Published Online
on August 4, 2003

A more recent version of this article appeared on August 19, 2003

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Submitted on March 5, 2003
Revised on May 29, 2003
Accepted on May 29, 2003

Quality of Life of Children After Repair of Transposition of the Great Arteries

Erin L. Culbert MD, David A. Ashburn MD, Geraldine Cullen-Dean RN, MN, Jay A. Joseph MSc, William G. Williams MD^*, Eugene H. Blackstone MD, Brian W. McCrindle MD, MPH, and and the Congenital Heart Surgeons Society

From the Congenital Heart Surgeons Society Data Center (E.L.C., D.A.A., G.C.-D., J.A.J., W.G.W., B.W.M.), Division of Cardiovascular Surgery (D.A.A., W.G.W.), and Division of Cardiology (B.W.M.), Hospital for Sick Children, University of Toronto, Ontario, Canada, and Department of Thoracic and Cardiovascular Surgery and Department of Biostatistics and Epidemiology (E.H.B.), Cleveland Clinic Foundation, Cleveland, Ohio.

^* To whom correspondence should be addressed. E-mail: bill.williams{at}sickkids.ca.

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.

Key words: heart defects • congenital • transposition of great vessels • pediatrics • quality of life

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