Health-related quality of life of children who have undergone surgery for transposition of the great arteries in a developing country—the outlook is good but meaningful measurement is challenging

Whilst the incidence of congenital heart disease (CHD) remains relatively stable, accounting for nearly one-third of all major congenital anomalies and resulting in 1.35 million babies being born worldwide with CHD every year [1], survival has dramatically improved over recent decades and there is an expanding population of children and adults living with CHD. A paradigm shift has seen a change in focus from short-term mortality prevention to assessment of long-term morbidity. Assessment of long-term health outcomes is now considered to be an essential component of follow-up [2], an important element of which is measurement of patient-reported outcomes such as health-related quality of life (HRQoL). Within the wider context of overall quality of life, HRQoL is defined as ‘the specific impact of an illness or injury, medical treatment, or health care policy on an individual’s quality of life’ [3], which encompasses an individual’s perception of the impact of their health condition on their physical health status and psychological, social and emotional functioning, in terms of both their ability to function in these areas of functioning and their satisfaction from doing so.

Much has been written about the challenges of assessing HRQoL in children, including the wide age range, changing developmental abilities as children age, identifying who is the most appropriate informant and the role of parent-proxy reporting. Within CHD, the heterogeneity of defects, diversity of medical, interventional and surgical approaches to treatment and spectrum of outcomes further complicate the picture, notwithstanding issues related to culture, ethnicity and socioeconomic status amongst others [4]. Finally, there is the choice of appropriate measures—the gold standard is to use both a generic (to allow for comparison with healthy or other illness populations) and disease-specific (to allow for discrimination between subgroups) measure, with self- and proxy-reporting where feasible, and ideally such measures should be validated in the language and culture of the population being studied. It is perhaps not surprising, therefore, that studies evaluating HRQoL in children with CHD from developing countries are rather less common than similar studies from the developed world.

Addressing the paucity of evidence and tackling some of the challenges outlined above, Ramanan et al. [5] report HRQoL outcomes from a single centre in Kerala, India, of children who underwent an arterial switch operation (ASO) for transposition of the great arteries between 2012 and 2018. Survival outcomes and HRQoL following ASO are generally reported to be excellent but most current knowledge comes from the developed world. As Ramanan et al. highlight, the developing world presents a range of challenges to the delivery of optimal health care, including access to healthcare facilities, lack of resources and socioeconomic factors. In their cross-sectional study conducted over 2 periods in 2019 and 2022, children who had undergone an ASO beyond the neonatal period and were between 3 and 12 years of age were recruited. Of the 208 patients who underwent ASO during 2012–2018, 196 survived to discharge, 13 were subsequently lost to follow-up and 5 were late deaths. Parents of children aged 2–4 and 5–7 years completed the parent-proxy disease-specific PedsQL3.0 cardiac module [6] and children aged 8–12 completed the child version. The questionnaire was originally developed in English but was translated into the local languages by a ‘language expert’ and the study team assessed the translated version for content validity and concurrence (although no details are provided about the translation process or approach to validity assessment). The majority of children were aged 5–7 years and 99% had started formal schooling in age-appropriate classes. Two children had severe neuromotor impairment and 175 (98%) were in New York Heart Association class 1. Overall HRQoL scores were skewed and very high, with a median total score of 97.9 (inter-quartile range 4.2), where scores can range from 0–100 and higher scores indicate better HRQoL. Furthermore, the median score for all of the HRQoL domains (heart problems and symptoms, treatment, perceived physical appearance, treatment-related anxiety, cognitive problems and communication) was 100. Interestingly, in their earlier study with a subgroup of the same cohort [7], the authors reported neurological abnormalities in almost one-third of the patients, including neuro-developmental delay, speech and language impairment and attention-deficit hyperactivity disorder—yet surprisingly these do not seem to be reflected in overall HRQoL scores in the current study.

There are many strengths of the study of Ramanan et al. but it also reflects a number of the challenges of evaluating HRQoL in children. Strengths include the setting, sample size, relatively tight age range and diagnostic homogeneity of the group. Scores are higher than reported in other studies; this may be partly explained by the cohort, all of whom had undergone the same surgery for a condition that generally has good outcomes, but there are other important considerations, such as social desirability bias and response shift (particularly as some of the data were collected soon after COVID-19). The choice of measure is also a potential limitation—is it culturally appropriate for this population? Is it measuring what we think it is measuring? The ceiling effects resulted in little variance in the data and precluded meaningful assessment of the potential role of socioeconomic, demographic or clinical factors or an understanding of who needed additional services and how they should be targeted. The lack of a generic measure of HRQoL further prevented comparison with healthy children, which would have given some important cultural context. HRQoL is subjective and reliance on proxy reporting for the majority of children is a further limitation. Finally, a particularly challenging and poorly addressed issue with HRQoL evaluation in general and which is also true in this study is dealing with missing data due to loss to follow-up and death, and how this should be reflected in reporting HRQoL for a particular patient cohort. Notwithstanding these limitations, this study has shown that the outlook for the majority of children undergoing (and surviving) ASO in Kerala, India, appears good—what we now need to see is what happens as these children grow up and whether these findings are replicated with other cohorts from developing countries.

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