Patient characteristics

Socio-economic hardship and lack of basic amenities available in high-income countries were common across all of our settings. Many of our patients experienced substantial loss of income since diagnosis likely mediated by long periods of school absence precluding ongoing parental employment, costs of medical care, and associated travel and medications. It must be stressed that without the surgical mission, it is unlikely that many of our patients would have undergone corrective surgery and equally unlikely that their quality-of-life living with an uncorrected lesion could be studied.

The vast majority of our patients had mild or moderate complexity congenital lesions.^{Reference Warnes, Williams and Bashore9} A small minority of patients had acquired lesions, mainly mitral valve rheumatic lesions, while only one patient had single ventricle physiology. In our settings, most patients born with complex lesions die prior to surgical workup often due to late presentation.^{Reference Mocumbi, Lameira, Yaksh, Paul, Ferreira and Sidi10} Anecdotally, some patients with complex lesions are transferred from remote healthcare-restricted settings to tertiary hospitals in the same country or even overseas surgical centres for self-funded or more rarely compassionate surgery.^{Reference Ramnarayan, Intikhab, Spenceley, Iliopoulos, Duff and Millar11}

Comparison to previous studies

The cross-sectional study by Raj et al in South India is to our knowledge the only large quality-of-life study in children with uncorrected cardiac defects.^{Reference Raj, Suhakar and Roy4} Despite more complex lesions in their cohort, mean emotional functioning scores were similar to our own.

Other previous studies included only patients with corrected/palliated defects or those with mild lesions that did not require corrective surgery (Table 4), and direct comparison is therefore challenging. This said, our mean emotional functioning scores were similar to those of patients with corrected defects in Pakistan,^{Reference Ladak, Hasan, Gullick, Awais, Abdullah and Gallagher18} higher than those of palliated single ventricle patients in two high-income countries,^{Reference Eagleson, Justo, Ware, Johnson and Boyle15,Reference Mellion, Uzark and Cassedy16} and lower than those of healthy control groups in both low-middle^{Reference Raj, Suhakar and Roy4} and high-income countries.^{Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni12,Reference Kwon, Mussatto, Simpson, Brosig, Nugent and Samyn14,Reference Mellion, Uzark and Cassedy16,Reference Uzark, Zak and Shrader17}

Table 4. Summary of previous paediatric heart disease studies which reported on emotional functioning score of PedsQL TM 4.0 Generic Core Scale

^a Warnes CA, Liberthson R, Danielson GK, et al Bethesda Conference Report Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001; 37: 1170–1175.

Established predictors of poorer emotional quality-of-life in patients with corrected or palliated defects or those with mild lesions that did not require corrective surgery include: low family income,^{Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni12,Reference Uzark, Zak and Shrader17,Reference Drakouli, Petsios, Giannakopoulou, Patiraki, Voutoufianaki and Matziou19–Reference Xiang, Su and Liu22} short duration of parental education,^{Reference Niemitz, Gunst and Hovels-Gurich23} single parent status,^{Reference Goldbeck and Melches24} ethnic minority status,^{Reference Wang25} absence of spirituality,^{Reference Wang25} at least one regular symptom,^{Reference Drakouli, Petsios, Giannakopoulou, Patiraki, Voutoufianaki and Matziou19,Reference Bertoletti, Marx, Hattge and Pellanda26} subjective exercise limitation,^{Reference Marino, Cassedy and Brown27} NYHA class,^{Reference Niemitz, Gunst and Hovels-Gurich23} ROSS Class,^{Reference Ong, Teh, Darshinee, Omar and Ang28} objective exercise capacity,^{Reference Drakouli, Petsios, Giannakopoulou, Patiraki, Voutoufianaki and Matziou19,Reference Dulfer, Helbing, Duppen and Utens29} specific lesion types,^{Reference Berkes, Varni, Pataki, Kardos, Kemeny and Mogyorosy13,Reference Ladak, Hasan, Gullick, Awais, Abdullah and Gallagher18,Reference Idorn, Jensen, Juul and Overgaard30–Reference Kahr, Radke, Orwat, Baumgartner and Diller32} lesion complexity,^{Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni12,Reference Berkes, Varni, Pataki, Kardos, Kemeny and Mogyorosy13,Reference Mellion, Uzark and Cassedy16,Reference Ernst, Marino and Cassedy33–Reference Landolt, Valsangiacomo Buechel and Latal37} previous surgeries,^{Reference Svensson, Idvall, Nilsson and Liuba38} number of clinic appointments,^{Reference Marino, Cassedy and Brown27,Reference Ernst, Marino and Cassedy33} school absence,^{Reference Niemitz, Gunst and Hovels-Gurich23,Reference Knowles, Day, Wade, Bull, Wren and Dezateux39} and medication burden.^{Reference Ladak, Hasan, Gullick, Awais, Abdullah and Gallagher18,Reference Marino, Cassedy and Brown27,Reference Ernst, Marino and Cassedy33,Reference Knowles, Day, Wade, Bull, Wren and Dezateux39}

Our trend towards poorer emotional quality-of-life in adolescents (though not statistically significant) was in contrast to the findings of Raj et al who found that 8–12 year-olds report poorest emotional quality-of-life relative to controls.^{Reference Raj, Suhakar and Roy4} It was also in contrast to studies in patients with corrected lesions which found younger children to report poorest emotional quality-of-life.^{Reference Berkes, Varni, Pataki, Kardos, Kemeny and Mogyorosy13,Reference Mellion, Uzark and Cassedy16,Reference Idorn, Jensen, Juul and Overgaard30,Reference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle40} Our findings may relate to the societal role of adolescents in many of our settings where there is often an expectation to engage in manual labour to contribute to household income or to support a family business, though we did not formally record rates of this practice.

Our finding that having three or more siblings was independently associated with better emotional quality-of-life might be explained by a pseudo-parental role played by older siblings as opposed to younger siblings who might compete for parental attention. Prior studies in both CHD^{Reference Manlhiot, Knezevich, Radojewski, Cullen-Dean, Williams and McCrindle40,Reference Im, Yun and Lee41} and other childhood chronic diseases^{Reference Inati, Sleiman, Akiki, Lteif, Kahale and Abbas42,Reference Ozyazicioglu, Avdal and Saglam43} are divided on the effect of siblings on emotional quality-of-life.

Our finding that a history of parental chronic morbidity or death was associated with poorer emotional quality-of-life is difficult to interpret in the absence of a control arm, although it is conceivable that past experience of parental illness may inform a child’s experience of their own disease.

Multiple previous studies have demonstrated that girls with heart disease report lower emotional functioning scores than boys,^{Reference Ladak, Hasan, Gullick, Awais, Abdullah and Gallagher18,Reference Ernst, Marino and Cassedy33} but we were unable to replicate this finding. Girls with heart disease are less likely to display disruptive behaviours than boys,^{Reference Spijkeboer, Utens, Bogers, Verhulst and Helbing31} and therefore problems that do arise may present late or be missed entirely. There may also be a cultural tendency in some settings to be more protective of girls resulting in overly cautious activity restriction.^{Reference Ladak, Hasan, Gullick, Awais, Abdullah and Gallagher18}

Aside from corrective surgery, regular exercise is the only evidence-based intervention to improve emotional quality-of-life.^{Reference Dean, Gillespie and Greene44} Our rates of patient-reported limitation in sports participation were similar to those of a large cohort in the US.^{Reference Dean, Gillespie and Greene44} Though we found that patient-reported limitation in sports participation was independently associated with symptom burden, we suspect that some of our patients reported limitation because of externally imposed restriction. While previous studies in high-income countries have shown that restriction is in most cases doctor-initiated, in a minority it is parent- or school-initiated and often inappropriate.^{Reference Dean, Gillespie and Greene44} We feel that inappropriate restriction is a particular risk in our settings as lack of regular clinic appointments and access to evidence-based exercise recommendations may result in patients following advice that is no longer age-appropriate, is parent- or school-initiated, and is often overly cautious.

Study limitations

Our study was retrospective, had small numbers, and was cross-sectional. Longitudinal data including data on long-term post-operative emotional quality-of-life would have been highly challenging to collect as our patients commonly changed their address and phone number prior to 1-year follow-up. The lack of sibling or age/sex-matched control arm and the absence of any available data in the literature on population norms in our settings was also a limitation.

Because we conducted the interview simultaneously with other components of the pre-operative workup, in some cases both patient and parent/proxy were present and parent/proxy input may have influenced patient scores^{Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni12,Reference Mellion, Uzark and Cassedy16,Reference Latal, Helfricht, Fischer, Bauersfeld and Landolt20,Reference Ong, Teh, Darshinee, Omar and Ang28} particularly in the adolescent age group.^{Reference Uzark, Jones, Slusher, Limbers, Burwinkle and Varni12} In some settings, use of more than one local language/dialect presented translation difficulties which could have influenced interpretation of questions.^{Reference Ladak, Hasan, Gullick and Gallagher45}

Missing data affected clinical variables in particular which decreased our numbers and introduced a potential sampling bias. Low numbers for clinical variables also increased the risk of type II error in the univariate analysis, and we suspect that with larger numbers we would have found lower emotional functioning scores in patients with severe disease.

Though previous studies have shown that socio-economic factors accounted for two-thirds of the variance in quality-of-life scores,^{Reference Ernst, Marino and Cassedy33} the exclusion of clinical variables from the regression analyses may have affected model accuracy.

Future directions in practice

Short-term surgical missions offer the possibility for novel one-off interventions that may improve emotional quality-of-life. Missions could provide plain-language pamphlets translated into local dialects for families to explain their condition to schools and combat stigma around exercise restriction and surgical scars. Informational leaflets for primary care doctors or community nurses might serve to minimise anxiety-provoking referrals to emergency departments. Social events during the surgical mission could connect families and encourage formation of local support groups. Didactic teaching to local cardiologists and nurses could include modules on up-to-date exercise recommendations.

One-off parental workshops with components of problem prevention therapy, psychoeducation, and parenting skills have been shown to improve maternal mental health and decrease number of days missed from school in CHD^{Reference McCusker, Doherty and Molloy46} and could be replicated in these settings with the aid of local translators. Supervised exercise testing can be used therapeutically to demonstrate to patient and parent that vigorous exercise can be performed successfully and safely thus combating exercise aversion.^{Reference McCusker, Doherty and Molloy46}

Long-term psychotherapy may be required but access to child psychology and mental health services varies greatly between settings. Nepal has only one outpatient child and adolescent mental health clinic, one trained adolescent psychiatrist, no post-graduate specialist training programme and few clinical psychologists specialising in paediatrics.^{Reference Chaulagain, Kunwar, Watts, Guerrero and Skokauskas47} In contrast, Peru is currently undergoing wide-scale expansion of community-based child and adolescent mental health services in remote areas.^{Reference Toyama, Castillo and Galea48}

Future directions in research

Models for conducting research during surgical missions need to be refined.^{Reference Murala, Karl and Pezzella49} Collaboration between different charitable organisations would increase patient numbers and potentially answer key questions, and data collected could be incorporated into existing international registries.⁵⁰ Primary screening programmes could be used to recruit local controls greatly improving study quality.

Longitudinal projects require “buy-in” from the local team which can be a challenge. Local ownership over research projects could be improved by: (a) establishing a single local contact for the research component of a mission as distinct from the clinical component, (b) one-on-one delivery of educational modules during the mission teaching basic descriptive research methodology, (c) short-term research fellowships for the local team member to work on the project at an overseas institution, (d) being listed as an author on a poster or journal article.

Future studies using specialised psychiatric screening tools could usefully define the burden of psychiatric morbidity in these settings and objective exercise testing could discern between externally imposed exercise restriction versus true limitation.