Background— We sought to assess quality of life of children with transposition of the great arteries (TGA) enrolled during transition in management strategy from atrial to arterial switch operation. Methods and Results— Neonates enrolled by the Congenital Heart Surgeons Society in a prospective study of TGA between 1985 and 1989 were eligible. A Child Health Questionnaire was sent for completion by the child between February and June 2000. Data were compared with published normative values. Child Health Questionnaires were completed by 306 of 708 survivors at a mean age of 13±1 years. Diagnosis included TGA (n=202, 66%), TGA/ventricular septal defect (VSD) (n=84, 27%), and TGA/VSD/pulmonary stenosis (n=20, 7%). Repair type was arterial switch (n=189, 62%), atrial switch (n=105, 34%; Senning=58, Mustard=47), or Rastelli (n=12, 4%). Children with TGA scored significantly higher than published norms in all categories except self-esteem. TGA/VSD/pulmonary stenosis was associated with lower scores than TGA and TGA/VSD in physical functioning ( P =0.002), general health perceptions ( P =0.012), and mental health ( P =0.048). Arterial repair was associated with higher scores than atrial or Rastelli repair in physical functioning ( P <0.001), pain ( P =0.004), mental health ( P =0.019), self-esteem ( P =0.004), and general health perceptions ( P <0.001). By multivariable analyses, the most common independent factors impacting scores were repair type, perfusion parameters, and gender. Conclusions— Quality of life and health status as perceived by children 11 to 15 years after TGA repair is excellent when compared with published normative data and is better after arterial switch operation than after atrial repair.

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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.

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