TY - JOUR
T1 - Functional health status of adolescents after the Fontan procedure - Comparison with their siblings
AU - Manlhiot, Cedric
AU - Knezevich, Stevan
AU - Radojewski, Elizabeth
AU - Cullen-Dean, Geraldine
AU - Williams, William G.
AU - McCrindle, Brian W.
PY - 2009
Y1 - 2009
N2 - Background: Studies have suggested that patients who have undergone the Fontan procedure experience lower functional health status and diminished exercise capacity compared with other children. Objectives: To compare the functional health status of Fontan patients with and without siblings, assess whether there are any differences between Fontan patients and their siblings, and determine associated factors. Methods: A cross-sectional, single-centre, observational study was performed on Fontan patients 10 to 20 years of age, and their sibling closest in age, followed in a tertiary pediatric hospital. Functional health status was measured by the Child Health Questionnaire Child Form and the Pediatric Quality of Life, Inventory. Results: A total of 68 patients and 38 siblings were enrolled. Patients with siblings scored significantly lower on numerous domains of physical functional status than those without siblings. Compared with their matched siblings, Fontan patients reported significantly lower scores in all domains of the Pediatric Quality of Life Inventory and on physical (but not psychosocial) domains of the Child Health Questionnaire Child Form. Factors associated with increased patient-sibling differences included younger patient age, female sex, intracardiac lateral tunnel Fontan connection and lower ejection fraction at the time of study enrollment. Conclusions: Adolescents with Fontan physiology reported a lower functional health status in physical domains than their siblings, but had similar status in psychosocial domains. Having a sibling was associated with lower reported functional health status, suggesting an important effect of self-perceived physical limitations over true limitations.
AB - Background: Studies have suggested that patients who have undergone the Fontan procedure experience lower functional health status and diminished exercise capacity compared with other children. Objectives: To compare the functional health status of Fontan patients with and without siblings, assess whether there are any differences between Fontan patients and their siblings, and determine associated factors. Methods: A cross-sectional, single-centre, observational study was performed on Fontan patients 10 to 20 years of age, and their sibling closest in age, followed in a tertiary pediatric hospital. Functional health status was measured by the Child Health Questionnaire Child Form and the Pediatric Quality of Life, Inventory. Results: A total of 68 patients and 38 siblings were enrolled. Patients with siblings scored significantly lower on numerous domains of physical functional status than those without siblings. Compared with their matched siblings, Fontan patients reported significantly lower scores in all domains of the Pediatric Quality of Life Inventory and on physical (but not psychosocial) domains of the Child Health Questionnaire Child Form. Factors associated with increased patient-sibling differences included younger patient age, female sex, intracardiac lateral tunnel Fontan connection and lower ejection fraction at the time of study enrollment. Conclusions: Adolescents with Fontan physiology reported a lower functional health status in physical domains than their siblings, but had similar status in psychosocial domains. Having a sibling was associated with lower reported functional health status, suggesting an important effect of self-perceived physical limitations over true limitations.
KW - Congenital heart disease
KW - Fontan procedure
KW - Functional health status
KW - Quality of life
KW - Single ventricle
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U2 - 10.1016/s0828-282x(09)70139-4
DO - 10.1016/s0828-282x(09)70139-4
M3 - Article
C2 - 19746247
AN - SCOPUS:70349561833
SN - 0828-282X
VL - 25
SP - S294-S300
JO - Canadian Journal of Cardiology
JF - Canadian Journal of Cardiology
IS - 9
ER -