Existing knowledge

There is a sizeable body of evidence supporting the efficacy of interventions which address known neurodevelopmental and psychosocial risk factors in other high-risk populations such as children born preterm and those diagnosed with developmental disabilities. Moreover, theoretical frameworks exist for adapting interventions for use in individuals with various medical conditions. ^{Reference Craig, Dieooe, Macintyre, Michie, Nazareth and Petticrew15,Reference Czajkowski, Powell and Adler16}

Individualised developmental care programmes show particular promise for promoting positive neurodevelopmental outcomes among medically at-risk children when implemented in the newborn period. Individualised developmental care is a model of care that minimises the mismatch between infant neurobiological needs and the often toxic hospital environment. The Newborn Individualized Developmental Care and Assessment Program ^{Reference Butler, Huyler, Kaza and Rachwal17,Reference Als18} is the only evidence-based developmental care programme and is well validated in the preterm infant population where it has been shown to decrease the length of hospital stay and improve physiological functioning, long-term neurodevelopment, parent confidence, and patient and family satisfaction among infants born preterm. ^{Reference Als18-Reference Als, Duffy and McAnulty21} Other programmes, such as Trauma Informed Care and Family Centered Care, incorporate an understanding of trauma and need to recognise the central importance of family into routine care and treatment of illness. ^{Reference Ko, Ford and Kassam-Adams22-Reference Stuber, Schneider, Kassam-Adams, Kazak and Saxe24} Specific aspects of these programmes, such as skin-to-skin contact, interdisciplinary developmental care rounds, cue-based care, family support, and education for providers, have been found to be developmentally supportive of children and families affected by CHD and contribute to improved neurodevelopmental outcomes. ^{Reference Butler, Huyler, Kaza and Rachwal17,Reference Miller, Lisanti and Witte25-Reference Harrison, Chen, Stein, Brown and Heathcock31} The use of breast milk and breastfeeding can also support infant growth and oral feeding, as well as promote bonding with family and improve cognitive development, ^{Reference Brown Belfort32} and social-emotional growth over time. ^{Reference Combs and Marino33,Reference Kramer, Aboud and Mironova34}

In addition to inpatient supports, early intervention has demonstrated positive effects on the developmental achievements of children with or at-risk for developmental disability. ^{Reference Nores and Barnett35-Reference Olds, Kitzman and Cole37} Families from high-risk populations who received prenatal and infancy home visits by nurses showed improved cognitive, academic, behavioural, and sociodemographic outcomes for their children. ^{Reference Eckenrode, Campa and Luckey38}

Interventions targeting areas of deficit commonly observed among individuals with CHD have been developed and well-established with non-CHD populations. As an example, practice guidelines for behavioural and psychotropic interventions for individuals diagnosed with attention-deficit/hyperactivity disorder are established for other populations, yet there has been limited research involving individuals with CHD who have special considerations due to cardiovascular effects of common medications ^{Reference Wolraich, Hagan and Allan39,Reference Vetter, Blum and Berger40} . Similarly, the efficacy of behavioural and psychotropic interventions for individuals with mood ^{Reference David-Ferdon and Kaslow41} and anxiety disorders, ^{Reference Wang, Whiteside and Sim42,Reference Hetrick, McKenzie, Cox, Simmons and Merry43} including procedural anxiety ^{Reference Cohen, Blount, Chorney, Zempsky, Rodrigues, Cousins, Roberts and Steele44} and coping with medical illness, ^{Reference Harbeck-Weber, Fisher, Dittner and Roberts45} has been established, but these interventions have been understudied among individuals with CHD. A 2013 Cochrane review identified no randomised controlled trials demonstrating the efficacy of cognitive-behavioural interventions for depression in adolescents or adults with CHD, and a ^{Reference Lane, Millane and Lip46} more recent review continued to report limited efficacy among these populations. ^{Reference Tesson, Wales and Butow13}

Among healthy school-age children and adolescents, as well as those with various medical conditions, there is a strong interest in addressing neurocognitive deficits (e.g., in executive function, attention), using, for example, computerised interventions such as Cogmed; however, data on the effectiveness of these programmes have been mixed. ^{Reference Melby-Lervåg and Hulme47,Reference Diamond and Ling48} Therapeutic camp programmes have been shown to improve mood, self-concept, empathy, quality of life, and emotional well-being for children with cancer and their families. ^{Reference Martiniuk, Silva, Amylon and Barr49} Adolescents with chronic illness also benefit, in terms of adjustment and well-being, from peer-based support programmes, including programmes that are school-based and disease-specific, as well as those that are community-based. ^{Reference Gomez-Gascon, Martin-Fernandez, Galvez-Herrer, Tapias-Merino, Beamud-Lagos and Mingote-Adan50} Emerging evidence exists for telemedicine and e-Health interventions in improving outcomes for adolescents with chronic medical and psychiatric conditions, including PTSD. ^{Reference Sawyer, Drew, Yeo and Britto51}

Significant gaps in knowledge

Despite decades of research evaluating neurodevelopmental and psychosocial interventions for other medical populations, the safety, feasibility, acceptability, accessibility, efficacy, and effectiveness of these interventions for use in individuals with CHD are largely unknown. Adapting interventions from other populations will require an understanding of the unique characteristics and challenges inherent in CHD, and their relevance to the particular intervention considered. In addition, while continued surveillance and consultation are recommended for children with complex CHD, it is unclear how many are receiving early intervention following discharge and how this impacts long-term development.

Outside the hospital setting, and particularly among school-age children, adolescents, and young adults with CHD, we remain largely uninformed regarding the long-term effectiveness of neurocognitive interventions (e.g., Cogmed), as well as their potential impact on academic and social domains; the effectiveness of specific educational and peer mentorship interventions; the impact of health inequities and barriers that may prevent individuals and families from accessing interventions; how best to engage telemedicine and e-Health, social media, and other technology tools to broaden the reach of interventions beyond the clinic setting; and the short- and longer-range economic implications and cost-effectiveness of intervening to mitigate the host of risks associated with CHD.

Investigations needed

1. (1) Investigate the safety and feasibility of individualised developmental care interventions delivered during a cardiac hospitalisation

Safety and feasibility studies of individualised developmental care interventions in the cardiac ICU are needed to adapt evidence-based programmes to the unique needs of infants with CHD and their families. Smaller-scale quality improvement studies should lead to larger-scale, multi-centre, randomised controlled trials to assess child neurodevelopmental and physiologic outcomes in the newborn period, as well as in early infancy and over the course of the lifespan. Such research could potentially highlight the implications of early-life intervention on later markers of health and well-being, along with improved family outcomes, decreased parent stress, and reduced healthcare utilisation and economic burdens.

1. (2) Examine best practices for promoting handling and moving of infants and young children during cardiac hospitalisation

Concept and safety/feasibility studies are necessary to examine safe practices for handling/moving infants in the inpatient acute cardiac care setting, providing skin-to-skin holding, and increasing parent involvement in care and handling. This should be followed thereafter by quality improvement projects geared at increasing parent/caregiver comfort regarding the full range of developmental care interventions while hospitalised. These endeavours would be strengthened by a team-based, interdisciplinary approach that includes collaborative partnerships among nursing, cardiology/cardiac surgery, physical therapy, occupational therapy, child life and music therapy, nutrition, speech/language pathology, and psychology.

1. (3) Study short- and longer-range outcomes associated with individualised developmental care interventions in an acute cardiac inpatient setting

As mentioned, there are many positive outcomes associated with individualised developmental care intervention, including decreased length of hospitalisation and improved feeding, among children born preterm. ^{Reference Als, Lawhon, Duffy, Mcanulty, Gibes-Grossman and Blickman19-Reference Als, Duffy and McAnulty21,Reference Nores and Barnett35} In the case of infants with CHD, for which length of hospital stay is among the strongest risk factors for adverse outcomes, ^{Reference Hansen, Rotermann and Logoteta52-Reference Newburger, Wypij and Bellinger54} reduced length of hospital stay would lower hospital costs and reduce exposures to potentially noxious elements in the acute inpatient environment (e.g., plasticizers, ^{Reference Gaynor, Ittenbach and Calafat55} loud sounds, bright lights, inadequate protection of sleep, inadequate attention to parent mental health, separation from family, stress reduction, and the use of non-pharmacologic comfort interventions ^{Reference Ryan, Jones and Allen56} ) that may contribute to worse outcomes for these children. Individualised developmental care interventions also advocate for staff support to reduce stress and burnout, which would positively affect the patient and family. Interventions to support growth and weight gain, use of human milk, early breastfeeding, and decreasing time to full oral feeding in patients with CHD ^{Reference Ross and Browne57-Reference Medoff-Cooper, Irving and Marino61} are of utmost importance as oral feeding ranks the greatest stressor for caregivers following cardiac surgery and often lengthens hospital stay. ^{Reference Imms62,Reference Furlong-Dillard, Neary and Marietta63} In addition, there is no current gold standard programme for infant feeding in cardiology, but this should be explored. Programmes that monitor development over time and provide intervention beyond infancy such as early intervention and early supports in the school system would likely reduce concerns seen in adolescence and adulthood.

1. (4) Conduct translational research studying empirically supported psychosocial and neurocognitive interventions developed for other populations in individuals with CHD

Building on existing knowledge of interventions that work in other populations, translational studies are needed to effectively adapt interventions for use among children with CHD. Psychosocial interventions targeting anxiety, mood concerns, and the effectiveness of cognitive-behavioural therapy in addressing these issues will be particularly important given their high prevalence among individuals with CHD. Efficacious interventions developed for individuals with other chronic illnesses, such as cancer and diabetes, that address comorbidities such as pain, adherence to medical regimens, family functioning, transition from paediatric to adult healthcare, and traumatic stress could be adapted to benefit individuals with CHD. ^{Reference Roberts and Steele64} Neurocognitive interventions that address attention, executive function, and visual-spatial deficits are also necessary and should investigate a range of delivery modalities including computerised and in-person formats.

1. (5) Investigate new modalities for delivering neurodevelopmental and psychosocial interventions within the CHD population

Concept, pilot, and quality improvement studies can explore new modalities of intervention delivery, followed thereafter by larger-scale, multi-centre implementation studies. For example, telemedicine for post-surgical developmental follow-up may promote earlier identification and treatment of neurodevelopmental and psychosocial issues. ^{Reference Stuckey and Domingues-Montanari65,Reference Young, Gupta and Palacios66} Randomised controlled trials of these interventions in hospital settings or via telemedicine ^{Reference March, Spence and Donovan67} may establish effectiveness for patients with CHD, reach a larger population, and provide preventative intervention.

Existing knowledge

Preliminary interventions in infants with CHD show improvement in infant oral feeding, ^{Reference Indramohan, Pedigo, Rostoker, Cambare, Grogan and Federman68,Reference Weston, Husain and Curzon69} physiological regulation, ^{Reference Harrison and Brown70} early cognitive development, ^{Reference Harrison and Brown70} family functioning, ^{Reference Harrison and Brown70} and reduced length of hospital stay following surgery. ^{Reference Indramohan, Pedigo, Rostoker, Cambare, Grogan and Federman68,Reference Weston, Husain and Curzon69} Moreover, findings from the Congenital Heart Disease Intervention Project, a series of controlled trials aimed at improving psychosocial and neurodevelopmental outcomes among young children with severe CHD, support the use of parent-oriented psychoeducation for improving infant mental, social, and emotional development at 6 months of age and gains in family functioning and fewer days of missed school among 4–6-year-old children. ^{Reference McCusker, Doherty and Molloy71-Reference Casey, Stewart and McCusker75} However, a similarly designed randomised controlled trial utilising both parent- and child-oriented psychoeducation reported only small, non-significant improvements in child psychosocial adjustment relative to standard care. ^{Reference van der Mheen, Meentken and van Beynum76} For children and adolescents with CHD, computerised interventions are being studied to examine their impact on executive function and social skills. ^{Reference Calderon, Bellinger and Hartigan77} Aerobic exercise has been associated with self- or proxy-reported improvements in cognitive functioning, social outcomes, and health-related quality of life. Recommendations for reducing child anxiety related to invasive cardiac procedures ^{Reference LeRoy, Elixson, O’Brien, Tong, Turpin and Uzark78} have been documented but not clinically tested. Single centre interventions including psychotropic medication, ^{Reference Cassidy, Ilardi and Bowen10} access to a psychologist in clinic, ^{Reference Marino, Lipkin and Newburger11} mindfulness training, ^{Reference Calderon and Bellinger12} and increased physical activity ^{Reference Tesson, Wales and Butow13-Reference Craig, Dieooe, Macintyre, Michie, Nazareth and Petticrew15} have demonstrated reduced symptoms of depression, anxiety, stress, and improved quality of life for adolescents and adults with CHD.

Significant gaps in knowledge

Most prior neurodevelopmental/psychosocial intervention studies are single-centred, cross-sectional, and have not made use of randomised controlled designs which remain the gold standard for clinical trials. Many of these investigations had limited statistical power to detect a meaningful effect, and outcome measurements varied greatly between studies. Further, the efficacy of interventions in adolescence, to date, has been weak, ^{Reference Tesson, Wales and Butow13} and more trials are needed. Finally, many of these intervention studies exclude individuals with CHD with comorbidities such as genetic syndromes, which may substantially impact intervention design, administration, interpretation, and generalisability of findings.

Investigations needed

1. (1) Operationalise clinically meaningful intervention outcomes across development

It is critical to carefully consider outcome measures based on the age and functional status of the child, and any behavioural and emotional constructions of relevance to the intervention. Initially, studies focused on global neurodevelopmental skills such as overall intelligence quotient scores, but as more has been learned about risk, outcomes are being tailored to aspects of neurodevelopment that are more often impaired among a CHD population such as executive function and visual-spatial processing. Standardised measurement protocols to assess neurodevelopmental outcomes, as well as key moderators of intervention efficacy and effectiveness (e.g., SES, language), must be identified and may include formal assessment, structured observational measures, caregiver-/self-report questionnaires, and measures of neurobiological change (i.e., structural or functional variations on neuroimaging). It is important to have consistency across sites to reduce bias that can come from single-centre reporting and promote generalisability of findings. The Cardiac Neurodevelopmental Outcome Collaborative has made recommendations ^{Reference Ware, Butcher and Latal4,Reference Ilardi, Sanz and Cassidy5} for a standardised assessment battery from infancy through teen years, which will help to guide future intervention research when selecting outcome measures to assess the impact of interventions on the neurodevelopment of individuals with CHD. ^{Reference Ware, Butcher and Latal4,Reference Ilardi, Sanz and Cassidy5} Large-scale, multi-centre studies, which will be feasible within the context of the Cardiac Neurodevelopmental Outcome Collaborative data registry, are necessary to allow for adequate clinical stratification and inclusion of potential comorbidities as well as more diverse sociodemographic variables.

1. (2) Conduct prospective randomised controlled trials with longer-term follow-up to investigate efficacy and effectiveness beyond the snapshot of a pre-post intervention

Studies with sequential post-intervention visits, at predetermined time intervals, would provide evidence of cost-effectiveness and potential generalisation of treatment effects in the long term. The number and timing of follow-up should take the developmental period into account with more immediate follow-up during early development and longer-term follow-up of more complex neurodevelopmental skills into adolescence and beyond. Further, efficacy trials (does an intervention work in an ideal setting) should be developed with a mind to effectiveness (does an intervention work in a real-world setting and are they feasible given limitations such as cost).

1. (3) Partner with key stakeholders to define “clinically meaningful” outcomes

Determining what constitutes a clinically meaningful change post-intervention involves more than statistical significance. Indeed, the threshold for clinically meaningful changes pre- versus post-intervention should be interpreted in light of both individual and population-based changes in CHD. ^{Reference Bellinger79} In all interventions research, it will be important to enlist the input of patients, families, and other stakeholders to ensure an accurate understanding of the real-world relevance of selected outcome measures and to consider such an understanding alongside quantitative indicators of change (e.g., effect sizes quantification and use of reliable change index estimates, along with statistical significance). For instance, a 2-point standard score drop on a measure of externalising behaviour may be a statistically significant change but is unlikely to be a noticeable change in real-world behaviour.

Existing knowledge

Early intervention programmes for high-risk populations, such as preterm and/or very low birth weight infants, are well-established and associated with improved neurodevelopmental ^{Reference Cioni, Inguaggiato and Sgandurra80} and psychosocial functioning ^{Reference Case-Smith81} and have demonstrated the positive impact of inpatient neurodevelopmental care ^{Reference Burke82,Reference Westrup83} and outpatient neurodevelopmental follow-up. ^{Reference Spittle, Anderson, Boyd and Doyle84} Networks of newborn follow-up programmes serve as data registries providing programme benchmarks, initiating multi-site quality improvement projects to improve the standard of care, and allow for the development of best practice guidelines. ^{Reference Horbar85} Indeed, the importance of standardised follow-up programmes for former medically fragile neonates is so strongly recognised that it is a requirement for accreditation for graduate medical education in neonatal–perinatal medicine by the Accreditation Council for Graduate Medical Education. ⁸⁶

The National Pediatric Cardiology Quality Improvement Collaborative ^{Reference Clauss, Anderson and Lannon87} and the Cardiac Neurodevelopmental Outcome Collaborative have created data registries to track neurodevelopmental outcomes for children with CHD. The creation of data registries and benchmarking, especially when approached through the lens of quality improvement science, will inform the development, implementation, and dissemination of best practice guidelines. For other complex paediatric conditions including cancer ^{Reference Walsh, Noll, Annett, Patel, Patenaude and Embry88} and cystic fibrosis, ^{Reference Fink, Loeffler, Marshall, Goss and Morgan89} the best practices of care have been driven by data derived from patient registries.

Significant gaps in knowledge

While much is known about the neurodevelopmental and psychosocial benefits of developmental follow-up programmes in neonatology, there are no published studies on the impact of participation in cardiac neurodevelopmental follow-up programmes. These programmes provide what are thought to be critical intervention supports and services, and yet empirical data are currently lacking.

Investigations needed

1. (1) Conduct feasibility, acceptability, and accessibility studies to examine processes (e.g., screening, monitoring procedures) and components (e.g., types of services) that result in the most beneficial outcomes

Outcome measurements, standardised across programmes, should focus on assessing domains that are most clinically meaningful to individuals with CHD and their families (e.g., quality of life, successful transition to independence). Studies may also include measurement of programme access, utilisation, cost-effectiveness, and socio-demographic variation as well as patient experience and pathways to care. Determining methods to reduce barriers to accessing cardiac neurodevelopmental programmes would boost attendance, a key aspect of universal protection/prevention screening and assessment programmes. It will be particularly important to examine the availability of trained personnel, time to appointment date or waitlist, physical space, cost, and insurance coverage.

1. (2) Examine whether centres that have coordinated cardiac neurodevelopmental programmes actually have improved neurodevelopmental and psychosocial outcomes for individuals with CHD

Study designs should include pre-post programme implementation data collection and should compare outcomes across time points as well as between centres with and without cardiac neurodevelopmental programmes on variables such as percentage of children entering school with appropriate educational supports, patient/family satisfaction, quality of life, and performance on formal measures of neurodevelopmental and psychosocial functioning. Establishing model programmes as the standard of care across medical centres will require clear evidence of effectiveness for a variety of stakeholders, including patients and families, advocacy groups, hospital administration, and insurance carriers.

1. (3) Develop efficient ways to screen individuals seen in cardiac neurodevelopmental programmes and tailor to different levels of intervention

Insofar as timely and appropriate identification and stratification of risk facilitate efficient access to limited assessment and treatment resources, it will be important to design and test procedures for screening individuals with CHD to ensure that resources are allocated appropriately. In line with screening models proposed by Kazak and colleagues, ^{Reference Kazak, Hwang and Fang Chen90} for paediatric psychology, and Hardy et al., ^{Reference Hardy, Olson and Sy91} for paediatric neuropsychology, large-scale, multi-site studies which evaluate the appropriateness of tiered screening procedures implemented within primary care/cardiology clinic settings would identify individuals most in need of neurodevelopmental and/or psychosocial support. As these models suggest, the majority of patients may succeed with only periodic surveillance and recommendations, while the minority will require more intensive interventions. Developing a way to screen patients into these tiered interventions should result in more efficient care and could result in resource savings.

Existing knowledge

The concept of resilience, defined as “a dynamic process wherein individuals display positive adaptation despite experiences of significant adversity or trauma,” ^{Reference Luthar and Cicchetti92} is perhaps best understood as a capacity that develops over time, rather than as an inherent personality trait. ^{Reference Ungar93} Resilience, and other wellness-promoting concepts such as post-traumatic growth and grit, is positively associated with better health outcomes ^{Reference Traub and Boynton-Jarrett94} and decreased stress responses ⁹⁵ within the general population, and improved psychosocial functioning and self-management in individuals with chronic illness. ^{Reference Stewart and Yuen96-Reference Sharkey, Bakula and Baraldi98} For example, among young, highly stressed children in foster care, therapeutic interventions have been shown to promote resilience by mitigating the effects of early adversity on hypothalamic-pituitary-adrenal axis activity and promoting the development of adaptive caregiver attachment relationships. ^{Reference Fisher, Gunnar, Dozier, Bruce and Pears99} In addition, adolescents with greater knowledge of their own medical history and associated complications, higher resilience, and more positive family dynamics have been found to better adhere to health-promoting behaviours such as following exercise and nutrition recommendations and reporting more adaptive stress management strategies. ^{Reference Huang, Chen and Chen100} Resilience in individuals with CHD is also related to a lower level of depressive symptoms ^{Reference Moon, Huh, Kang, Park, Jun and Lee101} and is influenced by parenting factors such as emotional warmth, rejection, punishment, control, and overprotection in adolescence. ^{Reference Moon, Song and Huh102}

Other than a feasibility study ^{Reference Kovacs, Bandyopadhyay and Grace103} and some evidence to support the use of a group-based intervention to improve aspects of resilience in adolescents with CHD, ^{Reference Wang, Whiteside and Sim42} studies examining resilience in individuals with CHD are limited. Numerous studies report that participation in disease-specific camp programmes positively influences perceived health, interpersonal relationships, and self-esteem, ^{Reference Martiniuk, Silva, Amylon and Barr49,Reference Desai, Sutton, Staley and Hannon104-Reference Walker and Pearman108} which are components of resilience, but resilience has not yet been specifically measured.

Significant gaps in knowledge

Literature on the experiences impacting resilience, interventions to bolster resilience, and measurement is limited in the CHD population. For both children and adolescents, there is evidence to suggest that exercise/physical activity interventions promote improved cardiovascular health and enhanced psychosocial functioning and quality of life individuals with CHD, ^{Reference Dulfer, Helbing and Utens109-Reference Dean, Gillespie and Greene112} although findings are somewhat mixed ^{Reference Dulfer, Helbing, Duppen and Utens113} and the impact of physical activity interventions on resilience remains to be examined. Even with more than 35 years of physical activity promotion and exercise training in patients with CHD, ^{Reference Takken, Giardini and Reybrouck114,Reference Duppen, Takken and Hopman115} research into optimal training methods and resilience as an outcome of physical exercise programmes is lacking.

Investigations needed

1. (1) Adopt a conceptual framework for designing and conducting resilience-promoting intervention studies that appropriately capture the nature and complexity of resilience

A model from the National Scientific Council on the Developing Child recommends that interventions designed to facilitate resilience should include 1) ways to improve the caregiver–child relationship, 2) methods for building self-efficacy and perceived control, 3) strengthening adaptive and self-regulatory functioning, and 4) incorporating faith, hope, and cultural traditions. ⁹⁵ Moreover, resilience interventions should encompass the entire lifespan and should begin with monitoring, supporting, and promoting the development of adaptive coping strategies for the family, if possible, before the child with CHD is even born, as further discussed below.

1. (2) Capitalise on early identification to begin bolstering caregiver/family resilience prior to delivery

At prenatal cardiac diagnosis, interventions designed to shape the communication provided to the family, with a particular focus on the developing parent–infant relationship, education regarding infant neurodevelopment, and maternal and paternal self-care would support family well-being. By focusing on optimisation and resilience, the emphasis of the prenatal visit may shift to infant neuroprotection and promotion of optimistic parent perceptions of their child, potentially reducing parental stress during the pregnancy. This focus on family well-being before the child is born could result in improved long-term outcomes for the child with CHD. ^{Reference Lisanti116}

1. (3) Recognise individual and family-based differences in perception and experience in living with CHD

The development of valid, CHD-specific tools to measure aspects of resilience will be important for assessing each child/family’s unique experiences with CHD, including systemic and cultural factors, and family stress and available support. Adding measurement of resilience to a standardised battery could help to better understand how different experiences bolster resilience and identify targets for future intervention.

1. (4) Identify interventions for promoting resilience during childhood and adolescence

Studies are needed to directly evaluate the potential benefit of physical activity interventions on resilience among individuals with CHD. Additionally, specifically measuring resilience as part of a CHD camp programme could help to better understand positive outcomes from this experience. Other potential interventions may include developing a mentoring programme for individuals with CHD or qualitative research with focus groups of adults with CHD to examine individual factors associated with resilience. Studies similar to those in adults with cancer, which utilise stress management to improve resilience, ^{Reference Loprinzi, Prasad, Schroeder and Sood117} would likely benefit adults with CHD as well and should be investigated.

1. (5) Include resilience as a primary outcome in CHD surgical trials

The effects of decreased stress ⁹⁵ should be looked at with respect to surgical outcomes, in addition to ICU length of stay, post-operative complications, and other aspects of health and recovery. Furthermore, child, family, and cultural markers of resilience should be carefully examined in clinical and surgical trials as potential moderators of outcomes.

Existing knowledge

The experience of living with CHD includes numerous transition points with possible vulnerability and potential for intervention. Some of the most salient transitions include: 1) acute inpatient care to stepdown unit care; 2) tube to oral feeding; 3) inpatient to outpatient settings; 4) surgical centre to local medical care; 5) early intervention to the school system; 6) childhood to adolescence; 7) adolescence to young adulthood; 8) paediatric to adult CHD care; and 9) for caregivers, from being primarily an observer of their child’s medical care to being the primary provider of daily medical surveillance and care and eventually becoming less active participants as their child moves towards independence.

The parents of medically complex infants with CHD can experience high levels of stress, post-traumatic stress, anxiety, and depression, ^{Reference Visconti, Saudino, Rappaport, Newburger and Bellinger118} which may negatively impact their ability to parent in ways most supportive of the high-risk child. The need to access services from multiple hospital and community systems makes communication and coordination among providers and caregivers critical. However, clear and understandable communication is often lacking. ^{Reference Penny119} Discharge instructions, for example, often include difficult medical terminology and can be confusing for families. School systems that are unfamiliar with the needs of children with CHD are unlikely to provide appropriate supports and services, increasing the risk for academic underachievement and discouragement. School-liaison programmes, which serve as a bridge between clinic and school and are considered standard-of-care in paediatric cancer, ^{Reference Wiener, Kazak, Noll, Patenaude and Kupst120} are effective in promoting access to services and associated with increased parent satisfaction and parent beliefs that their child is meeting his/her academic potential. ^{Reference Northman, Ross, Morris and Tarquini121,Reference Northman, Morris and Loucas122}

Specific to CHD, deficits in executive function, which are highly prevalent, ^{Reference Cassidy, White, DeMaso, Newburger and Bellinger123,Reference Sanz, Berl, Armour, Wang, Cheng and Donofrio124} are likely to become more problematic during the transition to adolescence, undermining the development of independence and adaptive skills that may further compromise the transition to adulthood. Indeed, 40%–60% of CHD patients experience a lapse in their care, particularly during the transition to adult medical care, and those who experience a lapse are three times more likely to require urgent cardiac intervention. ^{Reference Gurvitz, Valente and Broberg125,Reference Yeung, Kay, Roosevelt, Brandon and Yetman126} Lack of knowledge, self-management, and self-advocacy skills have also been documented among heart transplant patients. ^{Reference Grady, Hof and Andrei127} However, participation during adolescence in a nursing-led educational intervention designed to prepare transplant patients for transition to adult care resulted in better maintenance of medical follow-up and increased CHD knowledge and self-management skills, ^{Reference Mackie, Rempel and Kovacs128} so an initial transition intervention has shown some promise.

Significant gaps in knowledge

Intervention research aimed at promoting optimal adaptation across the full range of transitions inherent in CHD is limited, and existing research focuses almost exclusively on the transition from paediatric to adult care. ^{Reference Mackie, Rempel and Kovacs128} Interventions are still needed to aid parents in the transition from inpatient to outpatient care; to increase the effectiveness of patient/family CHD educational tools; and to support successful transitions from hospital to community-based CHD providers and schools. Moreover, among adolescents with CHD, it remains unclear how best to promote functional independence, adaptive skills, and self-awareness/knowledge of one’s medical condition.

Addressing these critical questions should result in a more accurate understanding of the range of transitions inherent to CHD, which would in turn facilitate the generation of smoother and more standardised procedures and practice guidelines for promoting optimal development across times of transition. With enhanced communication and coordination across providers and settings, fewer patients would be missed or lost to follow-up, child and family support needs would be more readily identified and would trigger appropriate referrals and access to therapeutic services, and barriers to accessing services would be recognised and addressed to reduce healthcare inequities.

Investigations needed

1. (1) Comprehensively characterise the full range of transitions inherent to living with CHD

Large-scale, population-based parent and/or self-report surveys with both qualitative and quantitative analytic techniques are needed to characterise the full range of transitions experienced by individuals with CHD, as well as key challenges to adaptive/functional independence across the lifespan.

1. (2) Utilise quality improvement science to improve strategies for assisting families in navigating CHD-specific challenges and transitions in the medical system

Quality improvement-oriented intervention studies are also indicated to evaluate and improve the effectiveness of strategies for preparing parents following prenatal CHD diagnosis. Additional interventions may include providing developmental summaries, modifying discharge information, and increasing the frequency of post-discharge follow-up and should deliberately consider how technology such as phone-based apps and teleconferencing may be used to support families following discharge.