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PEDIATRICS Vol. 108 No. 6 December 2001, pp. 1325-1331

Functional Limitations in Young Children With Congenital Heart Defects After Cardiac Surgery

Catherine Limperopoulos, OT, MSc*, Annette Majnemer, OT, PhD*,{ddagger},||, Michael I. Shevell, MD, CM, FRCP(C){ddagger},||, Bernard Rosenblatt, MD, CM, FRCP(C)*,{ddagger},||, Charles Rohlicek, MD, CM, PhD, FRCP(C)||, Christo Tchervenkov, MD, CM, FRCS(C)§ and H.Z. Darwish, MD, FRCP(C)

* School of Physical and Occupational Therapy, McGill University, Montreal Children’s Hospital, Montreal, Quebec, Canada
{ddagger} Department of Neurology and Neurosurgery, McGill University, Montreal Children’s Hospital, Montreal, Quebec, Canada
§ Department of Cardiovascular and Thoracic Surgery McGill University, Montreal Children’s Hospital, Montreal, Quebec, Canada
|| Department of Pediatrics, McGill University, Montreal Children’s Hospital, Montreal, Quebec, Canada
Division of Pediatric Neurology, Alberta Children’s Hospital, Calgary, Alberta, Canada


   ABSTRACT
TOP
 ABSTRACT
INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
 REFERENCES
 
With the recent dramatic decline in mortality rates of infants undergoing open-heart surgery (OHS), there is growing concern regarding neurodevelopmental sequelae. Outcome studies have primarily focused on delineating developmental impairments; however, the impact on function and family burden has not been investigated. The objective of this study was to determine the prevalence of functional limitations and burden of care of young children with congenital heart defects (CHD) after OHS.

Study Design. One hundred thirty-one eligible infants with CHD undergoing their first OHS were recruited prospectively. Patients were assessed pre- and postoperatively, and again 12 to 18 months after surgery. Functional assessments included the WeeFIM (Functional Independence Measure) and the Vineland Adaptive Behavior Scale.

Results. For the WeeFIM, mean quotients were 84.3 ± 23.8 (self-care), 77.2 ± 30.0 (mobility), and 92.4 ± 27.8 (cognition), with an overall quotient of 83.8 ± 23.4. Only 21% of the cohort was functioning within their expected age range. Moderate disability was noted in 37%, while only 6% demonstrated a severe disability. For the Vineland scale, mean score for daily living skills was 84.4 ± 17.6, and 80.3 ± 15.9 for socialization. Functional difficulties in daily living skills were documented in 40%, whereas >1/2 had poor socialization skills. Factors enhancing risk for functional disabilities included perioperative neurodevelopmental status, microcephaly, length of deep hypothermic circulatory arrest, length of stay in the intensive care unit, age at surgery, and maternal education.

Conclusions. The high prevalence of functional limitations and dependence in activities of daily living is currently underappreciated in the clinical setting, and deserves additional attention by pediatricians and developmental specialists.

Key Words: congenital heart defects • cardiac surgery • outcome • function

Abbreviations: OHS, open-heart surgery • CHD, congenital heart defects • CPB, cardiopulmonary bypass • DHCA, deep hypothermic circulatory arrest • HLHS, hypoplastic left heart syndrome • VABS, Vineland Adaptive Behavior Scale • OR, odds ratio • CI, confidence interval • ICU, intensive care unit


   INTRODUCTION
TOP
 ABSTRACT
 INTRODUCTION
METHODS
 RESULTS
 DISCUSSION
 REFERENCES
 
As mortality rates after infant open-heart surgery (OHS) continue to dramatically decline, the neurodevelopmental outcome of survivors has come under increasing scrutiny. Acutely, there is increasing electrophysiological and clinical evidence of brain injury in young infants undergoing surgical correction.1,2,3,4 It is as yet unclear whether acute neurodevelopmental abnormalities are transient or persist long-term. The long-term outcome of children with congenital heart defects (CHD) after early surgical repair has primarily focused on neurologic status and cognitive impairments.5 Severe neurodevelopmental sequelae, such as cerebral palsy and mental retardation, are uncommon. Overall, existing evidence would suggest that global developmental deficits are common across the developmental spectrum, particularly in gross and fine motor skills, language, reasoning, and behavioral difficulties.6,7,8,9,10 Educational difficulties and a need for special classroom placement or individualized instruction as a result of learning disabilities and attentional problems also seem to be common in this high-risk population.11,12,13 It is critical to define developmental deficits across all domains to target which rehabilitation professionals are needed for specific interventional purposes. However, the extent and nature of neurodevelopmental deficits/impairments and concomitant functional implications in this population requires additional definition.

One of the limitations related to current research design of pediatric outcome studies has been the lack of application of functional measures.14,15,16 It is important to measure function given that even if a child is found to have a poor motor or intelligence quotient, this does not necessarily inform us of the impact of such impairments on overall functioning. The impact of the spectrum of neurodevelopmental impairments on overall functioning in daily life (ie, the ability to carry out day-to-day tasks and activities expected for a child’s given age) has been underexplored in children with CHD. Moreover, the association between current medical and surgical interventions on family and child functioning and family burden needs to be addressed to best meet the ongoing resource needs of these children and their families. Therefore, the primary objective of this study was to describe the functional limitations and burden of care of young children with CHD after OHS. As a secondary objective, we examined factors documented during the initial admission for surgery that may be associated with a greater risk of disability.


   METHODS
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
RESULTS
 DISCUSSION
 REFERENCES
 
A consecutive series of young children with CHD who underwent OHS at the Montreal Children’s Hospital were recruited before surgery and are being followed prospectively. Preoperative and acute postoperative neurodevelopmental assessments were conducted and have been previously reported.3,4 A number of outcome measures (ie, neurologic, motor, developmental, and functional) were used to characterize the neurodevelopmental outcome of our cohort after OHS. This study will focus on the functional outcomes of the cohort after cardiac surgery.

Participants
Participant inclusion/exclusion criteria have been reported in detail previously.4 Participants included those with a diagnosis of a CHD undergoing their first OHS with cardiopulmonary bypass (CPB) and/or deep hypothermic circulatory arrest (DHCA) before 2 years of age. Participants were excluded if there were known risks for neurologic sequelae attributed to factors other than direct complications of the heart defect. These would include the following: 1) prematurity or small for gestational age, 2) clinical evidence of a central nervous system anomaly (eg, brain malformation) or insult (eg, perinatal asphyxia), and 3) those with a syndrome or chromosomal aberration associated with subsequent neurodevelopmental disability (eg, Down syndrome, DiGeorge syndrome). Children diagnosed with hypoplastic left heart syndrome (HLHS) were also excluded, as the literature has suggested a higher prevalence of neurologic morbidity in this subgroup of children.17,18,19,20

Procedures
Twelve to 18 months after OHS, evaluations were conducted when convenient for the families at the Montreal Children’s Hospital. Children ranged between 1 to 3 years of age (most 12–18 months) at the follow-up visit. An occupational therapist trained in the use of the WeeFIM conducted a parent interview, and a psychologist administered the Vineland Adaptive Behavior Scale (VABS) to the caregivers by a semistructured interview. The assessments were administered according to the established protocols for each instrument. The evaluators were not directly involved in the medical care of the patients. They were blinded to the child’s health status and past medical history, including any previous neurodevelopmental findings.

As part of this follow-up study, the association between preoperative and postoperative risk factors and functional outcomes were also determined. Chart reviews were conducted to document pertinent surgical and medical factors. Categorical risk factors included: type of CHD (ie, cyanotic vs acyanotic), low arterial oxygen saturations (Pao2<85) before surgery, type of cardiac surgery (ie, corrective vs palliative), and maternal/paternal education (more than high school). Continuous risk factors included the following: intraoperative procedures (ie, minutes of CPB and DHCA), age at surgery, length of stay in intensive care (days), total duration of hospitalization (days), and number of subsequent hospitalization and cardiac surgeries. In addition, neurodevelopmental status and head circumference measurements (<5th percentile) that were documented before and after OHS were examined (reported in Limperopoulos et al3,4). Variables at the time of follow-up testing that were correlated with functional outcomes were growth parameters (height/weight <5th percentile), persisting cyanosis, and ongoing need for medications.

Functional Measures
The WeeFIM instrument is a pediatric functional assessment designed to assess and track levels of functional independence in children aged 6 months to 7 years.21 This is a burden of care measure that quantifies the level of assistance or supervision required for daily tasks.22 Severity of disability is measured by determining how much assistance is required for a child to complete each functional activity, above and beyond that which is considered for a child of a given age, and what type of aids/adaptations are required to achieve or enhance level of independence. The WeeFIM total score describes a child’s usual or actual performance in basic daily living skills as opposed to the child’s best performance. This functional measure contains 18 items across the domains of self-care (6 self-care and 2 bowel and bladder management items), mobility (3 transfer and 2 locomotion items), and cognition (2 communication and 3 social cognition items). Self-care items examine how independent a child is in eating, grooming, bathing, upper body and lower body dressing, and toileting. The mobility domain includes chair/wheelchair, toilet, and tub/shower transfers, and locomotion with respect to walking, use of wheelchair or crawling, and going up/down stairs. The cognition domain examines how well a child expresses his/herself and understands communication. This domain also comprises social interaction (eg, skills related to getting along and participating with others in play situations). In addition, problem solving is addressed by assessing a child’s ability to solve problems of daily living and memory (eg, remembering daily routines, recognizing familiar people). Each item is rated on a 7-point ordinal scale ranging from 7 (complete independence) to 1 (total assistance). The WeeFIM is a psychometrically sound instrument in terms of its reliability, validity, and responsiveness.23,24,25 Age-based norms are used to calculate functional quotients from the raw scores for each domain. A moderate disability is defined as scores falling between 50 to 75, whereas a severe disability encompasses scores below 50 (Msall, personal communication, September 21, 2000). The latter would include children who are essentially fully dependent in self-care and mobility.

The VABS was also completed by parent interview. This assessment is a discriminative measure that records adaptive behavior in 4 domains: daily living skills, socialization, motor skills, and communication for children 0 to 18 years of age. An optional domain of maladaptive behavior can be included. The VABS is norm-referenced, based on the performance of representative national standardization samples of handicapped and nonhandicapped individuals.26 The standard version of the VABS consists of 301 items. The scoring options include the following: the activity is never performed (0 points), the activity is sometimes performed or performed with partial success (1 point), the activity is usually or habitually performed (2 points), or the interview respondent has no knowledge of the client’s performance (DK, don’t know). The 2 subdomains that were scored for this study included daily living skills (eg, drinks from cup unassisted, feeds self with spoon and fork), and socialization (eg, laughs/smiles appropriately in response to positive statements, participates in at least 1 game or activity with others). This assessment has also been shown to be reliable and valid.23,24,27,28,29 Age-based standard scores (mean: 100, standard deviation: 15) were used for purposes of analysis.

Statistical Analysis
Descriptive statistics (means, standard deviations) were used to characterize the functional outcomes of our sample. The proportion of participants with abnormal scores on the functional measures was also determined. For the VABS, a 1.5-standard deviation cutoff was used, whereby scores falling below 78 were considered as indicative of a functional difficulty in each subdomain. For the WeeFIM, scores <75 were considered abnormal. Univariate ({chi}2 for categorical variables and t tests for continuous data) and multivariate regression analyses were applied to determine the possible associations between risk factors (as outlined in the procedures above) and functional outcomes.


   RESULTS
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
DISCUSSION
 REFERENCES
 
Sample Characteristics
Of 131 participants that were recruited, 13 died and 20 did not undergo follow-up testing. Fifty-five participants (42%) underwent surgery in the neonatal period (mean age at surgery: 10 days), whereas 76 participants (58%) had OHS during infancy (mean age at surgery: 5.9 months). Eighty-four percent of the cohort underwent corrective OHS, whereas 16% had palliative procedures. Of the 20 children that did not have follow-up evaluations, 13 were from outside of the Montreal Metropolitan area, 4 could not be reached, and 3 were no longer interested in participating in the study. Comparisons between those participants that did not return for follow-up testing and those that did demonstrated no significant differences on baseline variables (eg, preoperative and acute postoperative neurodevelopmental testing, length of CPB and DHCA, low preoperative oxygen saturations [<85], cyanotic vs acyanotic CHD, palliative vs corrective surgeries, length of hospitalizations and subsequent hospitalizations/surgeries, maternal and paternal education). Ninety-eight participants underwent formal assessments (by an occupational therapist and/or psychologist) 12 to 18 months after OHS (98/118; 83% participation rate), at a mean age of 20.5 ± 8.1 months. Not all assessments were conducted on all participants, depending on the availability of testers at the time of evaluation. Growth parameters were documented and 21% had weights below the 5th percentile, whereas 22% had heights below the 5th percentile.

Participants fell into 1 of the following diagnostic categories of CHD: tetralogy of Fallot (30), ventricular septal defect (13), transposition of the great arteries (10), double outlet right ventricle with subaortic ventricular septal defect (9), atrioventricular septal defect (8), including 1 with severe pulmonary stenosis, pulmonary atresia with intact ventricular septum (4), univentricular heart (4), interrupted aortic arch (3), double outlet right ventricle with subpulmonary ventricular septal defect (3), atrial septal defect (3), transposition of the great arteries with ventricular septal defect (3), coarctation of the aorta with aortic arch hypoplasia (3), including 1 with aortic arch hypoplasia, aortic valve stenosis (2), cortriatriatum (1), total anomalous pulmonary venous connection (1), and anomalous origin of the left coronary artery (1).

Cardiorespiratory Status
At follow-up, 13% of the cohort were on medications which included lasix (N = 5), digoxin (N = 2), prophylactic penicillin (N = 1), propanolol (N = 2), pulmacort (N = 1), vasotek (N = 1), and ventolin (N = 1). Twelve percent of participants remained cyanotic, and 4% had congestive heart failure. Ninety percent were determined to have adequate cardiac function by the cardiologist.

Socioeconomic Status
Forty-seven percent of mothers and 52% of fathers had post-high school education.

Functional Outcomes
The WeeFIM was administered on 87 children. The mean quotient across the 3 domains ranged from 77 to 92 (Table 1). Only 21% of the cohort was functioning within the expected range for their age. Thirty-seven percent of participants scored in the moderate disability range on their overall WeeFIM scores (ie, functional quotients between 50–75), whereas 39% performed within this range in the self-care domain, 46% in the mobility domain, and 29% in the cognitive domain. Overall, scores for only 5 participants (6%) were in the severe disability range (ie, functional quotients <50), with 6 participants (7%) scoring in this range for self-care, 17 (20%) for mobility, and 5 (6%) for cognition. Participants’ performance within each of the 3 subdomains (ie, self-care, mobility, and cognition) of the WeeFIM were highly correlated (r = 0.62–0.80).


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  		TABLE 1. Means, Standard Deviations, Medians, and Ranges on the WeeFIM and VABS

 
The VABS was administered to 80 participants. The mean scores for both subdomains were shifted downwards approximately 1 standard deviation (Table 1). Forty percent (32/80) of children demonstrated difficulties in performance of activities of daily living (ie, <1.5 standard deviations below the normative mean of 100), while 53% presented with difficulties in socialization skills. Similarly, there was a strong relationship between participants’ performance in daily living skills and socialization skills on the VABS (r = 0.88).

Risk Factors Associated With Functional Limitations
Univariate analyses identified the following risk factors as being significantly associated the functional outcomes (Table 2 and 3). For the WeeFIM at baseline, there was a significant relationship between preoperative and acute postoperative neurodevelopmental abnormalities and the presence of microcephaly, time spent in intensive care, length of hospitalization, and palliative procedures, with subsequent functional difficulties. At follow-up testing, persisting cyanosis, persistent need for medications, lower weights, subsequent admissions, and surgeries were significantly associated with lower WeeFIM scores. For the VABS daily living skills subdomain, increasing age at surgery was associated with greater functional limitations. There was a significant association between acute postoperative neurodevelopmental findings, microcephaly, increasing age at surgery, and subsequent admissions and socialization difficulties on the VABS. There was no significant association between maternal or paternal education and functional outcomes.


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  		TABLE 2. Predictors of Functional Limitations 12 to 18 Months After OHS ({chi}2 Analyses for Categorical Variables)

 

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  		TABLE 3. Predictors of Functional Limitations 12 to 18 Months After OHS (t tests for Continuous Variables)

 
Multiple linear and logistic regression models revealed that preoperative neurodevelopmental status (odds ratio [OR]: 6.0, confidence interval [CI]: 1.9–18.9) and the presence of microcephaly (OR: 6.7, CI: 2.1–21.6) were significantly associated with WeeFIM functional scores (P < .01). The strength of this association was further strengthened using postoperative clinical (OR: 5.1, CI: 1.3–19.3) and head circumference assessments (OR: 5.2, CI: 1.8–14.6) as predictors of functional disability (P < .001). In addition, increasing DHCA time (OR: 1.0, CI: 1.0–1.1), as well as increasing days in the intensive care unit (ICU; OR: 1.0, CI: 1.0–1.2), and lower maternal education (OR: 8.1, CI: 1.2–53.0) were also important risk factors. When controlling for the possible confounding effects of each of these predictor variables, postoperative clinical status, presence of microcephaly before discharge, length of DHCA, and ICU days all remained important determinants (P < .05). Corrective OHS was associated with a better functional outcome than palliative surgery. Using daily living skills for the VABS as the outcome variable, multiple linear, and logistic regression models identified the following significant predictor variables: the presence of microcephaly after surgery (OR: 4.4, CI: 1.0–19.8), increasing age at surgery (OR: 1.1, CI: 1.0–1.3), and lower maternal education (OR: 1.7, CI: 0.5–5.7). Similarly, these regression models revealed 2 factors that were associated with poor socialization skills: postoperative microcephaly (OR: 4.6, CI: 1.5–14.0) and abnormal neurodevelopmental status after surgery (OR: 3.5, CI: 1.3–9.1).


   DISCUSSION
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
REFERENCES
 
Functional outcome measures assist in identifying the degree of dependence or activity restrictions encountered when carrying out everyday tasks in a manner considered to be normal for a person of the same age.16,22 A review of the literature reveals that there is a paucity of pediatric outcome studies to date that have used functional measures. In designing outcome studies, it is essential to characterize developmental as well as functional status, determine health status and quality of life, as well as appreciate the family’s perspective (burden).16,24,30,31 The need for accountability of medical/surgical interventions will drive future policy decisions that will in turn have a profound impact on pediatric health-service delivery, with regards to screening, treatment interventions, and appropriate allocation of services.

Intellectual abilities and neurologic status have received the greatest attention in outcome studies of children with CHD to date. Developmental delay, hypotonia, as well as microcephaly, have been frequently reported.9,10,32,33,34 Studies have consistently reported low average mean intelligence quotients (low- to mid- 90s), and mild language delays, particularly in expressive skills and vocabulary.6,8,9,18,30,32,34,35,36

It is increasingly recognized that functional outcome measurement is fundamental in the evaluation of the impact of novel medical and surgical interventions. However, only 2 studies have reported functional outcomes in children with CHD, and both studies focused specifically on infants with HLHS. Rogers et al (1995)17 examined 11 children with HLHS at a mean age of 38 months (11–67 months). Findings included acquired microcephaly (73%), mental retardation (64%), delayed gross motor development (45%), cognitive deficits (18%), and severe cerebral palsy (18%). In addition, severe functional disabilities (2 standard deviations below the normative mean) were reported in 73% of the cohort using the WeeFIM.17 Kern et al (1998)20 also examined the intelligence and adaptive functioning in children with HLHS and reported a more favorable outcome in 14 children with HLHS <3 years of age. The majority of children with HLHS had at least low-average intelligence, however they scored lower than matched family controls on adaptive functioning using the VABS. The children with HLHS scored lower than controls in each of the areas tested, especially in motor skills. Functional outcomes have not been described in other groups of children with CHD.

Results from our study demonstrate that before and after OHS, neurologic and developmental deficits were common,3,4 and persisted over 1 year later. Ongoing sequelae documented in our cohort have included neurologic abnormalities (41%), microcephaly (30%), gross and fine motor delays (42%), global delays (23%), and behavioral difficulties (35%).37 Functional disabilities were also highly prevalent and included difficulties in socialization and daily living activities. A moderate degree of disability was found in over one third of our cohort, although severe disabilities were documented in only a small minority (6%). Greater dependence in daily tasks undoubtedly places greater burden on caregivers. The potential consequence of persistent functional difficulties within the home, academic milieu, and the community at large deserves additional study.

The findings suggest that these functional measures produce ratings that are similar in certain areas, while different in others. Although the WeeFIM and VABS are measuring similar skill areas (ie, level of functioning and adaptation to daily demands/activities), it is not certain as to whether the specific skills are subsets of the same construct.24 The WeeFIM is designed to measure the construct of functional independence in children and is based on the conceptual framework of the World Health Organization’s International Classification of Impairments, Disabilities, and Handicaps38; however, this does not form the conceptual foundation of the VABS.24 Furthermore, these 2 functional measures quantify skill areas quite differently. The VABS examines a larger number of tasks but uses simple scoring procedures (ie, can/cannot do). In contrast, the WeeFIM assesses selected tasks, and the scoring quantifies the degree of assistance required to execute and complete different tasks. Also, different testers performed each assessment; therefore, this may decrease the strength of the association as well.39 Both assessments provide complementary information, in which the VABS examines a wide range of functional skills, whereas the WeeFIM accounts for the amount of supervision and/or assistance required to be functional in specific tasks. Nonetheless, both tools concur that functional limitations are common problems in young children 12 to 18 months after OHS.

Several studies have correlated medical and surgical factors with developmental outcomes to determine whether specific risk factors enhance risk for disability. At present, emphasis has been primarily placed on intraoperative mechanisms, in particular the prolonged use of DHCA and CPB time as the principle determinant of subsequent neurodevelopmental morbidity.1,6,40 Other factors identified in the literature that may enhance risk include lower preoperative oxygen tension, postoperative cardiac arrest, and abnormal neurodiagnostic tests such as the electroencephalogram or magnetic resonance spectroscopy.18,40,41,42 Socioeconomic status and parental stress have also been shown to modulate the developmental outcome of these children.41,43

There were several important risk factors identified in this study that enhanced the likelihood for functional limitations. First, clinical abnormalities evident before OHS were associated with greater functional disabilities, and this association was more significant with postoperative examination findings. This is likely attributed to the cumulative effects of insults before, during, and after surgical interventions. Nevertheless, baseline neurodevelopmental status is an important marker for subsequent disability. Second, microcephaly before or after surgery was an important determinant. Microcephaly has been associated with a broad spectrum of long-term neurodevelopmental and intellectual deficits in other high-risk populations.44,45 The presence of microcephaly in children with CHD is presumably attributable to congenital brain malformation and/or early acquired brain injury.3,46,47 The association between the length of DHCA and a greater risk for hypoxic-ischemic insult and subsequent neurodevelopmental morbidity has been well-documented both in experimental studies and clinical investigations.1,36,48 Our findings further support this relationship with regards to functional outcomes. A longer duration in the ICU and the need for palliative OHS were both important risk factors. Presumably, prolonged requirements for intensive care, as well as need for stage repair, are associated with a more complex medical course, and therefore a greater risk for brain insult. In fact, the relationship between prolonged intensive care after cardiac surgery and decreased functional capacity has been reported in adults.49 Increasing age at the time of surgery was also related to greater functional limitations. The delay in surgical correction (ie, elective cardiac surgery) may foster an environment whereby the parents, especially mothers, are more anxious, psychologically distressed, and experience greater social dysfunction and less adequate styles of coping.50 Maternal anxiety and overprotectiveness, as well as social and emotional maladjustment have been documented in this population.51,52,53 One could hypothesize that parental overprotectiveness may contribute to greater dependence in functional skills. Another possible explanation may be that functional limitations may become more evident with increasing age, as the child is challenged with more complex activities and task expectations.54 Maternal education was also an important predictor variable for functional disabilities. The relationship between lower socioeconomic status and greater risk for developmental delays in childhood is well-established.55 In summary, our findings would suggest that multiple medical, surgical, and environmental factors play important roles in shaping the outcome of these children.

Our sample includes young infants and, therefore, the long-term significance of these early functional limitations remains to be determined. Long-term follow-up at preschool age in currently underway to address this issue. This study describes a heterogeneous sample with a wide range of CHD. It would therefore be important to further differentiate these outcomes by lesion type. Nonetheless, this study illustrates that as a group these children are clearly at high risk.

Better delineation of the extent and range of functional limitations that children with CHD experience will allow for more effective interventions geared toward maximizing independence in everyday tasks in all environments. Current health care practice does not routinely involve developmental specialists in the early screening and subsequent developmental interventions of this high-risk population. The overall prevalence of functional disabilities in the infant cardiac population is high, although clinically underappreciated at the present time. The provision of adequate rehabilitative, social, and environmental supports will ultimately improve functional outcomes and ease burden of care. Furthermore, early remediation strategies and compensatory techniques may be implemented to increase functional independence and enhance the health and well-being of the child and family.


   ACKNOWLEDGMENTS
 
This study was funded by the National Health Research and Development Program (Health Canada), the Heart and Stroke Foundation, and March of Dimes. Dr Limperopoulos is supported by a studentship, and Dr Shevell and Dr Rohlicek have clinical research scholar awards (chercheur boursier-clinicien), all from Fonds de la recherche en sante du Quebec.

We thank the attending staff of the Division of Newborn Medicine, Dr Marie Beland and Dr Luc Jutras from the Division of Cardiology at the Montreal Children’s Hospital, and Johanne Therrien for their assistance in recruitment of participants. We acknowledge Dr Wood-Dauphinee for her methodological expertise in designing this study, and Dr Harder for assisting with follow-up assessments of a subset of our cohort at the Alberta Children’s Hospital. Special thanks to Lisa Steinbach for coordination of the project, chart reviews, and data entry, as well as the Biostatistical Consultation Service at the Montreal Children’s Hospital for statistical consultation. We are indebted to the families who participated in the study.

The use of the WeeFIM instrument to collect data for this research study was authorized and conducted in accordance with the terms of the special purpose license granted to Licensee by Uniform Data System for Medical Rehabilitation (a division of UB Foundation Activities, Inc, ‘UDSMR’). Licensee has not been trained or certified by UDSMR in the used of the WeeFIM instrument, and the patient data collected during the course of this research study has not been submitted to or processed by UDSMR. No implication is intended that such data has been or will be subjected to UDSMR standard data processing procedures or that it is otherwise comparable to data processed by UDSMR.


   FOOTNOTES
 
Received for publication Mar 22, 2001; Accepted Jun 25, 2001.

Reprint requests to (A.M.) Montreal Children’s Hospital, Neurology A-509, 2300 Tupper St, Montreal, Quebec H3H 1P3. E-mail: annette.majnemer{at}mcgill.ca


   REFERENCES
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
 REFERENCES
 

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PEDIATRICS (ISSN 1098-4275). ©2001 by the American Academy of Pediatrics

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