At the age of 52 weeks, the patients with HLHS had lower scores than the control group in all 4 AIMS subscales (P < .001), whereas the patients with UVH differed from the controls only in the prone and standing subscales (P < .001). There was no statistically significant difference between the patients with HLHS and UVH (Table 4).
The AIMS scores of controls and the patients with HLHS or UVH were proportioned to the standardized 50–percentage percentile ranks of the AIMS test. The values of patients with HLHS/UVH percentile ranks were 15% lower than the corresponding values of control group. The median of the controls (interquartile range) was 1.00 (0.96-1.05) and the median of patients with HLHS or UVH was 0.85 (0.47-0.96).
In the prone position, 46% of patients and 94% of controls achieved the maximum level of prone subscale scores. Age-adjusted risk ratio was 2.2 (95% CI: 1.5-3.1, P < .001). In the supine position, 71% of patients and 100% of controls achieved the maximum level of supine subscale scores. The age-adjusted risk ratio was 1.5 (95% CI: 1.2-1.8, P < .001).
In the sitting position, 69% of patients and 96% of controls reached the maximum level of sitting subscale scores. The age-adjusted risk ratio was 1.5 (95% CI: 1.2-1.8, P < .001). Nine percent of patients and 26% of controls acquired the highest level of standing subscale scores. The age-adjusted risk ratio was 5.0 (95% CI: 3.2-8.0, P < .001) (Figure 4).
In our study, the motor development of patients with HLHS or UVH was found to be delayed during the first year of life as has been demonstrated in other studies.9,17,18,20,21 According to Sarajuuri et al, the motor skills of children with UVH and HLHS were significantly lower than for control subjects at the age of 1 year.13 Also, they found psychomotor development of patients with HLHS and with UVH was significantly inferior to that of control subjects at the age of 30.2 months.34 The total AIMS scores were similarly inferior in patients with HLHS and UVH in our study. Patients were at risk for motor delay because of impaired fetal, pre- and perioperative cerebral blood flow. Also, the immobilization related to several exhausting operations during the first year of life might have influenced the child's motor development. There are also studies indicating that parental overprotection may hinder a child's motor development.35,36
In this study, we present a detailed analysis of motor development of patients with HLHS/UVH during the first year of life by comparing the motor development of these children with controls who were healthy using the 4 AIMS subscales (prone, supine, sitting, and standing). At the age of 16 weeks, the difference between patients with HLHS and the controls was smaller than that between patients with UVH and the controls. At the age of 52 weeks, patients with HLHS differed in all AIMS subscale scores from controls whereas patients with UVH differed only in the prone and standing subscale scores. All patients with HLHS and 5 patients with UVH went through 2 phases of the Norwood operations during this assessment period. This fact was associated with the components of delay in the early motor development of patients. The prone subscale was the most demanding for both HLHS and UVH groups in both observations. Only half of the patients reached the maximum scores in the prone subscale at the age of 52 weeks. By this age, children are supposed to attain the skills in the prone subscale. Also, the standing subscale appeared to be more demanding for patients at the age of 52 weeks compared with healthy controls. Almost all the controls reached the maximum scores in all AIMS subscales except in the standing subscale at the age of 52 weeks.
Because of several exhausting operations and the recommendation not to lie in the prone position during the first month postoperatively, patients with HLHS/UVH were not able to practice age-related motor skills such as upper extremity weight-bearing, weight shift, rotations, and head and trunk control against gravity. Normally, these particular skills appear during the first months of life. The prone position is a crucial element for the development of antigravity movements and upright trunk control.32 According to Dudek-Shriber et al,32 4-month-old infants, who spent less than 30 minutes per day in prone, were intolerant of the prone position. Prone positioning appears to be associated with infant's acquisition of motor milestones and the quality of motor development. Prone positioning provides a basis for the development of more advanced skills.37 Even sleeping position has an effect on the early motor development of the infant. Prone sleepers attain several motor milestones earlier than supine sleepers.38–40
In the supine and sitting subscales, the difference between patients and controls was not as remarkable as that observed in the prone and standing subscales. One third of the patients did not attain the maximum scores in the supine and sitting subscales at the age of 52 weeks. To reach the maximum scores in the supine and sitting subscales, children should be able to roll from supine to prone with rotation, have trunk control for independent sitting, and should easily be able to move in and out of the sitting position. Sitting skills require trunk extension and upper extremity weight-bearing, which is hypothesized to be facilitated in the prone position. Infants, who are provided with the opportunity to be in the prone position, appear to be able to develop movement skills and weight-bearing patterns against gravity which support the attainment of prone milestones and also milestones in the sitting and standing positions.38–40
In this study, only 9% of patients and 26% of infants in the control group reached the maximum AIMS score in the standing subscale at the age of 52 weeks. The reason why only 26% of controls reached the maximum scores in the standing subscale might be influenced by the fact that the assessment was carried out at the time when the infants were in the process of developing standing and independent walking skills. Among children that are typically developing, there is also large variation in milestone acquisition. Some children are not able to stand alone until an average age of 16.9 months and walk alone until an average age of 17.6 months.24 During the process of learning to stand independently, infants must learn to balance within significantly reduced stability limits compared with those used, for example, during sitting, and to control many additional degrees of freedom as they add coordination of the leg and thigh segments to those of the trunk and head.41 Patients with HLHS or UVH had obviously fewer opportunities to practice their motor skills in different positions with the effect most conspicuous in the standing subscale.
The results of this study indicate that the AIMS is a reliable and valid method that can be used for the evaluation and discrimination of the motor development of typically developing children and children with severe heart defects. However, according to our study, there is no statistically significant difference between the early standing skills of patients and controls at the age of 16 weeks in contrast to the standing skills of the groups at the age of 52 weeks. In the AIMS, there are 16 different items to assess standing skills. Most of these items are focused on the infant's ability to reach the standing position and the ability to walk with and without support, whereas there are only 2 to 3 items focused on early standing skills. We argue that including more items assessing early standing skills would improve discrimination between typical and delayed motor development. The AIMS, however, is an appropriate assessment tool that can help to detect the need for physical therapy and determine its timing.
A limitation of this study was that blinding was unsuccessful especially in the first observation because of symptoms and obvious visible signs in the appearance of the patients.
The results of this study provide clinically important information for physical therapists who are expected to evaluate young children and accurately determine whether an infant is eligible for early intervention.
The results of this study indicate that the motor development of children with HLHS or UVH is delayed. Motor development is most delayed in the prone and standing positions. Our results indicate that the AIMS provides a reliable measurement that can be used for the evaluation of motor performance of patients with HLHS or UVH.
The authors thank Ritva Haajanen, PT, for her valuable work in the motor assessment of these children. They also thank Hannu Kautiainen, BA, and Salme Järvenpää, MSc, for statistical advice and reviewing the statistical analysis of this study.
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