Relationship of Surgical Approach to Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome

Abstract

OBJECTIVE. Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS. A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 ± 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS. The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 ± 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS. Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.

Two-Year Survival and Mental and Psychomotor Outcomes After the Norwood Procedure

“…Longer hospitalization was identified previously as an independent predictor of lower full-scale IQ scores in a cohort of school-aged HLHS patients who had undergone either the Norwood procedure or heart transplantation. 42 The present results showed a significant improvement in PDI scores and a lower incidence of psychomotor delay in the RVPA era. Linear regression analysis identified only male sex as an independent predictor of lower psychomotor scores in the MBTS era.…”

Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease

“…10,[15][16][17][18][19][20][21][22] The greatest frequency of adverse outcomes is found among those with a single ventricle with obstruction to systemic outflow, such as hypoplastic left heart syndrome. 23 In this lesion, systemic perfusion occurs through the patent ductus arteriosus, and ductus closure results in shock and end-organ damage. Prenatal diagnosis of hypoplastic left heart syndrome has been reported in certain studies to reduce early neurological morbidity, with fewer adverse perioperative neurological events such as coma, 24 although earlier age at surgery has not been shown to result in better long-term neurodevelopmental outcomes.…”

“…Prenatal diagnosis of hypoplastic left heart syndrome has been reported in certain studies to reduce early neurological morbidity, with fewer adverse perioperative neurological events such as coma, 24 although earlier age at surgery has not been shown to result in better long-term neurodevelopmental outcomes. 23 One could infer that because delayed diagnosis is associated with damage to various end organs, it might also lead to hypoxic/ischemic brain injury; however, further studies are needed to demonstrate a true causal relationship.…”

Developmental Outcomes After Pediatric Heart Transplantation

“…18 More recent reports from single-center and multicenter studies have verified this pattern, that children who received heart transplants generally perform at about a 10-to 15-point deficit compared with nonclinical comparison populations. 16,19,20 Predictors of poorer cognitive function have included birth head circumference, prolonged stay after transplantation, and pre-transplant diagnosis of congenital heart disease (as opposed to cardiomyopathy). The report by Ikle et al 16 noted a correlation between waiting time to transplant and ultimate intelligence quotient (IQ) scores in a small sample of infants with HLHS, with a 4.5 IQ point decrease for each month waiting for surgery.…”