Criteria abstracted from The Users' Guide to Medical Literature, from the Health Information Research Unit and Clinical Epidemiology and Biostatistics, McMaster University

Highlighted lines and questions below provide links to the pertinent description of criteria in The EBM User's Guide, now available at the Canadian Centres for Health Evidence

Article Reviewed:

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

Culbert EL, Ashburn DA, Cullen-Dean G, et al.

Circulation 2003;108 857-862. [abstract]

Reviewed by David Stockwell MD, JoAnne Natale MD, Children's National Medical Center, Washington, DC

Review posted September 4, 2004

I. What is being studied?:

The study objective:

This study assesses the quality of life of children with transposition of the great arteries (TGA) who received surgical repair during a period of transition in surgical management strategy from atrial inversion to arterial switch operation.

The study design:

Prospective, nonrandomized, cross-sectional survey was mailed to 595 patients in February or June 2000 who had undergone TGA repair from January 1985-March 1989.

The study population:

TGA group: eleven to 15-yr-old (N=306) who completed the CHQ.
Control group: normative population of 10 to 15-yr-olds (N=278) with a similar age but a different ethnic and gender distributions.

The outcomes assessed:

The children's perception of their quality of life was measured using the Child Health Questionnaire (CHQ), an 87 question validated questionnaire of 11 child health concepts, (each scored on a 0 to 100 scale) that measures self-perceived physical and psychosocial well-being of children aged 11 years and older.

II. Are the recommendations valid?

1. Have the investigators measured aspects of patients' lives that patients consider important?

Yes. Although the measured outcomes have not been assessed by children as being important in their lives, surely the defined aspects (physical, emotional, behavioral and social health) have face validity in their application of the survey. The CHQ Version CF-87 appears to encompass all aspects of children's lives - physical, social, emotional, and behavioral. Also many previous quality of life assessment tools have asked parents their interpretation of their child's quality of life. This survey asks the child directly, rather than relying on parental perception.

2. Did the HRQL instrument work in the way it was supposed to?

There seems to be adequate signal (difference between patients) exhibited by the difference in the atrial switch procedure and the arterial switch procedure specifically in the general health perceptions, bodily pain, and self-esteem categories. The patients with the atrial switch or Rastelli procedure noted lower scores (worse CQL scores in these categories) that were statistically significant.

There is no discussion regarding the individual respondent's score and therefore no way to comment on the variability within patients (or the noise).

The investigators refer to the Child Health Questionnaire Version CF-87 (CHQ) as "validated" and provides a reference to the user's manual as the citation for this validation (1). This user's manual is not readily available for further review.

Of the 595 children send a CHQ, 317 (53%) were returned, despite 3 additional attempts to complete follow-up. There is no information provided describing the characteristics of those children who did not return the questionnaire. Therefore, it seems valid to ask if the participants that returned the survey represented the best possible outcomes, and thus selection bias must be considered in the interpretation of these results.

3. Are there important aspects of HRQL that have been omitted?

The child health concepts measured in this questionnaire seem applicable to a child with congenital heart disease, specifically addressing physical health (e.g., presence and extent of physical limitations in self-care, mobility, and strenuous activities attributable to health-related problems), emotional health (e.g., satisfaction with school and athletic ability, appearance, ability to get along with others and family, and life overall along a continuum, frequency of positive and negative states such as anxiety and depression), behavioral health (e.g., overt behavior, such as frequency of behavior problems and ability to get along with others), and social health (e.g., limitations in kind, amount, and performance of schoolwork and activities with friends because of physical, emotional, or behavioral problems).

If there were tradeoffs between quality and quantity of life, or and economic evaluation, have they used the right measure?

This type of analysis was not performed.

III. What are the results?

1. What was the magnitude of the effect on HRQL?

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. For example, CHQ scores ranged from 78.7 ± 13.8 (published norm: 72.7 ± 16.0) for mental health to 93.2 ± 11.3 (published norm 88.8 ± 14.0) for physical functioning. 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.

There are a number of factors that influence the magnitude of effect of TGA on quality of life. First, although statistically significant, the observed effect is small. As the authors state in the discussion, it is not clear if the observed differences are clinically significant. While the magnitude of effect on CHQ cannot be evaluated it is important to note that the TGA group's scores were good. Therefore it seems reasonable to conclude that patients having TGA repair have good expectations of CHQ, with a caveat to the 2.2% of excluded respondents that have some form of developmental delay and the response rate to begin with was only 53%.

Second, given that the TGA and comparison groups differ with respect to gender and ethnic composition, the choice of comparison group may be flawed. The CHQ scores for the TGA group are almost uniformly better than the controls. This information while encouraging is puzzling. This does raise the question again of selection bias. There does not seem to be any medical reason why patients who have had any cardiac surgery would have an improved CHQ score. These findings emphasize the importance of selecting an appropriate comparison group for such a study. For instance, matched sibling controls could have been used.

IV. Will the recommendations help me in caring for my patients?

1. Will the information from the study help me inform my patients?

This article will help in informing parents that their children with TGA have a very reasonable expectation of a meaningful life as far as measurement by HRQL studies.

2. Did the study design simulate clinical practice?

This study actually evaluated clinical practice. Any evaluation of a child with significant congenital heart disease will begin with the presumption that treatment is not usually an option. The child will need to undergo an operation or risk life. From this perspective the reviewed study does simulate clinical practice. There is no way to prospectively evaluate these patients.

References

1. Landgraf JM, Abetz L, Ware JE. Child Health Questionnaire (CHQ): A User's Manual. Boston: The Health Institute, New England Medical Center; 1996.

 

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