Quality of life in children with congenital heart disease: An emerging Indian perspective

Congenital heart disease (CHD) is the most common birth defect worldwide. In India, the estimated incidence of 8–10 per 1000 live births translates to over 200,000 affected newborns annually.[1] With significant advancements in pediatric cardiac care – including early diagnosis, surgical techniques, and intensive care – mortality rates have declined substantially. However, except for a straightforward patent ductus arteriosus ligation or device closure, virtually all CHDs carry some degree of long-term morbidity, however minor, necessitating regular medical contact. As survival improves, the focus shifts from mere survival to the broader concepts of neurodevelopmental outcome and quality of life (QoL), not just of the patient but also of their caregivers.

The Western world has been incorporating QoL metrics in pediatric CHD research for over three decades. One of the earliest landmark studies by Uzark and Jones explored parental perspectives on QoL in children following CHD surgery, demonstrating lower scores in several domains compared to healthy peers.[2] Subsequent studies stratified QoL based on lesion complexity, type of repair, and developmental stage, consistently finding that children with complex or cyanotic CHD, particularly those requiring multiple interventions, experience poorer QoL, especially in physical, emotional, and school functioning.[3,4]

From the 2010s onward, QoL research in pediatric CHD has grown more sophisticated, incorporating longitudinal designs, psychosocial predictors, and culturally adapted tools. Marino et al. developed the Pediatric Cardiac Quality of Life Inventory, a disease-specific instrument tailored for children with CHD.[5] A systematic review by Drakouli et al. identified multiple noncardiac factors that influence QoL, independent of lesion severity. These include (a) parental support, (b) lower socioeconomic status, (c) physical limitations, (d) the child’s sense of coherence, and (e) underlying anxiety and depression.[6] More recently, multicenter studies have investigated modifiable intensive care and surgical risk factors that contribute to poor long-term QoL in specific CHD subgroups.[7]

Despite these global advances, Indian CHD research remains largely focused on mortality and short-term procedural outcomes, with limited attention to psychosocial and developmental consequences.[8,9] Even studies on long-term comparative studies on functional capacity are limited.[10] A small number of studies from the Indian subcontinent have addressed QoL in unoperated children with CHD and found low scores across all age groups.[11,12] This disparity, though understandable, reflects a transitional phase in pediatric cardiac care.[13] As the saying goes, “When one is hungry, they think of food—not the presentation.” However, once survival becomes the norm, attention must shift to the lived experience of children with CHD.

In this issue of the Annals of Pediatric Cardiology, several contributions highlight this neglected yet critical dimension. Most notably, Maity et al. present one of the first comprehensive Indian studies evaluating both health-related physical fitness (HRPF) and QoL in children and adolescents with CHD, 1 year postintervention.[14] Their findings are both encouraging and sobering: while HRPF and QoL improved significantly following intervention, CHD patients continued to report significantly lower scores than healthy peers – especially in psychosocial well-being and school participation.

These findings mirror global data. For instance, Aguiar et al. studied Portuguese children with repaired tetralogy of Fallot and found that even after successful surgical repair, QoL scores remained suboptimal, particularly in emotional and physical domains.[15] Participation in extracurricular sports was a strong independent predictor of better QoL. A systematic review by Avedissian et al. further emphasized that overprotective parenting, while well-intentioned, may hinder motor development, autonomy, and emotional adjustment in children with CHD.[16]

Children with CHD face a complex array of challenges – including genetic syndromes, repeated surgeries or interventions, device implantations, lifelong medications, and organ-specific comorbidities. These are often compounded by psychosocial barriers such as parental anxiety, peer exclusion, school absenteeism, and reduced independence. Unfortunately, such issues frequently go unaddressed in routine cardiology follow-up.

While adult research increasingly includes QoL as a core outcome, pediatric data – particularly from India – remain limited. There is an urgent need for multicentric, longitudinal studies using validated, culturally appropriate tools to map QoL trajectories from infancy through adolescence. Such evidence is vital for informing clinical decision-making, individualized care strategies, and advocacy for school integration, physical activity promotion, and mental health support.

FUTURE CHALLENGES IN INDIA: LIFE COURSE OUTCOMES

As survival rates for CHD continue to improve in India, attention must extend beyond childhood QoL to long-term life course outcomes. Research from the West and opinions for India[17] consistently demonstrate disadvantages in employability among grown-ups with congenital heart disease (GUCH). Kamphuis et al. showed that only about 60% of GUCH patients had paid employment, significantly less than the general population, with educational attainment and disease severity being key determinants.[18] Crossland et al. reported that career advice was often inadequate, leaving many patients uncertain about appropriate job options.[19] More recently, Enomoto et al. demonstrated similar patterns in Japan, where complex CHD, frequent hospital visits, and functional limitations were independent predictors of unemployment.[20] In India, although systematic studies are lacking, anecdotal experience suggests that parallel barriers – such as exclusion from government services (armed forces, aviation, and railways) and frequent absenteeism for follow-ups – likely contribute to limited employability.[17] Lack of insurance coverage of CHDs and lifetime out-of-pocket expenses further compounds the problem in the Indian setup.[21]

The social burden of CHD has also been well described. Zomer et al., using a Dutch nationwide registry, highlighted that adults with CHD are more likely to be unmarried, live with parents longer, and report psychosocial difficulties compared to controls.[22] Kovacs et al. further reviewed the psychosocial dimensions of adult CHD, pointing to persistent stigma, overprotection, and impaired social adjustment.[23] Moons et al., in a multi-country study, demonstrated that patient-reported outcomes vary substantially depending on socioeconomic and healthcare system factors, underscoring the role of cultural and structural determinants.[24] In India, where societal myths around “weak hearts” persist and psychosocial services are sparse, these challenges are often magnified, leading to reduced independence and limited peer integration.

Marriage and family life constitute particularly sensitive areas. Registry data from Europe and North America suggest that adults with CHD are less likely to marry or have children than their peers, with barriers more pronounced in women due to concerns around maternal risk and genetic transmission.[24,25] Engelfriet and Mulder highlighted gender-specific challenges in GUCH, with women facing additional restrictions related to pregnancy and reproductive health.[25] These findings resonate strongly with the Indian experience, where parents frequently voice concerns about the “marriageability” of daughters with CHD, irrespective of surgical repair or functional status. Even the fear of scar and marriageability were among the prominent reasons for girls, even in infancy, not getting their due cardiac surgery in India.[26]

Taken together, these insights underscore the urgent need for longitudinal Indian studies that move beyond survival and childhood QoL to evaluate employability, social integration, and marital outcomes systematically. Innovation in the field of pediatric cardiology should not only focus on cost-cutting[27] but also on measures to improve the lifetime QoL of children across diseases cost-effectively. As India’s CHD survivors transition into adulthood, addressing these life course challenges will be essential for achieving truly holistic, patient-centered care.

“Better late than never.” – George Herbert, Jacula Prudentum (1651)