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

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Functional Limitations in Young Children With Congenital Heart Defects

After Cardiac Surgery

Catherine Limperopoulos, OT, MSc*; Annette Majnemer, OT, PhD*‡储; Michael I. Shevell, MD, CM, FRCP(C)‡储; Bernard Rosenblatt, MD, CM, FRCP(C)*‡储; Charles Rohlicek, MD, CM, PhD, FRCP(C)储; Christo Tchervenkov, MD, CM, FRCS(C)§; and

H. Z. Darwish, MD, FRCP(C)¶

ABSTRACT. With the recent dramatic decline in mor-tality rates of infants undergoing open-heart surgery (OHS), there is growing concern regarding neurodevel-opmental sequelae. Outcome studies have primarily fo-cused on delineating developmental impairments; how-ever, 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 in-fants with CHD undergoing their first OHS were re-cruited prospectively. Patients were assessed pre- and postoperatively, and again 12 to 18 months after surgery. Functional assessments included the WeeFIM (Function-al Independence Measure) and the Vineland Adaptive Behavior Scale.

Results. For the WeeFIM, mean quotients were 84.323.8 (self-care), 77.230.0 (mobility), and 92.427.8 (cognition), with an overall quotient of 83.823.4. 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 Vine-land scale, mean score for daily living skills was 84.417.6, and 80.315.9 for socialization. Functional diffi-culties in daily living skills were documented in 40%, whereas >1/2 had poor socialization skills. Factors en-hancing risk for functional disabilities included periop-erative 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 limi-tations and dependence in activities of daily living is currently underappreciated in the clinical setting, and deserves additional attention by pediatricians and devel-opmental specialists. Pediatrics2001;108:1325–1331; con-genital heart defects, cardiac surgery, outcome, function.

ABBREVIATIONS. OHS, open-heart surgery; CHD, congenital heart defects; CPB, cardiopulmonary bypass; DHCA, deep

hypo-thermic circulatory arrest; HLHS, hypoplastic left heart syndrome; VABS, Vineland Adaptive Behavior Scale; OR, odds ratio; CI, confidence interval; ICU, intensive care unit.

A

s mortality rates after infant open-heart sur-gery (OHS) continue to dramatically decline, the neurodevelopmental outcome of survi-vors has come under increasing scrutiny. Acutely, there is increasing electrophysiological and clinical evidence of brain injury in young infants undergoing surgical correction.1– 4 It is as yet unclear whether acute neurodevelopmental abnormalities are tran-sient or persist long-term. The long-term outcome of children with congenital heart defects (CHD) after early surgical repair has primarily focused on neu-rologic status and cognitive impairments.5 Severe neurodevelopmental sequelae, such as cerebral palsy and mental retardation, are uncommon. Overall, ex-isting evidence would suggest that global develop-mental deficits are common across the developmen-tal spectrum, particularly in gross and fine motor skills, language, reasoning, and behavioral difficul-ties.6 –10Educational difficulties and a need for spe-cial classroom placement or individualized instruc-tion as a result of learning disabilities and atteninstruc-tional problems also seem to be common in this high-risk population.11–13It is critical to define developmental deficits across all domains to target which rehabili-tation professionals are needed for specific interven-tional purposes. However, the extent and nature of neurodevelopmental deficits/impairments and con-comitant 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 –16 It is im-portant to measure function given that even if a child is found to have a poor motor or intelligence quo-tient, this does not necessarily inform us of the im-pact of such impairments on overall functioning. The impact of the spectrum of neurodevelopmental im-pairments 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 underex-plored in children with CHD. Moreover, the associ-ation between current medical and surgical interven-tions on family and child functioning and family burden needs to be addressed to best meet the on-From the *School of Physical and Occupational Therapy, Departments of

‡Neurology and Neurosurgery, §Cardiovascular and Thoracic Surgery, and

储Pediatrics, McGill University, Montreal Children’s Hospital, Montreal, Quebec, Canada; and ¶Division of Pediatric Neurology, Alberta Children’s Hospital, Calgary, Alberta, Canada.

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@ mcgill.ca

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going 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 disabil-ity.

METHODS

A consecutive series of young children with CHD who under-went 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.4Participants 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 follow-ing: 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 neurodevel-opmental disability (eg, Down syndrome, DiGeorge syndrome). Children diagnosed with hypoplastic left heart syndrome (HLHS) were also excluded, as the literature has suggested a higher prev-alence of neurologic morbidity in this subgroup of children.17–20

Procedures

Twelve to 18 months after OHS, evaluations were conducted when convenient for the families at the Montreal Children’s Hos-pital. 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 as-sessments were administered according to the established proto-cols for each instrument. The evaluators were not directly in-volved 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 pre-operative and postpre-operative 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

sur-gery (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, neurodevelop-mental status and head circumference measurements (⬍5th per-centile) that were documented before and after OHS were exam-ined (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), persist-ing cyanosis, and ongopersist-ing 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 com-plete 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 indepen-dence. 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 prob-lems 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.23–25

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 encom-passes scores below 50 (Msall, personal communication, Septem-ber 21, 2000). The latter would include children who are essen-tially fully dependent in self-care and mobility.

The VABS was also completed by parent interview. This as-sessment is a discriminative measure that records adaptive behav-ior 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 na-tional standardization samples of handicapped and nonhandi-capped individuals.26The standard version of the VABS consists

of 301 items. The scoring options include the following: the activ-ity is never performed (0 points), the activactiv-ity is sometimes per-formed or perper-formed with partial success (1 point), the activity is usually or habitually performed (2 points), or the interview re-spondent 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 appropri-ately 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.23,24,27–29Age-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 propor-tion of participants with abnormal scores on the funcpropor-tional mea-sures 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 (␹2for

categorical variables andttests for continuous data) and multi-variate regression analyses were applied to determine the possible associations between risk factors (as outlined in the procedures above) and functional outcomes.

RESULTS Sample Characteristics

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the cohort underwent corrective OHS, whereas 16% had palliative procedures. Of the 20 children that did not have follow-up evaluations, 13 were from out-side of the Montreal Metropolitan area, 4 could not be reached, and 3 were no longer interested in par-ticipating in the study. Comparisons between those participants that did not return for follow-up testing and those that did demonstrated no significant dif-ferences on baseline variables (eg, preoperative and acute postoperative neurodevelopmental testing, length of CPB and DHCA, low preoperative oxygen saturations [⬍85], cyanotic vs acyanotic CHD, pallia-tive vs correcpallia-tive 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 con-ducted on all participants, depending on the avail-ability 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), ventricu-lar septal defect (13), transposition of the great arter-ies (10), double outlet right ventricle with subaortic ventricular septal defect (9), atrioventricular septal defect (8), including 1 with severe pulmonary steno-sis, 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 hy-poplasia, 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 medica-tions 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 function-ing 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).

The VABS was administered to 80 participants. The mean scores for both subdomains were shifted downwards approximately 1 standard deviation (Ta-ble 1). Forty percent (32/80) of children demon-strated 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’ perfor-mance 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 func-tional outcomes (Table 2 and 3). For the WeeFIM at baseline, there was a significant relationship between preoperative and acute postoperative neurodevelop-mental abnormalities and the presence of microceph-aly, time spent in intensive care, length of hospital-ization, and palliative procedures, with subsequent functional difficulties. At follow-up testing, persist-ing 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, increas-ing age at surgery was associated with greater func-tional limitations. There was a significant association

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

Deviation

Median Range

VABS (Daily Living Skills) 84.4 17.7 85 25–144

VABS (Socialization) 80.3 15.9 76 36–116

WeeFIM (Self-care) 84.3 23.8 86 21–126

WeeFIM (Mobility) 77.2 30.4 80 16–146

WeeFIM (Cognition) 92.4 27.7 96 24–188

WeeFIM (Total Score) 83.8 23.4 87 20–132

The WeeFIM instrument. Copyright © 2000 Uniform Data System for Medical Rehabilitation (UDSMR). All rights reserved. Used with

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between acute postoperative neurodevelopmental findings, microcephaly, increasing age at surgery, and subsequent admissions and socialization diffi-culties on the VABS. There was no significant asso-ciation between maternal or paternal education and functional outcomes.

Multiple linear and logistic regression models re-vealed 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 as-sociation was further strengthened using postopera-tive clinical (OR: 5.1, CI: 1.3–19.3) and head circum-ference assessments (OR: 5.2, CI: 1.8 –14.6) as predictors of functional disability (P ⬍ .001). In ad-dition, 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 edu-cation (OR: 8.1, CI: 1.2–53.0) were also important risk factors. When controlling for the possible confound-ing effects of each of these predictor variables, post-operative clinical status, presence of microcephaly before discharge, length of DHCA, and ICU days all remained important determinants (P⬍.05). Correc-tive 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 pres-ence 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 sur-gery (OR: 3.5, CI: 1.3–9.1).

DISCUSSION

Functional outcome measures assist in identifying the degree of dependence or activity restrictions en-countered when carrying out everyday tasks in a manner considered to be normal for a person of the same age.16,22A 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 devel-opmental 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 inter-ventions, and appropriate allocation of services.

Intellectual abilities and neurologic status have re-ceived the greatest attention in outcome studies of

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

Risk Factors Functional Outcomes (PValues) WeeFIM

Total Score

VABS Daily Living Skills

VABS Socialization Skills

Preoperative status .003 NS NS

Preoperative microcephaly .002 NS NS

Postoperative status .0001 NS .01

Postoperative microcephaly .002 NS .01

Corrective versus palliative OHS .003 NS NS

Cyanotic versus acyanotic CHD NS NS NS

Oxygen saturation⬍85 preoperatively NS NS NS

Persisting cyanosis (at follow-up testing) .005 NS NS

Medications (at follow-up testing) .008 NS NS

Maternal education NS NS NS

Paternal education NS NS NS

The WeeFIM instrument. Copyright © 2000 Uniform Data System for Medical Rehabilitation (UDSMR). All rights reserved. Used with

permission of UDSMR, University of Buffalo, 232 Parker Hall, 3435 Main Street, Buffalo, NY 14214.

NS indicates not significant.

TABLE 3. Predictors of Functional Limitations 12 to 18 Months After OHS (ttests for Continuous Variables) Risk Factors Functional Outcomes (PValues)

WeeFIM Total Score

VABS Daily Living Skills

VABS Socialization Skills

CPB NS NS NS

DHCA NS NS NS

Age at surgery .02 .02 .04

Intensive care .007 NS NS

Length of hospitalization .001 NS NS

Subsequent admissions .03 NS .04

Subsequent surgeries .01 NS NS

Weight (at follow-up testing) .04 NS NS

Height (at follow-up testing) NS NS NS

The WeeFIM instrument. Copyright © 2000 Uniform Data System for Medical Rehabilitation (UDSMR). All rights reserved. Used with

permission of UDSMR, University of Buffalo, 232 Parker Hall, 3435 Main Street, Buffalo, NY 14214.

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children with CHD to date. Developmental delay, hy-potonia, as well as microcephaly, have been frequently reported.9,10,32–34 Studies have consistently reported low average mean intelligence quotients (low- to mid-90s), and mild language delays, particularly in expres-sive skills and vocabulary.6,8,9,18,30,32,34 –36

It is increasingly recognized that functional out-come measurement is fundamental in the evaluation of the impact of novel medical and surgical interven-tions. However, only 2 studies have reported func-tional 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 de-velopment (45%), cognitive deficits (18%), and severe cerebral palsy (18%). In addition, severe functional disabilities (2 standard deviations below the norma-tive mean) were reported in 73% of the cohort using the WeeFIM.17Kern et al (1998)20also 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 us-ing 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 de-scribed in other groups of children with CHD.

Results from our study demonstrate that before and after OHS, neurologic and developmental defi-cits were common,3,4and persisted over 1 year later. Ongoing sequelae documented in our cohort have included neurologic abnormalities (41%), microceph-aly (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 se-vere 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 mea-sures produce ratings that are similar in certain ar-eas, 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 Or-ganization’s International Classification of Impair-ments, 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 ex-amines 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 exe-cute and complete different tasks. Also, different testers performed each assessment; therefore, this may decrease the strength of the association as well.39Both assessments provide complementary in-formation, in which the VABS examines a wide range of functional skills, whereas the WeeFIM ac-counts for the amount of supervision and/or assis-tance required to be functional in specific tasks. Nonetheless, both tools concur that functional limi-tations are common problems in young children 12 to 18 months after OHS.

Several studies have correlated medical and surgi-cal factors with developmental outcomes to deter-mine 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 neurodevelop-mental morbidity.1,6,40Other factors identified in the literature that may enhance risk include lower pre-operative oxygen tension, postpre-operative cardiac ar-rest, and abnormal neurodiagnostic tests such as the electroencephalogram or magnetic resonance spec-troscopy.18,40 – 42 Socioeconomic status and parental stress have also been shown to modulate the devel-opmental outcome of these children.41,43

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an environment whereby the parents, especially mothers, are more anxious, psychologically dis-tressed, and experience greater social dysfunction and less adequate styles of coping.50 Maternal anxi-ety and overprotectiveness, as well as social and emotional maladjustment have been documented in this population.51–53One could hypothesize that pa-rental overprotectiveness may contribute to greater dependence in functional skills. Another possible ex-planation may be that functional limitations may become more evident with increasing age, as the child is challenged with more complex activities and task expectations.54Maternal education was also an important predictor variable for functional disabili-ties. 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, surgi-cal, 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 fol-low-up at preschool age in currently underway to address this issue. This study describes a heteroge-neous 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 func-tional 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 over-all prevalence of functional disabilities in the infant cardiac population is high, although clinically un-derappreciated at the present time. The provision of adequate rehabilitative, social, and environmental supports will ultimately improve functional out-comes and ease burden of care. Furthermore, early remediation strategies and compensatory techniques may be implemented to increase functional indepen-dence 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 Med-icine, 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 exper-tise 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 coordi-nation of the project, chart reviews, and data entry, as well as the Biostatistical Consultation Service at the Montreal Children’s

Hos-pital 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 UDSMRin the used of the WeeFIM

instru-ment, 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 UDSMRstandard data processing procedures or that it

is otherwise comparable to data processed by UDSMR.

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LIMITED CHOICE FOR CARE

“The poor . . . cannot choose the form of their care; the most they can do is resist the medical establishment’s attempt to deprive them of treatment. They might have added that in the context of managed care, where the large majority of Americans are covered by the plans their employers choose, it is not only the poor but most of us who find medical choices seriously limited by the decisions of others. Further . . . when someone suffers from a disease, choice is not the key issue; it is rather how one deals with pain, disability, and events over which one has no control.”

Dorff EN. A narrow ridge, a larger vision.Hastings Cent Rep.May-June 2001

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DOI: 10.1542/peds.108.6.1325

2001;108;1325

Pediatrics

Charles Rohlicek, Christo Tchervenkov and H.Z. Darwish

Catherine Limperopoulos, Annette Majnemer, Michael I. Shevell, Bernard Rosenblatt,

Cardiac Surgery

Functional Limitations in Young Children With Congenital Heart Defects After

Services

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(9)

DOI: 10.1542/peds.108.6.1325

2001;108;1325

Pediatrics

Charles Rohlicek, Christo Tchervenkov and H.Z. Darwish

Catherine Limperopoulos, Annette Majnemer, Michael I. Shevell, Bernard Rosenblatt,

Cardiac Surgery

Functional Limitations in Young Children With Congenital Heart Defects After

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located on the World Wide Web at:

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Figure

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

TABLE 1.Means,

Standard Deviations, Medians, and Ranges on the WeeFIM and VABS p.3
TABLE 2.Predictors of Functional Limitations 12 to 18 Months After OHS (Risk Factors

TABLE 2.Predictors

of Functional Limitations 12 to 18 Months After OHS (Risk Factors p.4
TABLE 3.Predictors of Functional Limitations 12 to 18 Months After OHS (t tests for Continuous Variables)

TABLE 3.Predictors

of Functional Limitations 12 to 18 Months After OHS (t tests for Continuous Variables) p.4

References