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AAP Grand Rounds 20:6-7 (2008)
© 2008 American Academy of Pediatrics
| The first 20% of the full text of this article appears below. |
| PICO Question: Among one-year-old survivors of palliation for hypoplastic left heart syndrome, what are the relative effects of underlying genetic factors and current management strategies on neurodevelopmental disabilities? Question type: Prognosis Study design: Prospective
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Authors from Childrens Hospital of Philadelphia performed neurodevelopmental testing at one year of age in a cohort of patients who underwent staged palliation for hypoplastic left heart syndrome (HLHS) and variants.
Eligible infants included those undergoing initial surgical repair between April 1998 and March 2003. Exclusion criteria included multiple congenital anomalies, recognized chromosomal or phenotypic syndromes at birth (except 22q11 deletion), and patients with non-English-speaking parents. Patients diagnosed with recognized syndromes after surgical repair or at the one-year evaluation were not excluded.
Evaluations included a neuromuscular examination and the Bayley Scales of Infant Development II, which provide the Mental Development Index (MDI) and the Psychomotor Development Index (PDI). The mean score on the Bayley Scales of Infant Development is 100 with a standard deviation of 15. Scores <70 are greater than two standard deviations below average.
One hundred sixty-one infants had staged repair
| Pediatric Cardiology, Childrens Hospital and Harvard Medical School, Boston, MA |
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