Paediatric quality of life in toddlers and children who underwent arterial switch operation beyond early neonatal period

Abstract

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OBJECTIVES

The aim of this study was to evaluate the quality of life (QOL) of children who underwent the arterial switch operation (ASO) for Transposition of Great Arteries in our population and, specifically, to explore early modifiable factors and the influence of parental and socioeconomic factors on the QOL of these children.

METHODS

Cross-sectional study using Paediatric Quality of Life Inventory™ 3.0 Cardiac Module was carried out on 3- to 12-year-old children who had undergone ASO between the years 2012–2018. Socioeconomic status was calculated using the modified Kuppuswamy scale (2019). Other clinical factors with possible bearing on the outcome were also analysed.

RESULTS

Immediate survival after surgery was 196 out of 208 (94.2%) with an attrition of 19 patients (9.6%) over the follow-up period. Most surviving children (98.9%) had started formal schooling in age-appropriate classes. Two children had severe neuromotor impairment. The median cumulative health-related QOL score of the children was 97.9 (interquartile range 4.2) at 5.6 ± 1.27 years of life. The median scores each of the health-related QOL parameters, viz, heart problem symptoms, treatment compliance, perceived physical appearance, treatment-related anxiety, cognitive problems, and communication was 100 with negative skewing.

CONCLUSIONS

Excellent QOL was observed in most children after ASO with the median total paediatric QOL scores in all domains of 97.9. Social factors did not show a statistically significant influence on the QOL parameters in the current cohort. The gradually declining trend across the age groups emphasizes the need for continued follow-up for early identification of possible correctable factors and initiating intervention to ensure good QOL into teenage and adulthood.

INTRODUCTION

The survival outcomes of transposition of the great arteries (TGA), one of the most common critical congenital heart diseases (CHDs) in the newborn period are excellent in the arterial switch operation (ASO) era. While the early survival rates vary from 91.2% to 97.8% [1, 2], late mortality is also reported after ASO. However, the 20-year survival probability after ASO is 94.9% and fast catching up with the general population (99.5%) [3]. Attention has shifted to improving the health-related quality of life (HRQOL) of survivors of CHD to ensure optimal developmental, cognitive, behavioural, and emotional outcomes.

Previous studies have documented reduced HRQOL in children with CHD in the developing world as compared to their western counterparts [4]. The developing world presents several logistic challenges for optimal health care including access to health care facilities, resource constraints, out-of-pocket expenditure, and other socioeconomic factors. Mild neuro-developmental delay was reported in 27.9% of children after ASO at 4 years of life [5]. While excellent HRQOL is reported in adulthood after ASO in Europe [6], there is paucity of literature regarding HRQOL in children after ASO in the developing world.

METHODOLOGY

Ethical statement

The study was conducted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its amendments. Approval was given by the Institutional Ethics committee (SCT/IEC/1195/AUGUST-2018 dated 6 September 2018) and informed parental consent in vernacular was obtained for the study. Patient detail privacy has been maintained and identity particulars have not been disclosed.

Ours was a single-centre cross-sectional study conducted over a period of 6 months from January 2019 to June 2019 and again from May 2022—October 2022. The study included all surviving children (aged 3 to 12 years) who had undergone ASO at our centre during the period Jan 2012–Dec 2018. The two-staged patient recruitment was initially planned to ensure that the children were older than 4 years for enrolment in the study. The second phase which was planned in 2020 was delayed due to the issues created by the Covid-19 pandemic. Patients were contacted over telephonic interviews or directly during the scheduled yearly follow-up visits. Patients who were not reachable by phone or refused to participate were excluded from the study. A structured Proforma was used to collect demographic, socioeconomic and relevant treatment details. Clinical data was collected from patient charts. Paediatric Quality of Life Inventory™ 3.0 Cardiac Module approved for the age groups: Toddler (2 to 4 years), young child (5 to 7 years) and Child (8 to 12 years) was used as questionnaire to measure quality of life (QOL).

Study tools

PedsQL™ 3.0 Cardiac Module and modified Kuppuswamy scale (updated for the year 2019)

The use of PedsQL3.0 cardiac module for the evaluation of HRQOL was licenced for this study by the licence holders (Mapi Research Trust, Lyon, France). The tool consists of child self-report and parent proxy report formats. We used parent proxy reports for the Toddler and the Young child group and the child self-report for the children. A 5-point Likert scale is then used to score the responses. Items are reverse- scored and linearly transformed to a scale from 0 to 100 point. Higher scores indicate better HRQOL. (For details of the domains, assessment questions, Likert scale for the different age groups [7]. The original tool in English was translated to the local languages (Malayalam and Tamil) by a language expert. The original and translated versions were checked for content validity and concurrence by the study team.

Modified Kuppuswamy’s socioeconomic status (SES) scale was devised by Kuppuswamy in the year 1976 and is the most widely used scale for determining the SES of an individual or a family in urban areas [8]. The scale is modified annually and includes 3 parameters—educational status of the head of family, occupational status and overall aggregate income of the whole family, pooled from all sources. The total score of Kuppuswamy SES ranges from 3 to 29 and it classifies families into 5 groups, ‘upper class, upper middle class, lower middle class, upper lower and lower socioeconomic class’.

The effect of the type of TGA (with or without ventricular septal defect), associated anomalies (aortic arch repair or left ventricular outflow tract obstruction), need for major cardiac reoperation, residual (at least moderate) pulmonary stenosis, residual (at least moderate) aortic regurgitation, current New York Heart Association classification, left ventricular function, history of rhythm disturbances and implanted pacemaker systems and need for anticoagulation or other current cardiac medication, on the QOL of these children were considered for analysis.

Statistical analysis

Since our main objective was the comparison the 5 QOL scale mean values among a normal population sample and our surgically treated TGA patients, we performed a post hoc power calculation with a conservative error probability level of 1% (according to Bonferroni, 5%/5 Tests -> 1%) [9]. According to PASS 2021 Power Analysis and Sample Size Software (2021). NCSS, LLC. Kaysville, Utah, USA, ncss.com/software/pass, the largest required sample size for a power of 90% and an error probability of 1% was 2 × 78, so that we can consider the achieved power to be sufficient. Statistical analyses were conducted using SPSS V.20.0 for Windows. All normally distributed continuous variables are presented as mean [standard deviation (SD)] and categorical variables as proportion. Descriptive statistics were used when variables were few for a meaningful statistical analysis. Effect of select factors (that have been previously reported by other studies as affecting QOL parameters after cardiac surgery), such as diagnosis, complexity of lesion, gender, age at surgery, age at follow-up, balloon atrial septostomy done or not, mothers’ age, education and income, socioeconomic scale on total and mean HRQOL scores in each domain, was computed using binary logistic regression taking 20th percentile of the HRQOL scores as the cut-off for faltering.

RESULTS

Patient characteristics

Two hundred and eight patients underwent ASO in the period January 2012–December 2018. A total of 196 patients survived to discharge. In-hospital mortality was 12 (5.7%). Thirteen patients were lost to follow-up and 5 were reported dead on telephonic enquiry. We had 178 responders to the telephonic interviews and direct questionnaires. All patients were included in the study. Most of the patients (64%) were young children (5–7 years). There were 118 boys and 60 girls (ratio 1.96:1). Parent proxy respondents were mothers (88%), fathers (11%) and others (1%). Patient demographics and relevant clinical details are listed in Table 1.

The mean age at follow-up was 5.6 (SD: 1.27) years (3.5–12.33 years). Two children required reoperations/reinterventions: 1 for severe neo-ascending aortic stenosis and the other for neo-pulmonary artery stenosis. One child had a pacemaker implantation. Most children were in New York Heart Association class 1 (n = 175; 98.3%). None of the children on follow-up were on cardiac medications.

Most surviving children except 2 with severe neuromotor involvement had started formal schooling in age-appropriate classes with none requiring special schooling.

Health-related quality of life scores

Overall scores

The median cumulative HRQOL score for the cohort was 97.9 (interquartile range 4.2). Forty-nine children scored 100 in the cumulative HRQOL score. While the data suggested that most children scored very well in the HRQOL scores, this presented a highly left-skewed distribution. The HRQOL mean scores were assessed for heart problem symptoms, treatment compliance, perceived physical appearance, treatment anxiety, cognitive problems, and communication. The median scores across each of these parameters were 100 with negative skewing (Table 2).

Details of mean scores in all domains for the different age groups are given in Table 2.

Socioeconomic class and correlates

Calculations were made taking the father as the head of the family excepting 2 children whose fathers were not alive. Socioeconomic status (SES) for the total cohort was as follows: upper SES (class I)—N = 5 (2.8%), upper middle SES (class II)—N = 48 (26.9%), lower middle SES (class III)—N = 75 (42.1%), upper lower (class IV)—N = 72 (40.4%), and lower (class V)—N = 1 (0.6%). Majority of the mothers of our patients had high school—N = 63 (35.39%) or higher secondary school education—N = 63 (35.4%). Most mothers were unemployed N = 159 (89.3%), and the rest N = 19 (10.6%) were employed as semi-skilled or skilled workers. None of the patients in the group belonged to broken families. Scores of the children in comparison with the different SES scales and the age group distributions are given in Table 3 and Fig. 1.

Parameters affecting health-related quality of life

Since the HRQOL results were negatively skewed with the majority performing well, the 20th centile was chosen as the cut-off to consider as the threshold for defining faltering in QOL scores. This threshold was obtained as 95.1 in the total HRQOL score. The odds of children with HRQOL below this threshold attending lower-than-for-age classes at school compared to those with higher scores was 11.0 (95% CI 1.1–109.4, P = 0.04) by logistic regression analysis. The number of children who needed major cardiac reoperation for residual (at least moderate) pulmonary stenosis or aortic regurgitation was 2. No child was in New York Heart Association class >2 or had poor left ventricular function or arrythmias. Only 1 child had an implanted pacemaker, and none needed anticoagulation or other current cardiac medication. The numbers were few for a meaningful statistical analysis.

Logistic regression was done to test the association of the clinical and demographic variables with the faltering HRQOL total score. Clinical and demographic parameters did not show any association with HRQOL total score faltering: viz, type of TGA (with or without VSD) (P-value 0.23), gender (P-value 0.85), socioeconomic scale (P-value 0.63), whether balloon atrial septostomy was done or not (P-value 0.29), age at surgery (P-value 0.19) and need for associated arch or left ventricular outflow tract repair (P-value 0.93). The effects of the above select factors on mean scores of each individual domain were also evaluated. Male children tended to falter more in their treatment anxiety mean scores below the 20th centile than their female counterparts (odds ratio = 1.355, P-value 0.035). None of the other variables showed a significant relationship to faltering scores with respect to heart problem symptoms, treatment compliance, perceived physical appearance, treatment anxiety, cognitive problems, or communication. Multivariable analysis was not done as univariable analysis did not identify any strong predictor of faltering HRQOL total scores.

Specific concerns

In the heart problems and treatment domain, 80 (44.9%) patients reported the concern of ‘Catching Cold Easily’. The response for the more specific cardiac symptoms was negative. Forty children (22.5%) reported an ‘anxiety in receiving medical treatment’. In the cognitive domain, 26 (14.6%) children reported ‘difficulty in paying attention’ and 17 (9.5%) reported a ‘difficulty in remembering what is read’. Two children (2.1%) in the age group (5–12) found trouble with numbers, and 1 child (1.05%) reported to have trouble writing letters or words. Twenty-four (13.5%) reported difficulty in communicating with nurses about how they feel. All these children are being closely followed up for the need for a more detailed neurological or psychological evaluation.

DISCUSSION

In children and adults undergoing congenital heart surgery, HRQOL is affected by multiple factors such as the socioeconomic status, physical status, emotional support available and anxiety levels [10]. Development of strategies for early detection, mitigation or reducing the impact of these factors should lead to better HRQOL in this group of patients. Our study was an attempt at early identification of these factors to initiate intervention strategies as needed.

We used the PedsQL^TM Version 3.0 Cardiac Module parent report for toddlers, young children, and children as the tool for the study because it is validated for cardiac patients and is children specific. The questionnaire is easy to use for the researcher, easy to understand for the child and the care giver, the responses are in simple answers—never, almost never, sometimes, often, almost always; the scoring system ranges from 0–5 and is easily translatable to regional languages. The scoring systems that are often reported for use in CHD patients such as the SF12 and the SF36 or the linear analogue scale assessments are validated for the adolescent and the adult patients respectively and reporting with these cannot be interpreted as accurate for the children in the age group 5–8 as in this study [11].

Within the heterogeneous group of CHDs, children with transposition of great arteries are observed to perform better in terms of QOL [6]. Our study with a near-uniform cohort of children (aged 3–12) with a single diagnosis of complex CHD (Bethesda Class III) correlates with the findings of Uzark et al. [12] who in their mixed diagnosis cohort of predominantly operated children reported mean scores in toddlers (2–4 years) ranging from 79.8 to 90.5 across various domains and ranging from 75.1 to 82.4 in young child (5–7 years). Abassi et al. [13] in their multicentric study of children aged 5–7 operated for varying CHD, report HRQOL values of 73–79 (self-reported–parent reported), with factors such as CHD severity and numbers of surgeries affecting the QOL.

Cleuziou et al. report that anatomical diagnostic factors such as the presence or absence of VSD and coronary anomalies have no bearing on the QOL after ASO. This study reported that patients’ self-assessed and self-reported HRQOL after ASO (using the German version of the Short-Form 36) was particularly good [6]. Factors such as reoperation, pacemaker requirement, and current cardiac medication or anticoagulant use were found to have negative influence on QOL. Our study however had 1 child requiring pacemaker implantation and none requiring anticoagulant usage, and the number of children requiring re-surgeries was few (n = 2) and both reported good HRQOL.

Moons reported that the 356 adults with surgically corrected transposition of great arteries (out of a cohort of 3538 patients) had minimal or no effects on their physical functioning, moderately less for linear analogue scale for QOL but had significantly lower levels in their mental component, anxiety levels and significant numbers had depression and were less satisfied with life [14]. Although the paediatric QOL score doesn’t measure mental health directly, this would be comparable with the values for treatment-related anxiety and perceived physical appearance and we found that at a mean age of 5.6 (SD: 1.2) years, 77.5% of the children following ASO do not exhibit significant symptoms of anxiety with mean scores of 94.1 (range 12.5–100). This parameter warrants follow-up and early intervention as these children grow into teenage. The average score for perceived physical appearance was not affected (98.8).

In a large multicentric study reported by Niemitz et al. [15], there was a recommendation to study parental variables on QOL of children with heart disease. This study found that greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart–lung machine, number of nights spent in a hospital and need for treatment in a paediatric aftercare clinic independently contributed to lower HRQOL. They concluded that beyond medical treatment, integration into school is important to increase HRQOL in children and adolescents surviving with chronic heart disease. Regular screening of HRQOL is recommended to identify patients with special needs. Drakouli et al. [16] found similar associations in a systematic review that reported that patients’ QOL was affected after cardiac surgery with more severe cardiac lesions, with diminished QOL concerning physical, psycho-social, emotional, and school functioning. They found parental support, lower socioeconomic status, limitations due to physical impairment, sense of coherence and finally the level of the child’s everyday anxiety and depression as the most important determinants of children’s QOL.

French data suggested that a sizeable proportion of survivors of ASO had cognitive and psychological difficulties affecting academic success and QOL in adulthood [17]. Data from Germany revealed 14% neurological abnormalities in adulthood after ASO affecting QOL and employment [18]. Multicentric data from North America also echoes the issues regarding employment and presence of symptoms or pacemaker implantation in functional health status survivors of ASO [19]. Our data suggest that though the cognitive impairment in ASO survivors is mild, it is not infrequent and manifests as early as school entry. This highlights the need for continued comprehensive rehabilitation of survivors of complex congenital heart surgery for optimal long-term QOL.

There is evidence that parental psychological well-being has an impact on their children’s psychological adaptation [20]. The current study tool is unable to analyse psychological well-being of the parents and needs further evaluation with a different mode of assessment to establish any causative role. It is our observation that with increasing maternal age, children were less anxious, although this is not found to be statistically significant. Duncan et al. [21] have extensively studied the relationship between maternal age and child development and predict a U-shaped curve with maternal age. It is our observation, from our cohort, that educated mothers are spending more time caring for their children with heart disease. This could be a positive influence on the growth and development of these children. Both psychological well-being and maternal age are factors that need further evaluation with larger cohorts and against controlled population.

Our study has been to evaluate the early effects of parental education and socioeconomic conditions on the QOL of children operated with the arterial switch for Transposition of Great Arteries to identify points of intervention. TGA is a complex CHD and in our own cohort we have identified neuro-developmental issues in 31.1% with 16.4% had speech and language impairment [5]. The learning difficulty is also reflected in our HRQOL scores in this study with 26 (14.6%) children reported ‘Difficulty in paying attention’ and 17 (9.5%) reported a ‘Difficulty in remembering what is read’. While the high HRQOL scores in this uniform cohort of children post arterial switch is encouraging, 2% of children were in classes lower-than-for-age which correlated with faltering HRQOL scores and suggests scope for improvement. In the age group of toddlers (2–4), treatment-related anxiety was the most altered parameter with 93.7 (62.5–100) and next was cognitive impairment with 95.9 (66.7–100). In the age group of young children (5–7), treatment-related anxiety was the most altered parameter with 94.4 (12.5–100), next was Cognitive impairment with 97.04 (58.3–100) and communication problems with 97.2 (66.7–100). Although we did not have many children in the 8- to 12-year age group (n = 4), the scores for these parameters were lower with treatment anxiety at 90.6 (75–100), cognitive impairment at 91.7 (66.7–100) and communication problems at 91.7 (83.3–100). We believe this trend is an indication for a gradual drop in QOL as age advances and highlights the need for close follow-up of these parameters even in children who are not undergoing repeat surgeries or continued medical treatment.

Strengths and limitations

In our study, treatment-related anxiety was a domain which had in comparison lower mean scores and received lower individual values than the other domains. It is our concern that this finding could be because of the age at which our children were analysed when they are wary of hospitals and medical treatment. Or whether it is an early reflection of the trends of anxiety demonstrated by adolescents and young adults in other studies. Since these children have not received detailed psychological evaluation at the time of this study, we are unable to confirm the cause for this trend.

The study is subjective in nature and most parents and children gave positive responses to our questionnaire. Since many have a mean score of 100 in all domains, we have kept a slightly higher cut-off of >90 as cut-off for analysis. With this higher cut-off, we hope to identify more children with problems earlier and initiate intervention.

The children in our cohort were operated at varying ages with some early and some late in the neonatal period with an average age at 44 days. While this is an older cohort than usual to undergo corrective surgery, we find this to be a favourable factor to see how delayed surgery affects the outcomes.

CONCLUSION

In our uniform cohort of toddlers and children who underwent the ASO beyond the early neonatal period, the median total of PedsQL scores in all domains is 97.9 (interquartile range 4.2) at 5.6 ± 1.27 years of life. Treatment-related anxiety was reported by 22.4%, 14.6% reported ‘Difficulty in paying attention’ and 9.5% reported a ‘Difficulty in remembering what is read’ indicating anxiety and cognitive defects. Social factors did not show a statistically significant influence in the age group of children in our cohort but warrant extended monitoring and evaluation. The gradually declining trend across the age groups emphasizes the need for continued follow-up for early identification of possible correctable factors and initiating intervention to ensure good QOL into teenage and adulthood.

ETHICAL STANDARDS

The study was approved by the Institute Ethics Committee and was conducted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its amendments. Written informed consent was obtained from all the patients in the study in English or in their vernacular. Patient detail privacy has been taken care of and identity particulars have not been disclosed.

Conflict of interest: none declared.

DATA AVAILABILITY

The data underlying this article are available in the article and in its online supplementary material.

Author contributions

Sowmya Ramanan: Conceptualization; Data curation; Formal analysis; Investigation; Methodology; Project administration; Resources; Software; Writing—original draft; Writing—review & editing. Arun Gopalakrishnan: Conceptualization; Data curation; Formal analysis; Investigation; Methodology; Project administration; Resources; Software; Writing—original draft; Writing—review & editing. Soumya Sundaram: Conceptualization; Data curation; Formal analysis; Methodology; Project administration; Resources; Software; Writing—original draft; Writing—review & editing. Ravi Prasad Varma: Formal analysis; Methodology; Writing—review & editing. Deepak Gopakumar: Data curation; Methodology; Project administration. Vinitha K. Viswam: Data curation; Methodology; Project administration. Rahul Satheesan: Data curation; Methodology; Project administration. Sudip Dutta Baruah: Data curation; Investigation; Methodology; Writing—review & editing. Sabarinath Menon: Data curation; Investigation; Methodology; Resources; Supervision; Writing—review & editing. Baiju S. Dharan: Conceptualization; Data curation; Formal analysis; Investigation; Methodology; Project administration; Resources; Supervision; Writing—review & editing.

Reviewer information

European Journal of Cardio-Thoracic Surgery thanks René Prêtre, Ali Dodge-Khatami and the other anonymous reviewer(s) for their contribution to the peer review process of this article.

REFERENCES

ABBREVIATIONS

ABBREVIATIONS
 
• ASO

  Arterial Switch Operation

 
• CHD

  Congenital heart disease

 
• HRQOL

  Health-related quality of life

 
• PedsQL

  Paediatric quality of life

 
• QOL

  Quality of life

 
• SD

  Standard deviation

 
• SES

  Socioeconomic status

 
• TGA IVS

  Transposition of great arteries with intact ventricular septum

 
• TGA VSD

  Transposition of great arteries with ventricular septal defect

 
• TGA

  Transposition of great arteries