Abstract

Background –Rheumatic heart disease (RHD) is one of the most frequent causes of acquired heart disease in children. It exerts negative impact on the health-related quality of life (HRQOL) of patients and their parents. The aim of this study was to assess HRQOL of children with Rheumatic heart disease at Tikur Anbessa Specialized hospital (TASH), Addis Ababa, Ethiopia. Methods – Facility based, cross-sectional study was conducted in the Pediatrics Cardiac Clinic at TASH from November, 2023 to April, 2024. The study included 242 children with RHD. Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales and 3.0 Cardiac Module was used to collect data. SPSS version 25 was used for data entry and analysis. The numerical variables were expressed as the means and standard deviation (SD). Ordinal logistic regression analysis was used to assess the relation between the outcome and predictor variable at a significance level of p<0.05. Results – The overall health related quality of life in children with RHD was high, moderate and poor in 40.5%, 45% and 14.5% respectively. Age, number of medications used and number of admissions had statistically significant association with HRQOL. The odds of having a higher HRQOL level reduced by a factor of 0.68 for each year increase in age; with each additional medication used the likelihood of having higher HRQOL level increased by approximately 58%; for each additional hospital admission, the likelihood of having a higher HRQOL level increased by a factor of approximately 62%. Conclusions – HRQOL was affected moderately in the majority of children with RHD in Tikur Anbessa specialized hospital. Factors significantly associated with health related quality of life were age, number of medications used and number of admissions.

1. Introduction

Rheumatic involvement of the cardiac valves is the most important sequela of acute rheumatic fever (ARF) and also the second most common major manifestation after arthritis ¹. Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world ². RHD is currently estimated to affect approximately 40 million people worldwide, the majority of whom are children and young adults in sub-Saharan Africa. About 395,000 children are thought to die from RHD annually, largely contributing to premature cardiovascular disease mortality, with an average age at death of 28 years. Women of childbearing age suffer the highest burden of RHD ³.

In Ethiopia cross-sectional echocardiographic screening of RHD in school children aged 6–18 years from 28 randomly selected primary and secondary schools found in six different geographic regions done and showed a prevalence of 19 cases per 1000 school children ⁴.

Health-related quality of life (HRQΟL) is а quality of life term that takes into account the impact an illness has on an individual’s quality of life (QΟL). Although ΗRQΟL is suggested to be a subjective patient-reported outcome (PRO), many of the current instruments used to measure ΗRQΟL for cancer survivors continue to adopt а biomedical clinical-centered approach ^{5, 6, 7, 8, 9, 10, 11, 12, 13, 14}.

Congenital or acquired cardiovascular diseases exert negative impacts on the health-related quality of life (HRQOL) of patients and their relatives. Advances in the clinical and surgical treatment of congenital and acquired heart diseases have contributed to a significant reduction in mortality rates in recent decades, thus increasing patients’ life expectancies. However, in spite of the increased survival associated with advances in medical and surgical care, problems such as organ dysfunction, psychosocial disorders, and effects on neurological development might still occur, limiting patients’ cognitive development and their productivity in adult life. Therefore, one of the aims pursued by health care providers and promoters is to increase the scores on the physical, emotional, social, and school-related dimensions ¹⁵. In addition to efforts at improving survival, in order to improve their life chances, we need to better understand how the social and emotional outcomes of young people with a chronic disease can be improved, and better support young people's emerging capacity for self-management ¹⁶.

Another descriptive cross-sectional study done on parents of 133 patients with rheumatic fever aged between 5 and 18 years showed the most common manifestation of the disease was articular symptoms associated with cardiac problems, present in 74 cases (56.1%). Participants had higher scores on the following concepts of the questionnaire: physical functioning, role/social-physical; role/social-emotional/ behavioral; bodily pain; and family activities. The items with the lowest scores were: family cohesion; general health; global behavior; and parental impact-emotional. Girls had higher scores on: self-esteem; role/social-emotional/behavioral; and general health. Patients belonging to middle-income families had higher scores on: mental health; physical functioning; role/social-physical; and family activities. Children from the lowest social class had higher scores on bodily pain and psychosocial aspects ¹⁷.

Descriptive cross-sectional study was done in primary school children (aged 6-12 years) with rheumatic fever in the outpatient medical clinics of Tanta city, Egypt. Rheumatic fever affects dietary habits of the majority (82.9% and 87.8% respectively) of children less than 10 years. More than half (58.6%) of children that their duration of illness ˂15 months had a school absenteeism ˂ 3 days/month, furthermore the majority of them (87.9%) had good academic performance. Two thirds of the studied children didn't participate in school activities, more than half of them didn't participate due to their frequent absenteeism from school ¹⁸.

A study done Uganda on HRQOL in children with RHD revealed that children with RHD had significantly lower HRQOL compared to healthy children. Specifically, 52.4% of children experienced impaired physical health, and 66.7% faced issues in emotional well-being, primarily due to anxiety and fatigue. The study found that 40% of children had difficulty with daily activities such as walking or participating in school activities. These findings emphasized the substantial emotional and physical burdens faced by these children ¹⁹.

In Tanzania, HRQOL in children with RHD was assessed and reported that 35% of children had poor school functioning, and 33% reported problems with physical health. Additionally, 40% of children reported experiencing frequent fatigue and pain, both of which were linked to lower scores in physical and emotional health domains. The study underlined the significant impact of the disease on daily life, with a particular emphasis on school absenteeism and participation in physical activities ²⁰.

In a study conducted in India, HRQOL in children with RHD was found to be significantly lower than in healthy peers. The study reported that 39% of the children had poor physical functioning, and 31.8% showed moderate physical functioning. 71.5% of children had poor emotional functioning, which was the most impacted domain. Social functioning remained relatively preserved. Severity of disease and hospital admissions were significantly associated with poorer HRQOL scores ²¹.

Descriptive cross-sectional study done to assess health related quality of life in adult patient with rheumatic heart disease in Namibia showed the mean QALY significantly improved from 0.773 pre-diagnosis to 0.942 after diagnosis. Treatment and surgery can improve HRQOL substantially among RHD patients. Being diagnosed with RHD affects patients living in socioeconomically disadvantaged rural areas through cost and time for healthcare visits ²².

Identifying the level and gaps in the health-related quality of life (HRQOL) of patients with rheumatic heart disease (RHD) is crucial for informing efforts to improve quality of care. Beyond medical or surgical treatment, evaluating HRQOL is essential, as it constitutes a major component of care for chronic illnesses like RHD. However, few studies have assessed the quality of life in children with RHD, particularly in sub-Saharan Africa, and existing studies consistently show that HRQOL is significantly compromised in these patients. Therefore, the aim of this study is to assess the health-related quality of life and its associated factors among children and adolescents with RHD attending follow-up at the pediatrics cardiac clinic of Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia.

2. Materials and Methods

An institution-based, cross-sectional study was conducted at the Pediatric Cardiac Clinic of Tikur Anbessa Specialized Hospital (TASH) from November 2023 to April 2024. TASH is the largest, pioneer tertiary-level teaching and comprehensive specialized hospital in Ethiopia. The Pediatric Cardiac Clinic is one of its specialized units, providing evaluation and treatment services for children with heart disease referred from all regions of Ethiopia. Approximately 1,200 children with rheumatic heart disease (RHD) visit the clinic annually.

All children between the ages of 5 and 14 who were diagnosed and/or on follow up for rheumatic heart disease at TASH pediatric cardiac clinic during data collection period and whose parents or legal guardians agree to participate in the study were included. The exclusion criteria were rheumatic heart disease patients with other chronic condition or co morbidities like HIV (human immunodeficiency virus), diabetes and hypertension; Patients who were in critical condition in need of immediate emergency care and; Patients who underwent valve replacement surgery or cardiac intervention.

Health Related Quality of Life was taken as a dependent variable. The independent variables were age, sex, place of residence, duration of illness, number of medications, number of hospital admissions, education level of child, maternal and paternal education level, and parent income.

The sample size for this study was determined using both single and double population proportion formulas. For the first objective, a sample size of 238 participants was calculated using the single population proportion formula: n=(Z_α/2)²×p×q/d² where Z_α/2=1.96 (for 95% confidence level), p=0.5 (due to the absence of similar studies at this hospital), q=1−p, and d=0.05 (margin of error). Since the source population was fewer than 10,000, a finite population correction was applied, along with a 10% adjustment for potential non-response. For the second objective, the sample size was determined using the double population proportion formula, based on significantly associated variables identified in a previous study ²³. Assuming a 95% confidence interval and 80% power, the calculated sample size was 242 participants. The sample size calculated for the second objective was greater than sample size calculated for first objective. Therefore, a sample size of 242 was used for the study. Participants were selected using a systematic random sampling technique, in which every other child attending the clinic was included until the required sample size was reached.

A standardized interviewer-administered questionnaire was used to collect the data. HRQOL was assessed by using Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales and 3.0 Cardiac Module. It was a validated multidimensional assessment tool for children aged 2 to 18 years. It encompassed 23 items that assesses physical (8 items), emotional (5 items), social (5 items), and school functioning (5 items). The 3.0 cardiac module is a disease-specific module and it has 6 subscales evaluating HRQOL related to heart problems and treatment (7 items), treatment barriers (3 items), physical appearance (3 items), treatment anxiety (4 items), cognition (5 items), and communication (3 items) ²².

The parents or legal guardians of the children were interviewed in this study. Data collectors were trained, oriented and supervised. The data was checked for completeness and consistency on daily basis. The acquired data was examined for accuracy and consistency before being cleaned, coded, entered, and analyzed using the Statistical Package for Social Sciences (SPSS) version 25.0. Descriptive analysis was employed. The numerical variable was expressed as the means and standard deviation (SD). We converted the domain score to a linear scale between 0 and 100. Adhering to the scoring criteria, a mean score of less than 50% in each dimension denotes poor quality, between 50% and 75% suggests moderate quality, and greater than 75% denoted high quality of life. Bivariable and multivariable ordinal logistic regression analysis was used to assess the relation between the outcome variable and the predictor variable. Variables with P-value of less than 0.2 on univariate analysis were selected for multivariable ordinal logistic regression, where variables with p<0.05 were considered statistically significant.

• Quality of life - An individuals’ perceptions of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns which is divided in to three using tool derived from Pediatric Quality of Life Inventory (PedsQL) ²².

√ High level of quality of life >75%

√ Moderate level of quality of life 50-75%

√ Poor level of quality of life <50%

• Adolescent - any person between ages 10-19 years

• Adequate adherence to benzathine penicillin - defined for each patient as receiving >80% of injections on time per year ²³.

Ethical clearance was obtained from Addis Continental Institute of Public Health ethical committee. A support letter was written, which was approved, to Addis Ababa University, College of Health Sciences, Tikur Anbessa Specialized Hospital, department of pediatrics and child health research ethics committee.

Participants got an information sheet outlining the study's objectives and a written consent and assent (for children who are 7-14 years of age) forms. Only participants who consented to participate in the study were asked to do so. The participants had the right to refuse study and their refusal did not interfere with their routine medical care. Confidentiality was assured by collecting data anonymously using only medical record number of the patient and these medical record numbers will not be presented in the study. Answers were kept confidential and not shared with others who was not members of the research team.

3. Results

The mean ± SD age of study participants was 12.5 ± 2.2 and more than half (56.6%) of children were females. One third (34.5%) of children’s mother/care giver had educational status of primary school while one fourth (27.3%) of them had no formal education. The majority 90.9% of children were primary school students and 13(5.4%) had no formal education, as shown in Table 1.

Majority (59.9%) of the studied children with RHD had disease duration of 1 to 5 years. The mean ± SD age at onset of the disease was 8.07 ± 2.5. Most (94.2%) of study participants had regular follow ups and the frequency of follow up in more than half (62.6%) of the children was between 1 and 3 months. The mean ± SD of number medications used were 2.7 ± 1.2. One forth (24.8%) of them had history of drug discontinuation. Most (90.1%) of the children were on monthly benzathine penicillin, as shown in Table 2.

The overall HRQOL in children with RHD was 40.5% high, 45% moderate and 14.5% poor. Most of the study participants had poor physical (39.3%) and emotional functioning (71.5%). In contrast, most of the children had high social (47.9%) and school functioning (52.8%). Only few of the study participants had poor perceived physical appearance (14.9%) and also few had treatment anxiety (9.5%). Majority of children did not have cognitive problems (64.4%) as well as communication problems (46.7%), as shown in Table 3.

In bivariable ordinal logistic regression, each independent variable was analyzed separately to determine its association with the dependent variable, without considering the effects of other variables. Nine variables were selected from bivariable ordinal logistic regression at P-value of less than 0.2 for multivariable ordinal logistic regression.

In multivariable ordinal logistic regression analysis, few independent variables such as age, number of medications used and number of admissions had statistically significant association with HRQOL at P–value of less than 0.05. The odds of having a higher HRQOL reduced by a factor of 0.68 for each year increase in age (Adjusted odds Ratio (AOR) = 0.68, 95% CI: 0.58-0.79). The odds of having a higher HRQOL increased by a factor of 1.58 for each additional medication used (AOR=1.58, 95% CI: 1.24 -2.03). The odds of having a higher HRQOL increased by a factor of 1.62 for each additional admission (AOR=1.62, 95% CI: 1.28- 2.04), as shown in Table 4.

4. Discussion

In our study, health-related quality of life among children with rheumatic heart disease was found to be poor in 14.5%, moderate in 45%, and high in 40.5%. Physical functioning was poor in 39.3% of participants, emotional functioning was severely affected (71.5% poor), while school and social functioning was relatively better with 52.8% and 47.9% achieving high scores, respectively. Increasing age was negatively associated with HRQOL, while a higher number of medications and hospital admissions were positively associated.

The mean ± SD age of study participants was 12.5 ± 2.2 years which is comparable with study done in Ethiopia which showed mean ± SD 13.2 ± 3.2years ⁴. Majority (56.6%) of our study participants were female which is consistent with study done in adult patients in Ethiopia that showed females contributed to 71% of the participants ²³. This observed female predominance might be due to predisposition to autoimmune disease like RHD and delayed or inadequate access to healthcare which may be attributed to socio-cultural factors.

Our finding of poor emotional functioning is consistent with studies conducted in India, Uganda, and Brazil, which similarly reported that RHD has the greatest impact on the emotional well-being of affected children ^{15, 17, 19, 21}. The chronic nature of RHD, frequent hospital visits, physical limitations, and the fear of disease progression likely contribute to heightened anxiety, sadness, and emotional distress in these children. Additionally, societal stigma and feelings of being different from peers may exacerbate emotional struggles. Poor physical functioning observed in our study is also in line with these previous reports ^{15, 17, 19, 21}, reflecting the burden of symptoms such as fatigue, dyspnea, and exercise intolerance commonly associated with RHD. These limitations restrict children’s participation in normal physical activities, reinforcing a cycle of physical deconditioning and reduced independence.

Despite these challenges, only 16.9% of children in our study had poor social functioning, which is comparable to the better mean social functioning scores reported in India, Uganda, and Brazil ^{15, 17, 19, 21}. This suggests that many children, even while coping with a serious chronic disease, are able to maintain social connections and peer relationships. However, variations in social functioning can still occur depending on the severity of the disease, the level of family and community support, and access to educational accommodations and healthcare services.

School functioning (52.8%) was relatively better compared to other domains in this study. However, a study from Egypt reported a higher good academic performance rate (87.9%) among children with rheumatic fever ¹⁸. This difference may be due to the milder, reversible nature of rheumatic fever compared to the chronic, disabling course of rheumatic heart disease. Frequent hospitalizations, physical limitations, and emotional challenges likely contributed to lower academic performance in our study. Better school accommodations and teacher awareness in Egypt may have also supported improved educational outcomes.

In our study, we found that age was significantly associated with health-related quality of life (HRQOL), with older children showing a lower likelihood of experiencing a higher HRQOL. This finding aligns with studies conducted in India and Uganda, where older children with RHD reported poorer HRQOL ^{19, 21, 26}. Several factors may contribute to this trend. As children age, chronic valvular damage tends to worsen, leading to increased symptoms and greater physical limitations. Additionally, older children are more cognizant of their condition and its long-term implications, which can result in heightened anxiety, depression, and stress about their future health, lifestyle restrictions, and social acceptance. The increased educational demands at older ages also contribute to academic challenges, as frequent medical appointments and physical limitations may lead to missed school days and diminished academic performance.

With each additional medication used, the likelihood of having a higher HRQOL increased by approximately 58% which is in opposite to study done in adults in Ethiopia ²³. This may be due to comorbidity in adults which burdens them to take more medications, natural progress of the disease and tool used. The association between additional medication and higher health related quality of in children with RHD could be due to prevention of disease progression and reduction of complications.

For each additional hospital admission, the likelihood of having higher HRQOL increased by a factor of approximately 62%. Hospital admission allow for early detection and timely treatment of complications that might lead to disease progression. Comprehensive and early care during hospital admission reduces frequent admissions and contributes to improved health outcome and quality of life.

The limitations of this study include the failure to assess all potential factors that could influence the quality of life of children with rheumatic heart disease. Notably, the clinical severity of the illness, a crucial determinant of health-related quality of life, was not evaluated, which may have led to an incomplete understanding of how the severity of the disease impacts the well-being of these children. Additionally, the cross-sectional study design limits the ability to track changes in HRQOL over time, making it difficult to draw conclusions about the long-term progression or fluctuation of the quality of life in these children. Furthermore, HRQOL was assessed using parent-reported questionnaires, which, while valuable, are inherently subject to bias, as parents' perceptions may not fully reflect the child's own experiences or could be influenced by their own emotional state or expectations. These factors should be taken into consideration when interpreting the findings of the study, and future research could benefit from a more comprehensive assessment, including longitudinal designs and direct child assessments of HRQOL.

5. Conclusion

In conclusion, this study demonstrates that the health-related quality of life of the majority of children with rheumatic heart disease is significantly affected, with emotional functioning being particularly poor. Key factors such as age, number of medications used, and number of hospital admissions were found to be significantly associated with HRQOL, highlighting the complex nature of the disease’s impact on children’s well-being. These findings underscore the need for a shift in focus from solely treating the physical aspects of RHD to also addressing the holistic needs of affected children. To improve HRQOL, healthcare providers and policymakers must adopt a comprehensive approach that encompasses not only the medical treatment of RHD but also the physical, educational, and psychosocial support necessary for these children to thrive. Interventions aimed at optimizing treatment regimens, and providing emotional and educational support could greatly enhance the overall quality of life of children living with RHD.

ACKNOWLEDGEMENT

We would like to recognize the contribution of Addis Continental Institute of Public Health for the success of this study. We would also like to extend our gratitude to Addis Ababa University, College of Health Science, Tikur Anbessa Specialized Hospital, Department of Pediatrics and Child Health where the study was conducted. This study would not have been possible without the participation of the parents and children involved in this study, for whom we are especially grateful.

Competing Interests

The authors declare that they have no competing interests.

Acronyms and Abbreviations

AOR – Adjusted Odds Ratio

ARF – Acute Rheumatic Fever

CI – Confidence Interval

COR – Crude Odds Ratio

GAS – Group A Streptococcus

HRQOL – Health Related Quality of Life

PedsQL – Pediatric Quality of Life Inventory

PRO – Patient-Reported Outcome

QOL – Quality of Life

QALY – Quality Adjusted Life Years

RHD – Rheumatic Heart Disease

SD – Standard Deviation

SPSS – Statistical Package for Social Sciences

TASH – Tikur Anbessa Specialized Hospital

VHD – Valvular Heart Disease

WHO – World Health Organization

References