Infants with CHD are at increased risk of neurodevelopmental delays or disabilities. Reference Mussatto, Hoffmann and Hoffman1–Reference Snookes, Gunn and Eldridge3 With an increasing understanding of the neurodevelopmental outcomes of this population, infants with CHD who have undergone open-heart surgery, as well as infants with cyanotic lesions not requiring open-heart surgery or with additional comorbidities, are considered to be high risk for developmental disorder or disability. Reference Marino, Lipkin and Newburger4 In their scientific statement on the evaluation and management of children with CHD released in 2012, the American Heart Association recommended that all children with CHD who are identified as high risk for developmental disability are referred for early intervention services. Reference Marino, Lipkin and Newburger4
Early intervention programmes provide services and support to babies and young children with developmental delays and disabilities. These services may include physical therapy, occupational therapy, speech therapy, and other types of services based on the needs of the child and family. Programmes are available in every state and territory in the United States of America, are publicly funded, and provide services for free or at a reduced cost for any child who is eligible including infants with CHD. 5 As recommended in the American Heart Association statement, Reference Marino, Lipkin and Newburger4 at our institution, we aim to refer all infants who receive open-heart surgery under the age of 1 year to an early intervention programme at the time of discharge. Furthermore, as recommended, our institution also refers these infants to a cardiac neurodevelopmental clinic for formal evaluation at 9–12 months of age. Yet, attendance has been demonstrated to be low at these specialised clinics, in part due to factors such as financial barriers and distance to the medical centre. Reference Loccoh, Yu and Donohue6 Early intervention services allow for earlier treatment of developmental deficits prior to formal neurodevelopmental evaluation.
The objective of the current study was to investigate the utilisation of early intervention services by infants with CHD following open-heart surgery within the first year of life. The study sought to determine whether infants with CHD received early intervention services following open-heart surgery, to describe the received services, to understand the value of the services as perceived by the caregiver, and to identify factors that were associated with receipt of services.
Materials and methods
The study included infants who underwent open-heart surgery during the first year of life at a single tertiary care paediatric hospital between 7 January, 2017 and 7 January, 2018. Infants were excluded if they died prior to contact or if their caregivers did not speak English as their primary language given restriction in survey administration. The study was approved by the Institutional Review Board. A comprehensive informed consent was obtained from the caregiver with a waiver of documentation. A waiver of consent was obtained to access the infant’s electronic medical record.
Caregiver contact information was obtained from the electronic medical record. Caregivers were initially contacted by telephone. If contact was not made within two telephone call attempts, a paper survey was mailed to the primary address. One final call was made to all eligible caregivers who did not return a paper survey. One researcher administered all of the scripted telephone surveys.
Information collected by the survey included infant/family demographics and receipt of early intervention services. If the infant was currently or previously received services, the caregiver was asked about the type and frequency of services received. Additionally, the caregiver was asked three questions on the utility of the services their infant received. These questions were (1) When you think about the services that your child is receiving/received, how helpful do/did you think these services are/were? (2) Do/did you feel like your child receives/received the appropriate amount of services from (name of state programme)? (3) Would you recommend services through an early intervention programme to other caregivers of children with CHD? For infants who never received services, the caregiver was asked about whether they were contacted by the early intervention programme, whether an in-home assessment was performed, and why their child ultimately did not receive services.
In addition to the survey, a retrospective medical record review was performed for all eligible infants. Demographic, preoperative, intraoperative, and post-operative clinical data for all infants was obtained.
Data were summarised using descriptive statistics including frequency (percentage) and median (interquartile range). To determine whether the infants with caregivers who completed the surveys were significantly different than the infants with caregivers who did not, demographic and clinical characteristics between the two groups were compared using the Chi-square test and Fisher’s exact test for categorical variables and Mann–Whitney test for continuous variables. Similarly, univariate comparisons of demographic and clinical characteristics and survey data between infants who received early intervention services and those who did not were made utilising the Chi-square test, Fisher’s exact test, and Mann–Whitney test, as appropriate. Variables that were identified as significantly different between the infants who did and did not receive services in the univariate analysis were considered for inclusion in a multivariable analysis. Multicollinearity amongst the variables included in the multivariable analysis was examined using the variance inflation factor. A multivariable logistic regression was constructed to examine independent associations of patient and clinical characteristics with receipt of early intervention services. All analyses were performed using IBM SPSS Statistics Version 26 (IBM Corp., Armonk, NY, United States of America) with a statistical significance level of 0.05.
Results
Amongst the 175 identified infants who received open-heart surgery prior to 1 year of age from 7 January, 2017 to 7 January, 2018, 158 infants were eligible for inclusion in the study. Eleven infants died prior to contact and six were from non-English-speaking households.
Surveys were completed by 98 caregivers (62%, 82 by phone and 16 by mail). The median age of infants at the survey was 1.3 years (IQR 1.1–1.6 years, range from 10.1 months to 2.4 years). Caregivers were predominantly mothers (90.8%), college graduates (53.1%), and residing in two caregiver households (77.6%). There were no significant differences in demographic and clinical characteristics between the infants with caregivers who did versus did not complete the surveys (Table 1). Of those surveyed, 53.1% of infants (n = 52) were either currently or previously enrolled in early intervention services. Infants most commonly received physical therapy (76.9%). Services were most often provided on a weekly basis. Thirty-eight percent of infants received additional services through an outpatient clinic or hospital while receiving early intervention services. When asked about their experience with the early intervention programme, 92.3% of caregivers described the programme as at least moderately helpful. The majority (76.9%) of caregivers felt as though their infant was receiving the right amount of services through the programme. Most caregivers (94.2%) would recommend early intervention services to caregivers of children with CHD (Table 2).
Table 1. Comparison of patient demographics and clinical characteristics by the completion of survey.
ECMO=extracorporeal membrane oxygenation; ICU=intensive care unit; STAT=The Society of Thoracic Surgeons – European Association for Cardio-Thoracic Surgery; VAD=ventricular assist device
* Data are presented as frequency (percentage) for categorical variables; median (interquartile range) for continuous variables
§ p-value from Chi-square test or Fisher’s exact test for categorical variables and Mann–Whitney test for continuous variables
† Chi-square test is used to compare Caucasian versus non-Caucasian
Table 2. Characteristics of early intervention services (n = 52).
* Infants could receive more than one service through early intervention programmes
Of the 46 caregivers with infants who did not receive early intervention services, 15 (32.6%) recalled telephone contact with the programme following discharge from the hospital; 9/15 caregivers had a subsequent in-home assessment of their infant. The most common reason, recalled by 8 of the 15 caregivers for not receiving services after a telephone or in-home assessment, was that the services were unnecessary. In addition, 5 of the 15 caregivers reported that they declined services as they, the parent/guardian, did not believe developmental support was necessary.
In comparison to infants who never received services, infants who received services had a higher proportion of single-ventricle disease (p = 0.002), known syndromes or genetic abnormalities (p < 0.0001), and extracardiac anomalies (p = 0.001) (Table 3). Additionally, patients who had experience with an early intervention programme had longer intensive care length of stay (p = 0.01), longer hospital length of stay (p = 0.02), and greater frequency of prolonged hospitalisations defined as greater than 2 weeks in duration (p = 0.049). There were no significant differences in the complexity of open-heart surgery, primary caregiver education, or proportion of single caregiver households between infants who did and did not receive services. There was no association of age at the survey with receipt of services (median age 1.3 years [IQR 1.1–1.6] with services versus 1.3 years [IQR 1.1–1.6] without services, p = 0.92).
Table 3. Comparison of patient demographics and clinical characteristics by receipt of early intervention services.
ECMO=extracorporeal membrane oxygenation; ICU=intensive care unit; STAT=The Society of Thoracic Surgeons – European Association for Cardio-Thoracic Surgery; VAD=ventricular assist device
* Data are presented as frequency (percentage) for categorical variables; median (interquartile range) for continuous variables
§ p-value from Chi-square test or Fisher’s exact test for categorical variables and Mann–Whitney test for continuous variables
‡ Comparison using Chi-square test between STAT 1 to 3 versus 4 or 5
Variables included in the multivariable analysis were single-ventricle disease, known syndrome or genetic abnormality, extracardiac anomaly, and prolonged hospitalisation. ICU and hospital length of stay were omitted due to collinearity with prolonged hospitalisation. Presence of single-ventricle disease (p = 0.004) and a known syndrome or genetic abnormality (p<.0001) remained independently associated with receipt of early intervention services (Table 4).
Table 4. Factors associated with receipt of services from early intervention programme (n = 98).
CI=confidence interval; OR, odds ratio
α p-value from univariate logistic regression
t p-value from multivariable logistic regression
Discussion
Our study describes the utilisation of early intervention services by infants with CHD who are at increased risk of developmental delay. In our cohort, only half of the infants who had undergone surgery in the first year of life were either actively or previously participating in early intervention services. It is encouraging that amongst infants who received services, the majority of parents expressed satisfaction with the early intervention programme. However, many at-risk infants with CHD failed to receive services.
Our study expands upon previous research into the utilisation of early intervention services by infants at risk of developmental deficits. Similar participation rates with early intervention services were demonstrated by 2-year-old children with a history of prematurity by Roberts et al Reference Roberts, Howard and Spittle7 who found almost half of the studied children with moderate to severe disability (49%) were not receiving services. Reference Roberts, Howard and Spittle7 Evaluating the patterns of participation with early intervention services in infants with hypoplastic left heart syndrome, Mussatto et al Reference Mussatto, Hollenbeck-Pringle and Trachtenberg8 found greater than half of these high-risk infants were not receiving early intervention services between 1 and 4 years of age. Reference Mussatto, Hollenbeck-Pringle and Trachtenberg8 Analysing social risk factors including maternal age at child’s birth, insurance type, and neighbourhood poverty index did not uncover any individual or cumulative social risk factors associated with receipt of services. Reference Mussatto, Hollenbeck-Pringle and Trachtenberg8 Consistent with these findings, we did not find that single caregiver status or education level were significantly associated with receipt of early intervention services.
The presence of single-ventricle disease was associated with receipt of early intervention services in our study. While Newburger et al Reference Newburger, Sleeper and Bellinger9 previously showed that children with hypoplastic left heart syndrome and related anomalies who were enrolled in the Single Ventricle Reconstruction trial had decreased scores on Bayley Scales of Infant Development Psychomotor Development Index and Mental Developmental Index testing at 14 months of age, they found only 47% of these infants were reported to have received developmental support within the first year of life. Reference Newburger, Sleeper and Bellinger9 In our study, infants with single-ventricle disease were significantly more likely to receive early intervention services than infants with biventricular heart disease. Mussatto et al Reference Mussatto, Hoffmann and Hoffman1 uncovered significant developmental disability in children with CHD in the first 3 years of life with most patients (75%) having scores greater than one standard deviation below the population mean during at least one developmental assessment. Reference Mussatto, Hoffmann and Hoffman1 Yet, infants with single-ventricle disease did not have worse developmental outcomes compared to the biventricular infants in the study. The authors suggest that many infants with biventricular heart disease are also at risk, but may not be getting the monitoring and developmental services they require in contrast to single-ventricle patients who benefit from a programmatic approach that systematically monitors this population at many institutions. Reference Mussatto, Hoffmann and Hoffman1
Multiple studies have demonstrated that infants with CHD and comorbid syndromes/genetic abnormalities have increased rates of developmental disability. Tabbutt et al Reference Tabbutt, Nord and Jarvik10 showed that the presence of a syndrome or genetic abnormality was associated with lower Psychomotor Developmental Index and Mental Developmental Index scores in patients with hypoplastic left heart syndrome at 12 months of age. Reference Tabbutt, Nord and Jarvik10 These trends in Mental Developmental Index scores were also observed at 14 months in the Pediatric Heart Network’s Single Ventricle Reconstruction cohort. Reference Newburger, Sleeper and Bellinger9 In our cohort, the majority of infants with a known syndrome or genetic abnormality received early intervention services.
Reasons for failure to receive early intervention services remain unclear. One in three caregivers of infants who failed to receive services and had interacted with an early intervention programme declined services due to a belief that developmental support was not necessary for their child. While it is possible that the parent’s assessment was accurate, parents of infants with CHD may be overwhelmed by their child’s cardiac diagnosis and may not recognise developmental needs. Sananes et al Reference Sananes, Manlhiot and Kelly2 investigated the parental perception of infant development after open-heart surgery. They showed that parental concern for their child’s development increased over the first 2 years of the child’s life. The researchers hypothesised that increasing concern may be due to decreased preoccupation with the child’s medical state and increased attention to the child’s developmental trajectory over time. Reference Sananes, Manlhiot and Kelly2 Further research is necessary to understand the parental perception of developmental needs during early infancy.
Yet, the responsibility for identifying developmental deficits in an infant with CHD does not belong solely to the parent. Improving the receipt of services by infants at risk of the development delay requires systematic assessment by primary care providers and paediatric subspecialists within a medical home model. Reference Marino, Lipkin and Newburger4 The American Heart Association’s Scientific Statement in 2012 complements the 2006 AAP policy on identifying infants and young children with developmental disorders by providing primary care providers an algorithm for risk stratification, surveillance, and screening of children with CHD. Reference Marino, Lipkin and Newburger4,Reference Lipkin and Macias11 Despite these recommendations, Knutson et al (2016) discovered that only 21% of 326 surveyed paediatric primary care providers were aware of the guidelines. Reference Knutson, Kelleman and Kochilas12 While 49% of the providers stated that they referred infants with CHD to formal medical or developmental evaluation “most of the time” or “always”, the majority of referrals were reactive and not related to specific cardiac indications or risk. Additionally, 53% of providers stated that they did not refer a patient with CHD to formal developmental/medical evaluation if the child was receiving early intervention services. Reference Knutson, Kelleman and Kochilas12 Improved education of paediatric primary care providers regarding the recommended surveillance and screening practices of infants with CHD is critical to connecting these infants to developmental services. More concerted efforts are needed to assure that all infants, including those less privileged infants who may have been under-represented in our study, are referred for evaluation for early intervention services. Paediatric cardiologists need to identify gaps in developmental care services and provide counselling and referral as needed.
Several limitations of our study must be acknowledged. As our study occurred at a single institution with limited racial and ethnic diversity, generalisation of our findings to the broader population may be limited. While it is our practice to refer all infants who undergo cardiac surgery in the first year of life to an early intervention programme, documentation of the referral was not consistently available to confirm that referral was completed. Furthermore, as our institution has a process for referral to early intervention programmes and formal developmental testing, our results may not generalise to institutions without these practices. Collecting information from the caregiver, our information may be biased by parental report and recall. Lastly, our study assessed participation at one point in time and our results cannot be extrapolated into later childhood. Further studies are needed to evaluate the impact of early intervention services on later developmental outcomes, which was beyond the scope of our study.
In our study cohort of infants with CHD at high risk for developmental delay, one in two infants received early intervention services. Bridging this gap in care requires the assistance of primary care providers and cardiac specialists who can regularly elicit parental concerns, identify infants with CHD who are at high risk of developmental impairments, and initiate referral and follow-up with early intervention programmes. Reference Marino, Lipkin and Newburger4 Investigation into parental perceptions of developmental deficits in infants with CHD as well as barriers that prevent the enrollment of this unique population into early intervention programmes may improve access and receipt of these services. Through these collaborative processes, we can continue to improve the developmental outcomes of all infants with CHD.
Acknowledgements
None.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Conflicts of interest
None.
Ethical standards
Not applicable.
