Early Developmental Outcome After the Norwood Procedure for Hypoplastic Left Heart Syndrome

Kern, Jeffrey H.; Hinton, Veronica J.; Nereo, Nancy; Hayes, Constance J.; Gersony, Welton M.

doi:10.1542/peds.102.5.1148

Cited by 137 publications

(76 citation statements)

References 20 publications

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“…As in other reports, 29,32 the diagnosis of HLHS was a risk factor for poor outcome in this series; however, the number of patients was exceedingly small (nϭ5), and the effects of diagnosis were confounded by use of circulatory arrest in this group. Kern and colleagues 33 reported a negative correlation between the duration of circulatory arrest during stage I reconstruction (mean, 56 minutes) and full-scale IQ. In our series, these patients represent an early portion of the learning curve with surgical management of this disease.…”

Section: Discussionmentioning

confidence: 99%

Cognitive development after the Fontan operation

Wernovsky

Tiles

Gauvreau

2001

ACC Current Journal Review

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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

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Section: Discussionmentioning

confidence: 99%

Cognitive development after the Fontan operation

Wernovsky

Tiles

Gauvreau

2001

ACC Current Journal Review

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“…[37][38][39][40] One study showed the duration of DHCA to be inversely associated with performance IQ in a small cohort of 14 patients. 39 For children after Norwood MBTS surgery, a strong correlation has been found between the MDI at 18 to 24 months of age and full-scale intelligence scores at 5 years of age. 41 In the present study cohort, MDI scores and the incidence of mental delay did not improve in the recent surgical era.…”

Section: Mental and Psychomotor Outcomesmentioning

confidence: 99%

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

Atallah

Dinu²,

Joffe³

et al. 2008

Circulation

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Background-The Norwood procedure for stage 1 palliation of hypoplastic left heart syndrome is performed with either the modified Blalock-Taussig (MBTS) or the right ventricle-to-pulmonary artery (RVPA) shunt. In our institution, surgical practice changed from use of the MBTS to use of the RVPA shunt in 2002. We analyzed survival and mental and psychomotor outcomes of the 2 consecutive surgical eras. Methods and Results-Between September 1996 and July 2005, 94 neonates with hypoplastic left heart syndrome underwent the Norwood procedure. Patients were recruited as neonates and followed up prospectively. Health, mental, and psychomotor outcomes (Bayley Scales of Infant Development-II) were assessed at 2 years. The study subjects were from the Norwood-MBTS era (nϭ62; 1996 to 2002) or the Norwood-RVPA era (nϭ32; 2002 to 2005). In the MBTS era, early and 2-year mortality rates were 23% (14/62) and 52% (32/62); the mean (SD) mental and psychomotor developmental indices were 79 (18) and 67 (19). In the RVPA era, early and 2-year mortality rates were 6% (2/32) and 19% (6/32); the mean (SD) mental and psychomotor developmental indices were 85 (18) and 78 (18). The 2-year mortality rate (Pϭ0.002) and the psychomotor developmental index (Pϭ0.029) were improved in the more recent surgical era. On multivariable Cox regression analysis, postoperative highest serum lactate independently predicted 2-year mortality in the MBTS and RVPA eras. Conclusions-Analysis of 2 consecutive surgical eras of hypoplastic left heart syndrome patients undergoing the Norwood procedure showed a significant improvement in 2-year survival and psychomotor development in the more recent era.

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“…Sixty-four per cent of these infants had varying degrees of mental retardation and 73% had substantial functional disability. However, Kern et al (44) noted that in their cohort, the median full scale IQ was 88 and only 8.3% of their patients met the diagnostic criteria for mental retardation. They found that although the results were not statistically significant, all HLHS patients scored lower on average on neurodevelopmental tests compared with matched controls (44).…”

Section: Surgical Treatment and Their Outcomesmentioning

confidence: 85%

“…However, Kern et al (44) noted that in their cohort, the median full scale IQ was 88 and only 8.3% of their patients met the diagnostic criteria for mental retardation. They found that although the results were not statistically significant, all HLHS patients scored lower on average on neurodevelopmental tests compared with matched controls (44). The cause of a high prevalence of developmental disabilities remains unclear, illustrating the need to evaluate these patients continuously to assess their quality of life (2,17).…”

Section: Surgical Treatment and Their Outcomesmentioning

confidence: 85%