A comprehensive evaluation of quality of life and its determinants in portuguese pediatric patients with repaired tetralogy of fallot: An observational study

Abstract

Background:

This study aimed to assess parental perceptions of quality of life in children aged 5–18 with simple and complex forms of repaired tetralogy of Fallot and its determinants, using an original survey including the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) questionnaire.

Methods and Results:

Data were gathered through an original online survey of repaired tetralogy of Fallot patients at a reference center for congenital heart disease. The survey, conducted between March and July 2023, included the PedsQL 4.0 (parental version), inquiries on extracurricular sports participation, sleep patterns, distance from the hospital, and social assessment. Of 74 contacts, 51 responses were obtained, representing children with an average age of 10.5 ± 3.7 years. Parents perceived lower quality of life in these children compared to healthy Portuguese counterparts, both in physical (72% vs. 83.5%, P < 0.001) and psychosocial (73% vs. 78.2%, P = 0.01) dimensions. Higher physical and psychosocial quality of life were associated with extracurricular sports practice (P = 0.012 and P = 0.008, respectively), as well as with a greater number of hours spent in extracurricular sports (P < 0.00001 and P = 0.01, respectively). Factors such as sleep patterns, distance from the hospital, socioeconomic status, and number of surgeries did not correlate with quality of life.

Conclusion:

Portuguese children with repaired tetralogy of Fallot exhibit a lower quality of life than their healthy peers, as perceived by parents. Regular sports engagement is associated with improved quality of life, emphasizing the importance of assessing sports habits as an integral component of their follow-up.

INTRODUCTION

Congenital heart disease is one of the most common congenital anomalies. Interventions have undergone significant advancements, resulting in a reduced mortality rate and an expanding population of children living with chronic heart conditions.[1] The evolving landscape of pediatric cardiology has expanded the focus from survival to the broader spectrum of improving the quality of life and health-related quality of life for children affected by these conditions.[1,2]

The impact of chronic diseases on living capacity, performance, and associated health costs highlights the necessity for a comprehensive assessment of quality of life to optimize treatment, care, and rehabilitation programs for affected children.[3,4] Congenital heart disease often manifests with physical symptoms that can potentially impede psychological and social well-being in pediatric patients. To delve deeper into its multifaceted implications, specific tools such as the Pediatric Quality of Life Inventory (PedsQL) have proven indispensable.[1,2,3,4,5] This instrument, tailored for children aged 2–18, encompasses physical, emotional, social, and school functioning dimensions.[1,2,4] Moreover, acknowledging the broader spectrum of physical activity as crucial for optimal development, especially in those with congenital heart disease, has become imperative.[6,7]

Exercise capacity emerges as a key predictor of health outcomes, particularly in children with tetralogy of Fallot after surgery.[8] Encouraging physical activity in childhood not only serves preventive roles against long-term health risks but also contributes significantly to positive emotional, social, and intellectual development.[9,10,11,12,13,14,15,16] Despite these benefits, children with congenital heart disease often exhibit sedentary behaviors, emphasizing the necessity for targeted counseling. Understanding the physical and psychosocial health benefits of physical activity becomes crucial for this population, which is at risk for exercise intolerance, obesity, and other cardiovascular risk factors.[6]

Our study aims to evaluate parental perceptions of the quality of life in children with corrected tetralogy of Fallot. By correlating these perceptions with factors such as sports participation, sleep patterns, proximity to the hospital, and social class, we seek to deepen our understanding of the intricate factors involved in the overall well-being of these patients.

MATERIALS AND METHODS

Study design and patient selection

We conducted an observational cross-sectional study, gathering data through an original online survey and a review of the patient database for individuals aged 5–18 with simple and complex forms of tetralogy of Fallot after surgery. These individuals were under the care of the pediatric cardiology department at a tertiary hospital and reference center for congenital heart disease. To participate, patients needed regular follow-up and a recent echocardiogram within the past 12 months. Phone contacts made by physicians who explained the study determined parental consent for participation. Unanswered calls prompted at least three attempts on different days, with noncontact leading to exclusion. Participants had the option to withdraw at any time. Investigators collected the tricuspid annular plane systolic excursion value as a measure of right ventricular function and the tricuspid valve Z-score as a measure of right ventricular dilatation of each patient from the most recent echocardiogram.

Online survey

The online survey was tailored for this study and included patient identification, the PedsQL 4.0 questionnaire for parents, sports practices, daily sleep hours, hospital distance, and social assessment using the Graffar scale. These measures provided a comprehensive evaluation of multiple well-being determinants. Surveys featured quantitative questions with multiple-choice, numerical, and Likert scale responses, ensuring only fully completed surveys were submitted. The survey period ranged from March to July 2023. Surgical intervention data was also collected.

The PedsQL 4.0 questionnaire is an instrument to measure health-related quality of life in children. It is used worldwide and has a Portuguese-validated version. It includes patient and parent versions. While the child’s version may be more complete, it usually is less objective. The parents’ version tends to reflect more objective and behavior-based observation. Therefore, the online survey used the parents’ version to standardize answers among all age groups. Depending on the age of the child, the corresponding survey was applied: 5–7 years old, 8–12 years old, and 13–18 years old.[1,2,5]

Hospital distance was questioned to determine the availability of easy healthcare access. Sports practiced and the number of sleep hours were questioned to assess healthy lifestyle habits. The adapted Graffar scale was used to determine the socioeconomic status of each family.

Statistical analysis

Data were gathered as means and standard deviations for continuous variables and absolute values and percentages for categorical variables. We compared the results from our cohort with data from healthy Portuguese children using the study from Lima et al. with simple regression analysis.[2]

The primary outcome was quality of life, especially the variables of physical quality of life and psychosocial quality of life. We determined the association between these and independent variables using simple and multivariable regression analysis. Statistical analysis was performed using the statistical program R. A significance level of P < 0.05 was accepted as statistically significant.

RESULTS

General data

We collected 51 questionnaires from 74 contacts. Twenty-three patients were excluded, comprising 17 whose parents did not respond to phone calls, five whose parents did not complete the postcontact questionnaire, and one patient whose parents declined participation.

Table 1 denotes the demographic and significant clinical characteristics of our cohort of patients.

Table 1.

Demographic, clinical, and echocardiographic characteristics of our cohort of patients

Demographic, clinical, and echocardiographic characteristics	Values
Number of patients	51
Age (years)	10.5±3.66
Gender (female)	24 (47)
BMI (%)	17.2±3.7
Anatomic diagnosis
Tetralogy of Fallot	35 (69)
Pulmonary atresia with VSD±MAPCAs	16 (31)
Syndromatic patients	7 (14)
DiGeorge syndrome (22q11.2 deletion)	4 (8)
Number of surgeries	1.9±0.9
Physical quality of life	0.72±0.47
Psychosocial quality of life	0.73±0.41
Extracurricular sports practice	26 (51)
Number of weekly hours of extracurricular sports	2.56±1.7
Distance from the hospital (km)
0–19	18 (35)
20–99	20 (39)
100–999	12 (24)
≥1000	1 (2)
Daily hours of sleep	9.5±1.3
Graffar scale
Class I	4 (8)
Class II	22 (43)
Class III	22 (43)
Class IV	3 (6)
TAPSE	17.28±4.44
Tricuspid valve Z-score	0.09±1.41

Values presented as mean±SD, or absolute number and percentages. TAPSE: Tricuspid annular plane systolic excursion, BMI: Body mass index, VSD: Ventricular septal defect, MAPCAs: Major aortopulmonary collaterals, SD: Standard deviation

There were 35 (69%) patients with repaired tetralogy of Fallot and 16 (31%) with repaired pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs). These last patients were all repaired with a right ventricle to pulmonary artery conduit, VSD closure, and variable degrees of MAPCAs unifocalization. Patients with repaired tetralogy of Fallot demonstrated a significantly higher average physical quality of life of 76% compared to those diagnosed with repaired pulmonary atresia with VSD and MAPCAs, who exhibited a lower average of 62% (P = 0.027). However, regarding psychosocial quality of life, patients with tetralogy of Fallot reported an average of 75%, while those with pulmonary atresia showed a slightly lower average of 68%, and this difference did not reach statistical significance (P = 0.164) [Figures 1 and 2].

Figure 1.

Physical quality of life by diagnoses (P = 0.027). VSD: Ventricular septal defect, MAPCAs: Major aortopulmonary collateral arteries

Figure 2.

Psychosocial quality of life by diagnoses (P = 0.164). VSD: Ventricular septal defect, MAPCAs: major aortopulmonary collateral arteries

There were also 7 (14%) syndromic patients, of which 4 (8%) had DiGeorge syndrome (22q11.2 deletion), 2 had an unknown diagnosis, and 1 with 22q11.2 duplication syndrome.

Practiced sports included swimming (46.15%), football (11.54%), volleyball (11.54%), karate (11.54%), dance (7.69%), therapeutic riding (3.85%), cycling (3.85%), futsal (3.85%), skating (3.85%), judo (3.85%), yoga (3.85%), gym (3.85%), teqball (3.85%), ballet (3.85%), hip-hop (3.85%), rhythmic gymnastics (3.85%), and canoeing (3.85%).

Results from the pediatric quality of life inventory 4.0 questionnaire

Our results showed a parental perceived physical quality of life of 72% and a psychosocial quality of life of 73%. Compared with data from healthy Portuguese children (2), our cohort exhibited lower quality of life scores, both in the physical dimension (72% vs. 83.5%, P < 0.001) and psychosocial dimension (73% vs. 78.2%, P = 0.01).

Determinants of physical and psychosocial quality of life

In simple regression analysis, higher physical and psychosocial quality of life were associated with extracurricular sports practice (P = 0.012 and P = 0.008, respectively), as well as with a greater number of hours spent in extracurricular sports (P < 0.00001 and P = 0.010, respectively) [Table 2]. The only other variables with a significant association with higher physical quality of life were the baseline diagnosis of Fallot tetralogy when compared with PA-VSD and MAPCAs and not having syndromic traits. Of note, neither distance from the hospital, hours of sleep, socioeconomic status, or echocardiographic variables had a significant association with quality of life in this cohort.

Table 2.

Determinants of physical and psychosocial quality of life

Univariate analysis
	Physical quality of life		Psychosocial quality of life
	Regression coefficient	P	Regression coefficient	P
Pulmonary atresia versus tetralogy of Fallot diagnosis	−0.148	0.025	−0.071	0.163
Not having a syndrome	0.186	0.035	0.151	0.024
Practicing extracurricular sports	0.138	0.023	0.111	0.015
Number of hours of extracurricular sports	Log trans 0.258	5.54E–07	0.046	0.008
Multivariate Analysis
Number of hours of extracurricular sports
	Physical quality of life		Psychosocial quality of life
	Regression coefficient (95% CI)	P	Regression coefficient (95% CI)	P
	Log trans 0.244	2.29E–06	0.134	0.004

Only statistically significant correlations (P<0.05) are shown. Independent variables used in univariate regression analysis: Age, gender, BMI, number of surgeries, type of diagnosis, being syndromic, practicing extracurricular sports, number of hours of extracurricular sports, distance from the hospital, daily hours of sleep, Graffar Scale, TAPSE, tricuspid valve z-score. Independent variables used in multivariate regression analysis were the ones statistically significant in univariate analysis. BMI: Body mass index, TAPSE: Tricuspid annular plane systolic excursion, CI: Confidence interval

In multivariable regression analysis, only the logarithm of the number of hours of sports practice was informative in predicting variations in quality of life [Table 2]. The remaining variables examined did not provide useful information for forecasting either physical or social quality of life.

DISCUSSION

Tetralogy of Fallot is the most prevalent congenital cyanotic heart condition. Given the improved survival rates after the corrective surgery, evaluating the quality of life in these patients and exploring ways to optimize their well-being is imperative. Our study provides valuable insights into how parents perceive the quality of life in children with corrected tetralogy of Fallot.

The diminished quality of life noted in these children, as conveyed by parental reports, both in physical and psychosocial dimensions, underscores the multifaceted impact of this disease and the need for a comprehensive understanding of these dimensions to tailor effective interventions and support systems.

Interestingly, as outlined in previous studies, parents consistently reported their children’s quality of life to be lower than healthy children.[17,18,19] It is essential to consider that parents’ perceptions of their children’s quality of life mainly influence healthcare decision-making, so it would be interesting to compare this perspective with the self-reported quality of life. In fact, in studies comparing the self-reported and parent proxy-reported quality of life in children with congenital heart disease, self-reported quality of life appears similar to healthy peers. In contrast, the parents reported a lower quality of life.[17,18,19] Furthermore, parents may be interpreting it in the context of their own experience as caregivers, as it is known that, in general, parents of children with chronic health diseases are prone to developing stress and anxiety, undermining the well-being or overall stability of their family.[20] Therefore, it is necessary to integrate psychosocial support dedicated to such parents and develop multidisciplinary family-centered psychosocial care for patients with congenital heart disease, as recommended by the AEPC psychosocial working group.[21]

Health-related quality of life in children is a multidimensional concept encompassing physical, psychological, social, and academic functions; however, for children with chronic illnesses and congenital heart disease, exercise capacity and functional status appear to play a significant role in determining quality of life.[22] In our multivariable regression analysis, the logarithm of the number of hours of sports practice was informative in predicting variations in quality of life, according to different studies reporting quality of life positively correlated with the child’s exercise capacity.[17,22]

According to Portugal’s 2022 report card on physical activity for children and youth, only 20%–26% of Portuguese youngsters were sufficiently active (considering WHO guidelines of 60 min per day of moderate-to-vigorous physical activity). Furthermore, results from this report showed that 40%–46% of Portuguese children are engaged in an extracurricular sport. Our studied population showed a slightly higher percentage of extracurricular sports practice (51%), which may be due to greater awareness among these families to maintain a healthier lifestyle with sports practice in children with congenital heart disease.[23]

The positive correlation between extracurricular sports participation and improved quality of life suggests that physical activity may potentially enhance overall well-being. The observed higher physical and psychosocial quality of life in children participating in extracurricular sports aligns with existing literature, highlighting the wide-ranging benefits of physical activity. Notably, the variety of sports the study participants engage in reflects the extensive choices accessible to these patients, accommodating diverse interests and preferences.

Beyond this, the positive correlation between the weekly hours spent on extracurricular sports and improved physical quality of life highlights the potential dose-response relationship. This discovery suggests that greater time investment in sports positively impacts the physical well-being of children, emphasizing the significance of regular and continued participation.

Therefore, comprehensive follow-up should include cardiac rehabilitation and psychosocial evaluation to ensure an active lifestyle, improve health perception, and prevent acquired heart disease later. Showing parents that their children have better exercise performance by participating in a cardiac rehabilitation program may not only foster a healthy lifestyle for the children but also may educate caregivers and improve perceptions of quality of life by both children and their parents.[17]

The observed disparity in physical quality of life between patients with tetralogy of Fallot and its most complex form, pulmonary atresia with VSD, underscores the impact of diagnosis on these patients’ well-being. It highlights the need for tailored interventions and support systems to address the unique challenges associated with each cardiac condition, as more complex patients tend to have lower quality of life.

Contrasting with other studies, our results did not confirm the negative impact of repeated invasive cardiac procedures (surgery) on quality of life.[18,19] It is also notable that other factors analyzed, including sleep patterns, distance from the hospital, and socioeconomic status, did not have a significant correlation with the perceived quality of life of these children, both in physical and psychosocial dimensions. This suggests that these aspects, commonly considered influential factors in health-related evaluations, may not directly impact the overall well-being of this specific population.

These findings highlight the importance of assessing sports behaviors during follow-up care. They contribute to a comprehensive understanding of the challenges encountered by these children and guide potential interventions.

This study has some limitations. First, this is a single-center study with a small sample. The strength of our results points toward their generalization, even if the sample were to be higher, but it remains difficult to extrapolate, as with any scientific work. Second, we rely on parental perceptions; therefore, there is a risk of a potential response bias. Future studies with a larger sample size could explore additional variables influencing the quality of life in these patients.

CONCLUSIONS

Our study underscores the critical role of extracurricular sports in enhancing the overall well-being of children with simple and complex forms of tetralogy of Fallot. This association is linked to notable improvements in both physical and psychosocial aspects of quality of life. These findings contribute essential information for clinicians, emphasizing the necessity of sports assessments as an integral component of the follow-up care for these patients. This can be done using comprehensive questionnaires such as ours in these and other cardiac conditions.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.