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Finer N, Horbar J, Carpenter MS. Pediatrics V2000;105:1082-1089

This paper describes the cognitive and neurologic function of school-aged children who had undergone staged palliation for hypoplastic left heart syndrome (HLHS). A questionnaire was used for all patients at one institution who had undergone palliation. Local patients were given standardized testing. Of the 143 survivors of the Fontan procedure, there were 111 completed questionnaires. The mean age of the children at the time of the survey was 9 +/- 2 years. In the quality of life measures, health was considered excellent in 45%, good in 35%, fair in 12% and poor in 9% of respondents. Thirty-six percent of the children were not taking any medications. Eighty-eight percent of respondents had little or no limitations on activity. Based on the response to the questionnaires, school performance was considered above average in 42%, average in 42% and below average in 16% of respondents. Eighty-two percent of those eligible to do so participated in standardized testing. For this group of 28 patients, the mean age was 8.9 years (range: 6-13.6 years). The median full scale IQ for this group was 86 (range: 50-116). The IQ was below 70 in 5 children (18%) and borderline (70-80) in 10 (36%). Verbal, performance IQ and language measures were also lower than those for normal controls. Neurologic examinations were performed on 23 of the 34 patients who underwent standardized testing. Seventy percent (16) had evidence of ADHD (attention-deficit hyperactivity disorder) by history and physical exam. Four subjects (17%) met criteria for cerebral palsy. Only 3 of the 23 patients were thought to be completely normal with respect to all historical and observational data. In analysis of the data from the study for predictors of neurodevelopmental outcome, preoperative seizures were associated with lower full-scale IQ scores. Neither socioeconomic scale nor length of DHCA were predictors of full scale IQ. Lower math and reading scores on one of the batteries given were associated with longer cumulative CPB times.

Comment: Is the glass half full or half empty? These children are generally behind their peers in intelligence measures, neurodevelopmental findings and health BUT their lives are infinitely richer and more complete than children who have not had the benefit of tertiary cardiac care. Those of us in the cardiac OR should remember that every detail of care could influence both short and long term outcome. Just a few decades ago, HLHS was considered a fatal lesion with no palliation offered to these families. The survivors described in this paper may still have problems to overcome, but with their families and continued excellent care, I predict they will continue to surprise us with their achievements.

Reviewed by: Thomas J. Mancuso, MD, FAAP
Children's Hospital, Boston, MA