Cognitive Development After the Fontan Operation

Abstract

Background-Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure. Methods and Results-A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3.7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7Ϯ17.4 (PϽ0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores Ͻ70 (Pϭ0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (Pϭ0.002), the anatomic diagnoses of hypoplastic left heart syndrome (PϽ0.001) and "other complex" (Pϭ0.05), and prior placement of a pulmonary artery band (Pϭ0.04). Mean composite achievement score was 91.6Ϯ15.4 (PϽ0.001 versus normal); 14 patients (10.8%) scored Ͻ70 (PϽ0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (Pϭ0.004) and "other complex" (Pϭ0.003) or prior use of circulatory arrest (Pϭ0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (Pϭ0.01). Conclusions-Most

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

“…Such variables may include home and school environment, economic status, and genetic or geographic factors. In a study of cognitive development after Fontan operation by Wernovsky et al, 16 surgical and diagnostic factors explained 6% of variation in achievement and IQ scores. The addition of socioeconomic status to their analysis increased explained variation nearly 4-fold.…”

Health-related quality of life: A closer look at related research in patients who have undergone the Fontan operation over the last decade

“…The subgroup of those with HLHS were noted to have a higher risk for neurodevelopmental deficits associated with prolonged periods of DHCA, congenital brain abnormalities, or hypoxic-ischemic insult. 4,5,8,38,39 The majority of the studies reviewed sampled the population who underwent the Fontan operation in the late 1970s and early 1990s. Patient selection and surgical techniques, however, have advanced over the past decade.…”

“…The majority of authors identified similar neurodevelopmental outcomes, but variations were noted depending on the severity of the SV anatomy and sample selection. 4,5,7,8 Uzark et al 8 provided one of the first neurodevelopmental outcome studies in the population who have undergone the Fontan operation. Neurodevelopmental testing in patients 2 to 16 years of age found that the majority of children had intellectual function within the normal range.…”

“…[4][5]7,8,10 In addition, the patients with HLHS who had deep hypothermic circulatory arrest during their first-stage surgical palliation were noted to have worse outcomes. 5,7,8 Mahle et al 4 evaluated outcomes in school-aged adolescents with HLHS and identified IQ scores within normal range with mean performance of the group lower than that of the general population. Of note, the majority of parents or guardians in this study described their child's health as good (34%) or excellent (45%) and their academic performance as average (42%) or above average (42%).…”

“…Past research in patients with SV CHD has focused on functional and neurodevelopmental outcomes producing a growing body of literature with documented deficits that seem to be multifactoral. [4][5][6][7][8][9][10] However, little is known about the other determinants of HRQOL such as social well-being and function. Moreover, earlier studies in this population had identified the perceptions of family members rather than the actual self-perceived HRQOL of the individual, who may not have been of sufficient age to participate in the evaluation.…”