Analysis

We presented two types of results. On the one hand, we presented the bibliometric analysis using VOSviewer. After applying the term-selection criteria, the initial number of 894 terms in the titles and abstracts of the 23 selected articles was reduced to 36 items in the network map. For instance, terms such as “score”, “heart disease”, “quality”, and “life” represent the most used terms, with more than 25 occurrences each (Fig. 2A). The analysis also revealed a map of 276 links and five main clusters. One of the clusters had the term score as the most common, and the terms “adolescent” and “neurodevelopment” also presented high occurrences. This cluster also presented many strong links within terms (Fig. 2B). A central cluster, with heart disease as the main term, showed a high number of links with terms in the other clusters (Fig. 2C). These included terms related to adolescence, attention, and executive functions, as well as QoL. A third cluster with 11 terms exhibited a more dispersed structure with attention as the most occurrent term and risk as the central term (Fig. 2D). The fourth cluster had quality and life as the main terms (Fig. 2E) and was strongly connected with the fifth small cluster that presented the terms executive function and QoL as the only terms.

Alternatively, we performed a meta-analysis of the effects of CHD on the total IQ of each patient. Nine articles reported data regarding the total IQ of patients using the WISC (8 studies) or the DAS-II (1 study) questionnaires. The combined sample size was 689, and we found a significant total IQ decrease in patients (SMD = –0.38, 95% CI [–0.51; –0.25], t = –6.71, p = 0.0002; Fig. 3). We observed moderate heterogeneity (I² = 42.3%, p = 0.0855) in the random effects model. Meanwhile, the sensitivity analysis showed that SMD remains stable when removing any study from the model (z = –6.71; p 0.0001; Supplementary Fig. 1), and we found no significant publication bias (t = 0.49; df = 7; p-value = 0.6384; Egger test; Supplementary Fig. 2). Subsequent linear regression analysis of the meta-analysis model showed no effect of the age of the patient or the used IQ questionnaire in the heterogeneity of the model (F(2,6) = 0.2688; p = 0.7730; Supplementary Table 1), which indicates that the age and the questionnaire do not affect the IQ decrease in the CHD patients used in our analysis.

The second meta-analysis evaluated the QoL of the patients. Only four articles reported complete data regarding the QoL assessment. We found that the combined sample size was 220 for both the physical and psychosocial QoL dimensions of the questionnaire, and the combined effect was not statistically significant, although a tendency could be inferred (mean difference [MD] = 4.57, 95% CI [–0.71; 9.86], z = 1.69, p = 0.0904; Fig. 4). The small diamond-shaped square falls to the right of the null line, which implies that the physical QoL is higher than the psychosocial one. Moderate heterogeneity was observed in this study (I² = 53.3%, p = 0.0926), as determined by a random-effects model. This heterogeneity was primarily based on the article by Zampi et al. [71], whose study is the only one to provide physical QoL values lower than those of the psychosocial QoL. Thus, after removing this study in the sensitivity analysis, the combined effect of the remaining studies was statistically significant (MD = 6.49; 95% CI [2.47; 10.51]; z = 3.16; p = 0.0016; Supplementary Figs. 3,4), and the study observed very low heterogeneity (I² 0.5%).

Discussion

This article aimed to review the literature on HR-QoL and CFs in children and adolescents with heart disease, highlighting the implications. This review emphasizes the importance of assessing and addressing the neurodevelopmental needs of children and adolescents with CHD, aligning with the guidelines of the American Heart Association and the American Academy of Pediatrics [42]. Meanwhile, emerging interventions [75, 76] and executive function assessment protocols [43, 77] continue to highlight that executive function impairments are among the most prevalent neurodevelopmental morbidities in this population. These results align with numerous studies in the field that document these same phenomena [61, 78].

Children with heart disease report higher levels of depressive and anxiety symptomatology than their healthy counterparts, particularly during periods of hospitalization [79]. Therefore, addressing this aspect as poor mental health, which has detrimental effects on both the short- and long-term physical and psychological health and QoL of this population, is of paramount importance [80]. Progress in personalized multidisciplinary medical interventions has substantially enhanced the survival rate and QoL of the pediatric population with CMC, especially those with cardiopathy/heart disease. However, psychological interventions for this population are limited; nonetheless, these remain crucial to the well-being of the child and involve patient rights and shared duties and responsibilities of the healthcare systems [81]. Intervention programs focused on psychological support and suitable information should be implemented, programs that not only impact the care that parents should provide to their children, but also enhance self-care in children and adolescents.

Numerous studies have explored the outcomes of these children from the perspectives of the parents. However, limited research exists comparing these perspectives with those reported by the children. Muñoz-Violant et al. [25] found that the perception of disease in youth with complex health conditions, including CHD, was significantly lower than that detected in caregivers. This is important as the perceptions of parents toward their children may act as a self-fulfilling prophecy.

The meta-analysis revealed substantial variability in the presentation and analysis of cognitive function data in children with CHD. This heterogeneity precluded a meta-analysis that would enable direct comparisons of cognitive function between children with CHD and the general population, as well as assessments of correlations with cardiomyopathy severity or socioeconomic status. However, further analysis showed that all included studies reported similar outcomes, with none contributing disproportionately to heterogeneity or displaying significant bias. We examined the potential influence of age and the type of cognitive assessment used (WISC or DAS-II), but neither factor had a significant effect on the results. This suggests that the moderate heterogeneity observed in the study (42.3%; p = 0.0855—borderline significance) is likely related to differences in the severity or type of heart disease or variations in patient cultural and socioeconomic backgrounds.

Four studies were identified that reported comparable HR-QoL data using the same measurement instrument. Based on these studies, our meta-analysis compared physical and psychosocial QoL assessments in children with CHD. The results demonstrated a consistent trend toward higher physical QoL relative to psychosocial QoL. Nevertheless, statistical significance was not reached due to data heterogeneity, primarily driven by the Zampi et al. study [71]. This study focused on patients with severe CHD who had recently undergone open-heart surgery and pulmonary valve replacement. Sensitivity analysis excluding this study revealed a significant reduction in psychosocial QoL compared to physical QoL, underscoring the importance of addressing psychosocial health in the management of children with CHD.

Limitations

This study has several limitations. First, the literature review is limited to the past decade (2015–2024). While this timeframe was considered adequate for capturing the current state of research on the QoL and CF of children and adolescents with heart disease, it may have led to the exclusion of relevant studies published before 2015. Second, this review does not account for long-term outcomes of cardiac interventions, as it focuses on studies involving pediatric and adolescent populations. As a result, research examining the health consequences and potential psychosocial comorbidities during the transition to adulthood was not included. Finally, the substantial heterogeneity of congenital and acquired heart diseases in childhood poses a challenge to generalizability. The conclusions drawn from this study may be influenced by the conditions most represented in the selected literature, introducing a potential bias and limiting their applicability to less frequently studied cardiac disorders.

Conclusions

Caring for children and adolescents with heart disease involves going beyond the disease itself and the surgical interventions that may be performed. Neurodevelopment and QoL are fundamental aspects that must be addressed in a preventive manner. Indeed, implementing specific intervention programs is essential for improving the QoL and cognitive functions of young CHD patients. This requires the integration of psychometric data with functional magnetic resonance imaging procedures. In recent decades, it should be noted that there has been an advance in diagnostic, therapeutic, and repair techniques, giving rise to a population that reaches adulthood with a cardiovascular disease.

This review responds to the challenges faced by children and adolescents with CHD who have undergone one or more surgical interventions, particularly regarding neurodevelopmental outcomes and executive function deficits, as examined through cohort and cross-sectional studies. However, there remains a critical gap in the literature regarding longitudinal studies that evaluate the impact of short-, medium-, and long-term interventions specifically designed for this population.

Availability of Data and Materials

All data points generated or analyzed during this study are included in this published article.

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Funding

Next Generation EU (Generalitat de Catalunya)(DDS008/22/000524)