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Published on: May 11, 2015
Growth in children with pulmonary arterial hypertension: a longitudinal retrospective multiregistry study
Mark-Jan Ploegstra1, D Dunbar Ivy2, Jeremy G Wheeler3
1Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.
Insights
Children with pulmonary arterial hypertension (PAH) show impaired growth, particularly younger children and those with congenital heart disease. Favorable clinical course correlates with catch-up growth, making height a useful monitoring parameter.
Area of Science:
- Pediatric Cardiology
- Growth Monitoring
- Pulmonary Arterial Hypertension
Background:
- Pulmonary arterial hypertension (PAH) impacts child development.
- Accurate interpretation of growth measurements is crucial for managing pediatric PAH.
Purpose of the Study:
- To assess growth patterns in children with PAH.
- To identify determinants associated with growth in pediatric PAH.
Main Methods:
- Retrospective longitudinal study using pooled data from 4 prospective pediatric PAH registries.
- Analysis of height and body-mass index (BMI) Z scores against WHO growth standards.
- Multivariable linear mixed-effects modeling to identify associated factors.
Main Results:
- Children with PAH exhibited lower mean height and BMI Z scores compared to WHO standards.
- Impaired growth was more pronounced in younger children (≤5 years) and those with PAH associated with congenital heart disease.
- Age, PAH cause, prematurity, WHO functional class, trisomy 21, and time since diagnosis influenced height Z scores.
Conclusions:
- PAH is linked to growth impairment, especially in specific pediatric subgroups.
- Growth deficits are associated with PAH etiology, comorbidities, disease severity, and duration.
- Height for age can serve as a valuable, globally accessible clinical parameter for monitoring patient status.
Background:
To enable adequate interpretation of growth measurements in the management of children with pulmonary arterial hypertension (PAH), we assessed growth and its associated determinants in children with PAH.
Methods:
We did a retrospective longitudinal study of height and body-mass index in reference to WHO growth standards by pooling data from four contemporary prospective registries of paediatric PAH representing 53 centres in 19 countries. The main outcome measures were median height for age and body-mass index for age percentiles and longitudinal deviation of height for age and body-mass index for age Z scores from WHO standards.
Findings:
601 children were followed up for a median of 2·9 years (IQR 1·5-4·4). Baseline median height for age percentile was 26 (IQR 4-54) and baseline median body-mass index for age percentile was 41 (IQR 12-79). Mean height for age Z score was significantly lower than the reference (-0·81, 95% CI -0·93 to -0·69; p<0·0001), as was body-mass index for age Z score (-0·12, -0·25 to -0·01; p=0·047). Height for age Z score was particularly decreased in young patients (aged ≤5 years) with idiopathic or hereditary PAH and in all patients with PAH associated with congenital heart disease. Although Z scores increased in some patients and decreased in others, we detected no significant trend in height for age Z score (p=0·57) or body-mass index for age Z score (p=0·48) before taking account of covariates. Multivariable linear mixed effects modelling showed that age, cause of PAH, ex-prematurity, WHO functional class, trisomy 21, and time since diagnosis were associated with height for age Z score, whereas age, ethnicity, and trisomy 21 were associated with body-mass index for age Z score. A favourable WHO functional class course was independently associated with increases in height for age Z score.
Interpretation:
PAH is associated with impaired growth, especially in younger children and those with pulmonary arterial hypertension associated with congenital heart disease. The degree of impairment is independently associated with cause of PAH and comorbidities, but also with disease severity and duration. Because a favourable clinical course was associated with catch-up growth, height for age could serve as an additional and globally available clinical parameter to monitor patients' clinical condition.
Funding:
Actelion Pharmaceuticals.
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