Don’t Delay – The Time is Now to Prioritize Addressing Modifiable Risk Factors Through Developmental Care

As surgical outcomes have improved in the field of congenital cardiovascular care over time, there has been a paradigm shift away from focusing clinical care and research on mortality reduction to short-term and long-term morbidity reduction. The single most common morbidity affecting children with complex congenital heart disease (cCHD) is the combined outcomes of their developmental disorders and disabilities (DD).¹ Since the previous 2012 AHA Scientific Statement² summarized the concerns with neurodevelopmental outcomes in children with cCHD, attention to the development and maturation of outpatient Cardiac Neurodevelopmental Programs has been a priority. These initiatives however have not specifically targeted the vulnerable infant brain during the perioperative period – a critical time when normal developmental processes are frequently interrupted. The science advisory from the American Heart Association entitled ‘Developmental Care for Hospitalized Infants with Complex Congenital Heart Disease' sought to address this issue directly. Created by an interdisciplinary team purposely representing pediatric cardiology, neurology, cardiovascular and critical care nursing, cardiac intensivists, psychology and developmental therapists, the purpose of the advisory was to: 1) describe the burden of developmental disorders, disabilities, and delays for infants with cCHD, 2) define the potential health and neurodevelopmental benefits of developmental care for infants with cCHD, and 3) identify critical gaps in research aimed at evaluating developmental care interventions to improve neurodevelopmental outcomes in cCHD.

What is the Burden of DDs in the CHD population?

Multiple studies have recognized and highlighted the significant burden that DD has placed on the cCHD patient and their family unit.² Delays noted early in life impact multiple domains and often become additive. What might start out as a mild motor delay and poor feeding in a patient in the first year of life may only be a hint of the concerns to come, such as cognitive impairment, attention deficits, and poor academic achievement, as the child progresses in their life journey. These are often then compounded with the addition of anxiety, depression, poor self-image, and social withdrawal. This ultimately translates into lower educational achievement, need for remedial academic services, and diminished quality of life even with good supports along the way.^3-5 These delays are multifactorial including both biological and environmental factors,⁶ including white matter injury and compromised brain maturation over time, most significantly during the initial neonatal hospital stay.⁷

What is "Developmental Care"?

Developmental care (DC) for infants is a validated approach that aims to maximize neurologic development and reduce long-term DD with proven success in the preterm infant population.⁸ Similar to the premature infant, the infant with cCHD often experiences prolonged hospital stays during critical brain maturation windows. Fetal ultrasound and MRI studies have shown that infants with cCHD may have blunted brain maturation and growth at birth compared to others at the same gestational age (specifically with evaluation of left-sided obstructive lesions and transposition of the great arteries).⁹ This immature and delayed brain development in newborns with cCHD poses a unique vulnerability for further injury from environmental and toxic stress, pain, and parent separation that occurs during critical illness. In simple terms, DC methodology incorporates a holistic approach individualizing family-centered care by careful observation and interpretation of an infant's behavior allowing to incorporate modifications of the environment and cue-based interventions to promote neuroprotection.¹⁰ Is it possible to translate DC practices from the neonatal intensive care unit to the pediatric cardiac intensive care unit? Theoretically, it is possible, but will require individual adaptation based on the patient's physiology, acknowledging the significant influence that cCHD has also on the mental, behavioral, and emotional impact on the infant and family unit.¹⁰ It will require systems and cultures to change in order to be successful. Currently, we often provide bedside care based on the schedules and preferences of the healthcare team, not the needs of the infant. Similar to the experience in the premature infant, successful DC needs to include a proactive relationship with the family to promote active engagement and involvement in their infant's care from birth through the entire hospitalization, including the challenging periods that have historically been handled by asking parents to leave for procedures or not touch their child when considered "unstable". The advisory statement nicely outlines intentional interventions to facilitate neuroprotective care using family-centered approaches in multiple domains across the continuum of care while in the hospital including during fetal life, before and after surgery, and in preparation for hospital discharge.¹⁰ Core components of DC include partnering with parents, cue-based family-centered care, and provision of positioning and motor support.^8,11,12

What is the potential benefit of DC in the care of cCHD patients?

The potential benefits of DC for cCHD patients seems to be a loaded question. The benefits very well may be limitless. As this population unfortunately has many medical variables that are non-modifiable, specific attention needs to focus on any and all modifiable factors. Infants are easily ignored or don't have a voice. They are vulnerable because they can't advocate for themselves. DC gives them a voice by purposefully focusing on infant communication through their behavior and cues. We are only beginning to see the benefits of DC interventions such as environmental sound and lighting modifications, skin-to-skin care, massage, containment, and awake prone positioning.^13-17 Ongoing development, implementation, and dissemination of these early neuroprotective interventions hopefully will help alter the trajectory of DD in this population by promoting early brain maturation and mitigating known risk factors. Actively including the family early in the hospital course (preferably even in the fetal clinic) and continuously supporting their engagement and parental role throughout their infant's hospitalization may help to reduce or minimize the associated anxiety, depression, and post-traumatic stress that these families experience.

What are the critical gaps in research involving DC interventions?

The biggest strength of this advisory is its transparency in what is not known. Well, at least what has not been proven with solid, reproducible science. Critical gaps exist and need to be addressed. Research is needed to evaluate the impact of DC interventions on brain maturation, DD, and parental mental health. The time is now. Table 2 in the advisory highlights 14 detailed DC goals for potential research projects and more importantly, suggested DC interventions that will need to be evaluated in multidisciplinary studies to achieve the associated goal. To maximize the potential that this research could produce, I would posit that future work needs to continue to have interdisciplinary teams working together in a team science approach. If these gaps are addressed and the goals are achieved in a timely fashion, I can envision that this advisory will morph into a future scientific statement that will forever alter the way infants with cCHD are managed in pediatric cardiac intensive care units.

Conclusion

Family-centered DC for hospitalized infants with cCHD has the potential to greatly improve the way infants are cared for in the pediatric cardiac intensive care unit, rewriting our infants' futures with the anticipated goal of decreased incidence of DD, improved quality of life, and improved mental and emotional health. The science advisory is a call to action for the entire community (families, clinicians, research scientists, policy makers, government agencies, advocacy groups, and healthcare administration) to come together and support a culture of change. Funding will be essential to fully implement DC intervention practices across programs. Likewise, funding will be necessary to fully evaluate the effect of these DC interventions on short-term and long-term DD. Dissemination of findings and best practices will be needed. Organizations such as CNOC (Cardiac Neurodevelopmental Outcome Collaborative) and its special interest group - CNNN (Cardiac Newborn Neuroprotective Network) will likely take the lead to push this agenda forward.